Clinical Assistant Professor of Orthopaedics and Rehabilitation
Yale University School of Medicine
New Haven, Connecticut
It is also commonly involved in eosinophilic granuloma hormone androgen deprivation therapy for prostate cancer generic casodex 50mg without a prescription, particularly the C2 vertebra in adults androgen hormone network cheap casodex 50mg with mastercard. T Soft Tissue Extension Some of the primary spinal tumors have a proclivity for soft tissue extension prostate 24 theralogix buy casodex 50mg cheap. Occasionally mens health xtreme order casodex 50mg amex, hemangiomas may be associated with an extensive soft 1900 Tumors prostate cancer and diet buy discount casodex 50mg on line, Spine mens health yoga purchase 50 mg casodex otc, Intradural, Extramedullary tissue component and are then referred to as aggressive hemangiomas. Enostosis appears very dense, with a density similar to cortical bone, lying within the cancellous vertebral body. Contrast enhancement following administration of gadolinium chelates can help characterize the tumor. A typical ring and arc pattern of enhancement is characteristic of chondroid tumor. Dumbbell or mushroom shape may be seen in chordoma together with preservation of disc space. The patterns mentioned are useful in characterizing the lesions, but they need to be cautiously interpreted taking into account the rest of the features. More recently, due to intensified therapy of systemic malignancies and prolonged patient survival as well as increased sensitivity of imaging techniques the incidence of detected leptomeningeal metastases has significantly increased. The mass usually displaces the spinal cord and enlarges the ipsilateral subarachnoid space. Nerve sheath tumours arise from dorsal sensory roots, although occasional examples on motor nerve roots have been recorded. They are often located in the anterolateral part of the spinal canal; their distribution throughout the spine is fairly uniform with a slight lumbar predominance. Two main types of nerve sheath tumours are found in the spine: schwannoma and neurofibroma. Schwannomas are encapsulated, well-circumscribed round or oval tumours that often show cystic degeneration and haemorrhage. Necrosis and cystic degeneration are rare in neurofibromas and the parent nerve typically runs through the lesion with nerve fibres dispersed throughout the tumour. Occasionally an intra-dural tumour extends through the intervertebral foramen to expand into a greater mass on the peripheral nerve. The extra-vertebral tumour is often the significant clinical feature and may be found in the neck, posterior mediastinum, behind the peritoneum or in the pelvis, depending on the level of origin of the mass (3). The peak incidence is in the fifth and sixth decades with predominance in women (80%). The thoracic spine is the most common site (80%), followed by cervical spine (15%). These tumours are usually located in the posterior aspect of the spinal canal and as in the cranium, the tumour is usually firmly fixed to the dura (2). Paragangliomas: Paraganglioma is a tumour of paraganglia, the accessory organs of the peripheral nervous system. Spinal paragangliomas are rare tumours that are found in the cauda equina and filum terminale. Males are affected slightly more than female, and the average age at diagnosis is around 50 years (2, 3). They can be congenital or acquired, as a late complication of lumbar puncture by deposition of a fragment of epidermis within the dural sac. Congenital epidermoids are mainly located at the conus or cauda equina level and are often associated with vertebral abnormalities (spina bifida, hemivertebrae). Acquired cysts are found in the lower lumbar region and lack osseous abnormalities (2). Dermoid Tumours: Dermoid tumours are congenital midline cystic tumours and account for 20% of intra-dural tumours seen during the first year of life. Half of them are intra-medullary and others are extramedullary intra-dural masses. Neurenteric Cysts (spinal enterogenous cysts): these are uncommon congenital intra-dural cysts usually located in the thoracic spine anterior to the spinal cord. Lipomas: these tumours account for about 1% of all tumours of the spinal canal and are distributed equally between the sexes. Associated congenital abnormalities, such as spina bifida or other forms of dysraphism are found in about a third of the cases. Teratomas: these are very rare in the spine, and may occur in isolation or in association with congenital spinal anomalies. Age of patients at diagnosis 1902 Tumors, Spine, Intradural, Extramedullary depend on the type of the primary neoplasm. Clinical Presentation Clinical presentation is often non-specific and related to the location and the volume of the lesion. Pain and radiculopathy are the most frequent presenting symptom, followed by paresthesia and limb weakness. Both schwannomas and neurofibromas usually become symptomatic in the middle decades with a slight female predominance for schwannomas. Schwannomas never become malignant, whereas sarcomatous transformation occurs in 4% to 11% of patients with neurofibromatosis. Most spinal meningiomas are benign, slowly compressing the cord and causing progressive motor (90%) or sensory (60%) deficits. On plain films, scalloping of vertebral bodies, pedicle erosion and enlarged neural foramen may be found. Paragangliomas: these are well-encapsulated masses, isointense with cord on T1- and iso- to hyperintense on T2-weighted images. Paragangliomas are highly vascular tumours and enhance strongly following contrast administration. In case of rupture, multi-focal high signal areas are found on T1-weighted images. Figure 1 Cystic schwannoma: Sagittal T1-weighted (a), sagittal T2-weighted (b) and sagittal T1-weighted with contrast (c) images of the lumbar spine show a well-defined mass appearing isointense on T1, isointense on T2 and enhances after contrast injection, corresponding to a schwannoma (black arrows). Tumors, Spine, Intradural, Extramedullary 1903 Tumors, Spine, Intradural, Extramedullary. Figure 2 Meningioma: Sagittal T2-weighted (a) and sagittal T1-weighted images after contrast (b) of the thoracic spine show a meningioma which appears isointense to the cord on T2, and enhances homogeneously after contrast (white arrows). Note the dural attachment of the mass (black arrows) and the mass effect on the spinal cord which is displaced anteriorly. Sagittal T2-weighted (a) and sagittal T1-weighted with contrast (b) images of the lumbar region show multiple small nodular lesions in the intra-dural extra-medullary space which appear hypointense on T2, and enhance after contrast injection. The cord and the cauda equina are compressed and displaced by the lesions (arrows). Metastases: these lesions appear as thickened roots or nodular lesions which are isointense with spinal cord in most cases. Following contrast administration even small nodular metastases usually enhance strongly and permit better depiction of the lesions. Intravenous contrast injection may also demonstrate diffuse meningeal infiltration. The process starts as hyaline degeneration, which is then followed by calcification. Temporal Bone, Inflammatory Diseases, Acute, Chronic Tyrosinemia Genetic error of metabolism associated with severe liver disease in infancy due to the absence of an enzyme (fumarylacetato hydrolase) resulting in accumulation of toxic metabolic products and progressive liver and kidney failure. Infants may show liver and spleen enlargement, hemorrhagic diathesis, and jaundice. The chronic form is characterized by a gradual onset and more severe liver damage with liver and spleen enlargement, ascites, jaundice. Neoplasms, Oral Cavity Synonyms Gastric ulcer; Duodenal ulcer Definition Peptic ulcer is defined as a loss of mucous surface, causing gradual disintegration and necrosis of the tissues (1). Partial or complete loss of the mucosa only, without penetration into the submucosa, is generally called erosion. Peptic ulcers affect about 10% of the adults in Western countries and may be located in the stomach or the duodenum (which is four times more common). They are clinically important not only because of symptoms (mainly pain) but also because of complications such as bleeding and perforation. A peptic ulcer is a benign condition that, in cases of gastric ulcers, rarely transform to malignancy. Gastric ulcers are usually larger than duodenal ulcers and affect an older population (2). Ulcerative Colitis Colitis, Ulcerative Ulcerative Pancolitis Colitis, Ulcerative Ulcerative Proctitis Colitis, Ulcerative Pathology/Histopathology Both duodenal and gastric ulcers are essentially gastric ulcers because they occur in the gastric mucosa of the stomach or 1906 Ulcer Peptic in gastric metaplasia of the duodenal mucosa (3). Peptic ulceration in the stomach or duodenum is considered the outcome of an imbalance between the aggressive acid/peptic factors and the mucosal defense factors. The large majority of peptic ulcers are strongly related to Helicobacter pylori infestation and/or nonsteroidal anti-inflammatory drug use. Gastric ulcers result from weakened mucosal resistance in patients who have normal or even decreased acid secretion. Aspirin and other nonsteroidal antiinflammatory drugs inhibit the synthesis of prostaglandins that contribute to the integrity of the gastric mucosal barrier. Chronic gastritis, duodenogastric reflux of bile, and delayed gastric emptying also play a role in the pathogenesis of gastric ulcers. Patients with duodenal ulcers usually have antral gastritis and peptic acid hypersecretion. A combination of other hereditary and environmental factors also plays a role in the development of peptic ulcers. Hereditary parameters include family history of ulcers, Oblood type, and some genetic syndromes. The list of etiologic agents includes stress, smoking, alcohol, coffee, and cocaine. Nowadays, radiological assessment is required in those who are unable or unwilling to tolerate an endoscopy. Ulcers on the lesser or greater curvature are better depicted in profile views, whereas those on the anterior or posterior wall are better seen en face. Supine and oblique views are ideal for revealing ulcers located on the posterior wall or greater curvature. Flow technique (slow rotation of the patient from side to side) is helpful to identify shallow ulcers on the posterior wall or near the gastric cardia because it may lead the barium to the dependent surface of the stomach. Ulcers on the lesser curvature are better evaluated in the upright compression views. Prone compression views or views in prone Trendelenburg position are recommended for anterior gastric wall ulcers. Clinical Presentation Duodenal Ulcers Postprandial epigastric pain during the night is the typical and major symptom. Other symptoms consist of right upper quadrant, back, or chest pain; bloating; belching; nausea and vomiting; anorexia; and weight loss. Complications include free or defined perforation (peritonitis and constant pain that radiates to the back are the relevant symptoms), gastric outlet obstruction, and gastrointestinal bleeding (more likely to occur in gastric ulcers). Conversely, symptoms can be typical for peptic ulcer, but both radiological and endoscopic examinations may reveal no pathology (2). A biphasic examination that also includes upright compression views and doublecontrast views of the duodenal bulb is considered complete (2). When they are greater than 2 cm (giant ulcers), they are more prone to bleeding or perforation. It has also been noted that malignancy is more common in giant ulcers than in their small counterparts (3). Gastric ulcers are commonly round or ovoid collections of barium, but they can also be linear (which probably represent a healing process), rod-shaped, rectangular, serpiginous, or flame-shaped. When seen en face, an ulcer on the dependent wall of the stomach fills Imaging Gastric Ulcers Despite excellent diagnostic results, the number of barium studies performed to diagnose peptic ulcer disease has Ulcer Peptic 1907 with barium, whereas an ulcer on the nondependent wall will appear as a ring. Most of them (about 80%) are located in the lesser curvature or the posterior wall of the stomach. Inflammatory changes in adjacent soft tissues in addition to gastric wall thickening denote an ulcerous penetration. Ulcers on the lesser curvature typically appear as smooth protrusions beyond the normal contour of the stomach. When this radiolucent line becomes wider because of edema, it gives rise to the ulcer collar or the ulcer mound. Inflammation of the surrounding mucosa is responsible for enlarged areae gastricae, while retraction of the adjacent gastric wall leads to the development of radiating folds (2). Figure 1 A smooth, round ulcer (arrow) is seen on the lesser curvature, projecting beyond the normal contour of the stomach. Radiating folds and enlarged areae gastricae are seen in the adjacent mucosa because of associated inflammation and edema. They may appear to have an intraluminal location, with an inner margin concave toward the lumen, and their crater may be incompletely filled because of overhanging edematous tissue (quarter moon or crescent sign). Although there is a history of aspirin ingestion in most of these ulcers, implying benign gastric ulcers, endoscopy may be required because of suspicious findings.
Leiomyosarcoma Splenic leiomyosarcoma is an extremely rare nonlymphoreticular malignant tumor that originates from muscular cells prostate cancer 15 year survival rates by stage purchase casodex 50mg with amex. Even in poorly differentiated examples man health wire order casodex 50mg, actin and myosin are present in the tumor cells (3) prostate cancer proton therapy generic casodex 50mg without prescription. Leiomyosarcoma and Fibrosarcoma Fibrosarcoma Fibrosarcoma of the spleen is another extremely rare nonlymphoreticular malignant tumor man health blog discount 50mg casodex amex. This is a fibroblastic malignant lesion that consists of bundles of spindle-shaped cells arranged at angles to one another (3) oncology prostate cancer discount casodex 50mg. Leiomyosarcoma and fibrosarcoma prostate cancer recovery buy casodex 50mg with amex, extremely rare primary nonlymphoreticular malignant tumors of the spleen, present clinical findings like those of splenic hemangiosarcoma. These lesions are aggressive with high rates of both local recurrence and metastatic disease. N Metastases Metastases of the spleen are rare and usually associated with disseminated disease. Splenic metastases usually occur as a result of hematogenous spread from lung, breast, prostate, stomach, or ovarian cancer or from malignant melanoma. The gross features of splenic metastases are variable, including either single or multiple lesions with cystic, solid, or complex structure. Metastases Metastatic disease involving the spleen is uncommon, and isolated metastasis to the spleen is extremely rare. Most patients with splenic metastases have widely disseminated metastatic disease usually arising from lung, colorectal, endometrial, ovarian, thyroid, pancreatic, or gastric cancers or, most commonly, melanoma. Although most patients with splenic metastases are clinically asymptomatic, painful splenomegaly, splenic vein thrombosis, and splenic rupture have been rarely reported (4). This contrast is selectively taken up by the normal spleen and not by malignant cells, thus improving the tumor/ spleen contrast on T2-weighted images (1, 2). Chronic Myeloproliferative Disorders Imaging findings of chronic myeloproliferative disorders are similar to those of lymphoma. Hemangiosarcoma Sonographically, splenic hemangiosarcoma appears as a complex, poorly defined mass with a heterogeneous echostructure. Increased Doppler flow may be seen in the more solid echogenic portions of the tumor. However, areas of increased and decreased signal intensity due to the intratumoral presence of blood products and necrosis are often observed. Postcontrast-enhanced computed tomography shows an enlarged spleen with numerous hypodense lesions, smaller than 1 cm, corresponding to foci of lymphoma. Leiomyosarcoma and Fibrosarcoma Imaging findings of leiomyosarcoma and fibrosarcoma are similar to those of hemangiosarcoma. Postcontrast-enhanced computed tomography demonstrates splenomegaly with a typical low-attenuating mass; a second small lesion is also appreciable. Postcontrast-enhanced computed tomography demonstrates splenomegaly with multiple ischemic areas. In conclusion, although accurate evaluation with crosssectional imaging may be helpful in differentiating between benign and malignant splenic lesions, percutaneous biopsy or splenectomy should be performed in doubtful cases. Nuclear Medicine Metabolic imaging allows the recognition of active tumor mass because of its fixed tracer. On imaging, the malignant lesions are more likely to be multifocal due to metastases, or they tend to be diffuse and ill defined due to rapid growth (4). They may present different structures, including a homogeneous composition in lymphoma, variable patterns in metastases, and, usually, a complex cystic appearance in hemangiosarcoma. Irregular borders, signs of infiltration of the surrounding splenic parenchyma, lymphonodal masses, and extrasplenic metastases are highly suggestive for malignancy. Most tumors are acquired, but congenital tumors such as congenital cholesteatoma also occur. Most tumors of the temporal bone have a typical age peak and characteristic symptoms. Pathology/Histopathology Exostosis Exostosis is a broa-based overgrowth of compact bone in the medial osseous part of the external auditory canal. It occurs in ocean swimmers, divers, and surfers and is 1318 Neoplasms, Temporal Bone known as a response to cold water exposure. Osteoma Osteoma is a benign bony tumor that can arise in the external auditory canal, the mastoid, or the petrous pyramid. Middle Ear Adenoma Middle ear adenoma is a benign tumor of glandular origin, originating from the mucosa and growing slowly. Malignant Neoplasm of the External Auditory Canal Squamous cell carcinoma is a malignant epithelial tumor that may arise from the external auditory canal, pinna, or middle ear. Basal cell carcinoma is a slowly growing local aggressive tumor that can occur at the outer ear. Ceruminal gland adenocarcinoma is a malignant tumor of modified apocrine glands, located in the external auditory canal. Ceruminal gland carcinomas are characterized by a loss of the normal glandular double cell layer and presence of pleomorphic luminal epithelial cells. It is predominantly located at areas that are exposed to sunlight or at which chronic inflammation is present. Other malignant tumors, such as malignant melanoma and sarcomas, can also occur within the outer auditory canal or ear. Intralabyrinthine schwannomas originate from the Schwann cells surrounding the peripheral fibers of the cochlear nerve or the cristae or maculae of the vestibule. Meningioma this benign tumor represents the second most common tumor in the cerebellopontine angle and originates from the dura. This strongly vascularized tumor develops from the arachnoid villi component of the leptomeninges. Acquired Cholesteatoma Cholesteatoma is a sac lined by keratinizing stratified squamous epithelium, trapped and growing in the middle ear space, mastoid, or other pneumatized spaces of the temporal bone. It may develop following retraction of the tympanic membrane due to negative middle ear pressure associated with Eustachian tube dysfunction. Cholesteatoma can also develop after invasion of keratinized stratified squamous epithelium from the surface of the tympanic membrane into the middle ear after (small) perforation of the tympanic membrane. They originate from maldevelopment of mesoderm or of meningeal precursor tissue, namely the meninx primitive. Endolymphatic Sac Tumor Endolymphatic sac tumor is an adenomatous tumor arising from the pars rugosa (middle third) of the endolymphatic sac. It is hypervascularized and has been reported to contain a speculated calcified matrix in almost all known cases. It arises in the glomus bodies situated along Neoplasms, Temporal Bone 1319 Rhabdomyosarcoma Rhabdomyosarcoma is a malignant tumor of skeletal muscle origin. It is a highly aggressive neoplasm arising from rhabdomyoblasts, which are embryonal cells that usually differentiate into striated muscle cells. In the temporal bone it is supposed to originate from the muscle cells in the vicinity of the Eustachian tube in the middle ear. Middle Ear Adenoma Dizziness, tinnitus, and conductive hearing loss are the most common symptoms. Patients with acoustic schwannoma classically present with sensorineural hearing loss, tinnitus, and disequilibrium. If the tumor is large, it can lead to retention of debris, external otitis, and/or hearing loss. If the tumor obstructs the outer auditory canal, debris can accumulate and cholesteatoma may result in the long run. Because nerve fibers run through the lipoma, surgical therapy is extremely difficult. Malignant Neoplasm of the External Auditory Canal and Ear Basal cell carcinomas are asymptomatic. Suspicion should be raised if erosions at sunlight-exposed areas persist over 3 weeks. Malignant neoplasms of the external auditory canal and ear have a peak at higher ages (>70 years). Endolymphatic Sac Tumor Sensorineural hearing loss and tinnitus are common symptoms. Otalgia, bloody otorrhea, facial nerve palsy, and sensorineural hearing loss are common symptoms. In more advanced stages, symptoms are due to progressing bone erosion and may include vertigo (erosion of the lateral semicircular canal) or facial palsy (erosion of the facial nerve canal). Meningitis, abscess in the temporal lobe, and lateral sinus thrombosis are possible late-stage complications. It has a broad base and begins deep to the isthmus within the medial aspect of the osseous internal auditory canal in the close vicinity of the annulus. Glomus Tympanicum Due to the vascular nature of the tumor, pulsatile tinnitus is the most common symptom at initial presentation (90%). Conductive hearing loss occurs in 50% of patients and is due to blocked movement of the tympanic membrane. However, erosion of the underlying bone and infiltration of neighboring structures are signs of malignancy of a mass. The main role of imaging is to assess the extent of the neoplasm and infiltration of vital structures and determine the presence of metastasis. Cholesteatoma does not enhance after administration of contrast medium, but due to granulation tissue, there is enhancement at the rim of the tumor. After intravenous administration of contrast medium, the tumor strongly enhances on T1-weighted images. Brain edema in the vicinity of the tumor is a sign of infiltration of the arachnoid. In these cases the tumor is difficult to remove, and a higher rate of recurrence must be expected. The axial high-resolution computed tomographic image shows a middle ear cavity opacification. Axial T1-weighted magnetic resonance image before (left) and after intravenous administration of contrast medium (right). The tumor is isointense to the brain before administration of contrast medium (arrow in the left image). It strongly enhances after administration of contrast medium (arrow in the right image). Neoplasms, Thyroid, Benign and Malignant 1321 Diagnosis Several tumors, such as schwannoma and lipoma, have a pathognomonic appearance on imaging. In all tumors without such a pathognomonic imaging appearance, biopsy or surgical removal and histology are essential for correct diagnosis. In patients with exostosis, otoscopy shows a circumferential stenosis of the external auditory canal. In cholesteatoma, otoscopy shows a retraction pocket or perforation of the tympanic membrane and a white mass behind the tympanic membrane. If otoscopy shows a reddish mass behind the posteroinferior part of the tympanic membrane, glomus tympanicum tumor must be considered. Before biopsy can be performed, imaging is required to rule out an aberrant internal carotid artery. This developmental anomaly causes similar clinical symptoms (pulsatile tinnitus and conductive hearing loss) and has the same appearance at otoscopy. Furthermore, glomus tympanicum must be differentiated from glomus jugulare, which arises from the jugular foramen and may extend through the posterior floor of the middle ear cavity into the middle ear, because the two tumors require different surgical approaches. The T2-weighted magnetic resonance image shows a small tumor (arrow) in the internal auditory canal. On T1-weighted images, the mass is hypointense and enhances after intravenous administration of contrast medium. Bone scintigraphy with technetium 99 m methylene diphosphonate may lead to detection of metastasis to the temporal bone at an early stage or show metastasis from a temporal bone neoplasm to other parts of the body. Department of Radiologic Sciences, University of Messina, Messina, Italy cvisalli@unime. Nodules are palpable masses in the thyroid gland, often found incidentally on ultrasound examination. This pathological condition is four times more common in women than in men and occurs more often in people who live in geographic areas with iodine deficiency or who are exposed to ionizing radiation. Differentiated tumors (papillary or follicular) are highly treatable and usually curable. Poorly differentiated tumors (medullary and anaplastic) are aggressive, metastasize early, and have a much poorer prognosis. Women are affected more often than men, and any age group can be affected, although thyroid cancer usually occurs in people between the ages of 25 and 65 years. It is also the predominant cancer type in children with thyroid cancer and in patients who have had radiation to the head and neck. In this group the cancer tends to be multifocal, with early lymphatic spread and a poor prognosis. Papillary carcinoma is a nonencapsulated lesion that histologically consists of a stromal core and packed papillae with a small amount of colloid within follicles. Pathology/Histopathology In general, nodular disease of the thyroid is common; however, malignancy of the thyroid is rare.
In acute middle ear and mastoid inflammation the mucopurulent fluid is a result of vasodilatation mens health xtreme buy 50 mg casodex amex, increasing glandular secretion with mucous production prostate back pain order casodex 50mg line, and polymorphonuclear reaction occurring from the neutrophil cells prostate in spanish buy 50 mg casodex otc. Resolution frequently occurs prostate lesion cheap casodex 50mg, but prostate biopsy recovery generic casodex 50 mg on-line, if for some reason prostate removal surgery buy casodex 50 mg without a prescription, the condition is prolonged, such as inability of the secretions to be drained out of the eustachian tube, the number of glands and goblet cells increases. The areas formerly covered by a cuboidal or flat epithelium change into areas of a less differentiated pseudostratified columnar epithelium. Localized areas of the mucosa become hyperplastic with invasion of fibroblasts, capillaries, macrophages, plasma cells, and lymphocytes. Chronic middle otitis can be distinguished into two main groups according to the integrity of the tympanic membrane: (a) otitis with integrity of the tympanic membrane, which generally represents serous-mucous otitis; (b) otitis with open tympanum, which includes chronic suppurative otitis, chronic noncholesteatomatous otitis with ossicular erosion, and cholesteatomatous otitis. In serous-mucous chronic otitis the aerial cavities of the middle ear are filled by a transuded serous and serousmucous fluid that can change into an exudate due to bacterial superimposition. Chronic noncholesteatomatous otitis is characterized by a tympanic perforation with otorrhea and a range of irreversible modifications that can involve the mucous membrane, the temporal bone, the ossicular chain, the mastoid air cells, and the eustachian tube. Telescope Phenomenon Telescope phenomenon is a characteristic aspect of inflammatory mutilation in late stages of psoriatic arthritis, with destruction not only of the epiphysis but also of the meta- and diaphysis of the phalanges, resulting in excessive shorting of the digit. It consists of a cyst-like mass lined with stratified squamous epithelium, usually keratinizing, and filled with desquamating debris often including cholesterol crystals. It behaves as an expansive lesion whose slow growth determines erosion and discharge of bony fragments. Sequelae or variants of chronic noncholesteatomatous otitis are polypoid otitis, adhesive or fibroadhesive otitis, and tympanosclerosis. External malignant otitis is the most significant pathological process of the external ear. Acute labyrinthitis is an inflammation of the fluidfilled space of the inner ear and the membranous labyrinth. Clinical Presentation the inflammatory process of the middle ear is generally the consequence of acute or chronic inflammatory affections of the main airway and almost always it also involves the mastoid (otomastoiditis). Inflammatory disease of the ear is easily assessed clinically: conductive hearing loss, otalgia (earache), and secretion represent the typical clinical signs of the inflammatory process of the external and middle ear. The symptomatology of acute labyrinthitis is characterized by vertigo and sensorineural hearing loss; tinnitus can sometimes be present. In malignant external otitis, together with general symptoms of inflammation, the local symptomatology consists in strong pain during movements of the temporomandibular joint due to solicitations of the tympanic membrane. It allows information to be obtained about the bony tissue and the content of the middle ear cavity. In some advanced cases, erosion of the lateral wall of the lateral semicircular canal can be found (labyrinthine fistulas). Imaging Acute otomastoiditis does not need evaluation by means of diagnostic imaging, with the exception of rare forms that are accompanied by intracranial complications. Chronic inflammatory diseases of the middle ear represent the main field of application of radiological imaging. Partial erosion of the malleus and polypoid hyperplasia of the internal wall of the tympanic membrane (arrow). Nuclear Medicine Bone scintigraphy does not have a role in the assessment of acute and chronic diseases of the temporal bone. In almost all cases of inflammatory disease of the external and middle ear, the diagnosis is based on the clinical and otoscopy data. Diagnostic imaging is indicated in all those situations in which suitable information is not available for correct therapeutic planning and in local or cranial complications such as labyrinthine fistulas and/or intracranial spreading of the disease. In cholesteatomatous disease it is well known that microscopic fiberoptic otoscopy allows an accurate evaluation of the tympanic cavity only, and is not able to assess the involvement of the attic, the antrum, and the mastoid. Interventional Radiological Treatment Interventional radiology does not have a role in the treatment of acute and chronic diseases of the temporal bone. Ed Idelson Liviana, Naples Sanjeev S, Pitkin L (2005) Middle Ear, Inflammatory Diseases. Inflammatory infiltration of adipose spaces (arrow) and bony erosion of the mandibular condyle. Involvement of the base of the skull and the temporomandibular joint can also be demonstrated. Temporomandibular Arthropathy Internal Derangement, Temporomandibular Joint Temporomandibular Joint Disorders Internal Derangement, Temporomandibular Joint 1792 Teratocarcinomas Teratocarcinomas Neoplasms, Chest, Childhood Definition Teratoma is a tumor consisting of tissues foreign to the site of origin, originating from all three germ layers. Overall most teratomas are benign lesions, but malignant degeneration is always possible, increasing with age. Teratoma Incidence A benign germ cell tumor with mature tissues derived from pluripotential cells of all three germinal layers. These tumors are usually large and consist of unilocular or multilocular cysts filled with solid components, including sebaceous material, hair, teeth, calcium and skin. Microscopically the cysts are lined by ciliated or squamous epithelium, while the wall contains dermal appendages (sebaceous glands and hair follicles) and sometimes other tissues (teeth, cartilage, bone). They contain hyperechoic material (due to hair, teeth and fat) and hypoechoic areas due to fluid material. They present as a mass at the level of the buttocks and extent below the gluteal fold. Presacral teratoma: pain, constipation, and urinary frequency or signs of urinary tract obstruction. When they become large, tracheal/bronchial compression resulting in chest pain, coughing, and wheezing may occur. Hyperglycemia or precocious puberty due to ectopic production of insulin or sex hormones is possible. Conventional: soft tissue mass, scoliosis, mediastinal widening, canal/foramina widening, pedicle erosion, abnormal calcification. Synonyms Benign teratoma; Cystic teratoma; Dermoid; Dermoid cyst Ultrasound assists in determining the cystic, or partial cystic, part of the tumor and the extent of the tumor. After contrast medium administration, the solid component may show enhancement. Definitions Ovarian teratomas comprise tumors composed of mature or immature tissues of primordial germ cell origin. They can be classified into three main categories: mature teratomas, immature teratomas, and monodermal teratomas (1). The mature cystic teratoma which constitutes 99% of these tumors contains mature elements of ectodermal, mesodermal, and endodermal origin. The mature cystic teratoma also known as dermoid cyst or dermoid is the most common ovarian neoplasm of women younger than 45 years. Although teratomas may occur at any age, they typically affect a younger age group than epithelial tumors, and are the most common ovarian masses in children (2). Nuclear Medicine Nuclear medicine is used for the evaluation of bone metastasis if the tumor shows malignant degeneration. Macroscopically, benign teratomas present most often as unilocular tumors surrounded by a firm capsule of varying thickness (1). Arising from the cyst wall is commonly one or more protuberances, the dermoid plug or Rokitansky nodule, composed of a variety of different tissues. Hair, bone, or teeth and rarely cartilage as well as phalanges may be encountered in this region (1). Clinical Presentation the majority of benign teratomas remain clinically asymptomatic. They are often discovered incidentally during clinical examinations or imaging studies. Especially larger teratomas may cause nonspecific symptoms including pelvic pain, abdominal fullness, abdominal swelling, and dysuria. Rarely, abnormal uterine bleeding and hemolytic anemia may be associated with a dermoid (1). Teratomas may become clinically apparent due to complications which include in order of decreasing frequency torsion (16%), chemical peritonitis caused by rupture, and infection (3). Rarely, giant dermoids may be encountered, which grow to a size of up to 40 cm (1). Rapid growth of a dermoid in a postmenopausal woman is a sign of malignant degeneration (2). Figure 1 Dermoid cyst in a 20-year-old patient: transvaginal sonography shows a well-delineated oval adnexal mass containing multiple homogeneous fine echoes and a solid protruding nodule, the Rokitansky nodule. In this patient the latter did not contain calcifications or teeth, and may be misdiagnosed as a solid element in ovarian cancer. Gruber, Salzburg) Imaging Conventional radiographs are not suited for the diagnosis of teratomas. Rarely, teeth, bone, or wall calcifications may be detected on abdominal plain films. Sonography is the primary imaging modality for assessing suspected adnexal masses. The combination of transabdominal and endovaginal sonography combined with Doppler findings allows optimal characterization of the morphology and perfusion of adnexal masses. They most commonly present as diffusely or partially echogenic masses with acoustic shadowing due to hair balls, calcifications, teeth, or the Rokitansky protuberance. However, imaging findings are often noncharacteristic, and the dermoid plug may be misinterpreted as a solid lesion (4). The lesion contains areas of higher density representing floating hair and mural calcifications. Teratoma, Ovaries, Mature, Ovalar 1795 Suppression of the high-signal-intensity sebaceous contents or fat within the lesions is diagnostic (2). Furthermore, chemical shift artifacts in the frequency-encoding direction may assist in detecting fat and in its differentiation from hemorrhage (2). Nuclear Medicine Nuclear medicine has a very limited role in the assessment of teratomas. It may be useful only in detecting monodermal teratomas, for example, struma ovarii or carcinoids. Diagnosis the diagnosis of teratomas is based on the demonstration of sebaceous material or fat within an adnexal mass (Figs 2, 3). In dermoid cysts, fat is demonstrated in more than 90% of cases, teeth in 31%, and calcifications in the dermoid plug, or more often in the cyst wall, in 56% (5). As hemorrhagic adnexal masses, endometriomas and hemorrhagic cysts in particular may display similar signal intensities. In these patients, small foci of fat may be found in the dermoid cyst wall or in the dermoid plug. When dermoids show no fat they cannot be differentiated from other cystic and solid ovarian tumors, including ovarian cancer. Malignant degeneration in mature cystic teratomas is typically due to carcinomatous or sarcomatous malignant transformation. The corresponding T1 fat saturation image shows fat suppression of an ovoid area smaller than 1 cm in diameter. At surgery, a 15-cm cystic dermoid was found, the Rokitansky nodule contained hair and only small areas of fat. In children dermoid cysts may be associated with an ipsi- or contralateral immature teratoma. The latter presents typically as a large, often solid mass that may contain tiny foci of fat or calcifications. Hyperparathyroidism Thoracic Aorta the thoracic aorta carries blood away from the heart and distributes it to the head, the arms, and passes into the abdomen where it becomes the abdominal aorta. Aneurysm, Aortic and Thoracic Testicular Torsion Torsion of the testis with twisting of the spermatic cord. It presents as acute scrotal pain and needs immediate surgical treatment to avoid loss of the testis or of testicular function. Brachial Ischemia Thrombocytopenia Thrombopoietic System, Diseases of the Thrombopoietic System, Diseases of the 1797 Thrombolysis Intravenous infusion or intra-arterial local application of agents with the potential to resolve blood clots. With a smaller thrombocyte count (<30,000 mm-3), donor blood must be used, because the 111In- or 51Cruptake of the platelets is relatively low in comparison to the erythrocytes. With a greatly reduced life span, blood samples are taken and measurements are performed at shorter time intervals. Simultaneously, the radioactivity from the liver, spleen, and heart (with reference to the blood pool) is measured. In particular, indications are the differential diagnosis of increased sequestration or a production disturbance, but also the therapy control of the thrombocytopenia. An increase in the thrombocytes in the spleen, and more seldom in the liver, is to be observed with organ-specific platelet sequestration and can support the indication for splenectomy. Pathology/Histopathology Thrombocytopenia may be due to a reduced production (marrow failure, megaloblastosis) or a decreased survival. T References Clinical Presentation Thrombocytopenia presents with prolonged bleeding from cuts, purpura, and bleeding from the mucous membranes.
Syndromes
Paralysis
Convulsions
Muscle weakness
Severe skin and soft tissue infection (necrotizing fasciitis)
Less than 1:80 (1 to 80) titer
Sore throat and postnasal drip
Heart problems
However prostate nutrition cheap casodex 50 mg overnight delivery, this method gives only regional localization and therefore is reserved for cases where other imaging modalities are negative prostate cancer early symptoms order 50 mg casodex mastercard. For patients with foregut carcinoids without hepatic metastases prostate cancer 5 year survival rates cheap casodex 50 mg free shipping, the 5-year survival in one study was 95% and with distant metastases androgen hormone qui discount casodex 50mg amex, 20% duke prostate oncology purchase 50mg casodex with mastercard. With gastrinomas prostate cancer 20s buy generic casodex 50 mg on-line, the 5-year survival without liver metastases is 98%, with limited metastases in one hepatic lobe it is 78%, and with diffuse metastases it is 16%. Radiotherapy with radiolabeled somatostatin analogues that are internalized by the tumors is an approach under investigation. These results suggest this novel therapy may be helpful, especially in patients with advanced metastatic disease. The use of liver transplantation has been abandoned for treatment of most metastatic tumors to the liver. Although no randomized studies have proven that hepatic resection extends life, results from a number of studies suggest it likely increases survival and therefore is recommended, if possible. How long tumor stabilization lasts or whether it prolongs survival has not been established. Several distinct genetic disorders predispose to endocrine gland neoplasia and cause hormone excess syndromes (Table 23-1). Menin interacts with JunD, suppressing the JunD-dependent transcriptional activation. It is unclear how this accounts for Menin growth regulatory activity, since JunD is associated with inhibition of cell growth. Each child born to an affected parent has a 50% probability of inheriting the gene. The variable penetrance of the several neoplastic components can make the differential diagnosis and treatment challenging. Hypercalcemia may develop during the teenage years, and most individuals are affected by age 40. This syndrome is characterized by neoplasia of the parathyroid glands, enteropancreatic tumors, anterior pituitary adenomas, and other neuroendocrine tumors with variable penetrance (Table 23-1). The neoplastic changes inevitably affect multiple parathyroid glands, making surgical cure difficult. Screening for hyperparathyroidism involves measurement of either an albumin-adjusted or ionized serum calcium level. Age at onset is the age at first symptom or, with tumors not causing symptoms, age at the time of the first abnormal finding on a screening test. The rate of diagnosis of hyperparathyroidism increased sharply between ages 16 and 20 years. Management is challenging because of early onset, significant recurrence rates, and the multiplicity of parathyroid gland involvement. Hyperplasia of one or more parathyroid glands is common in younger patients; adenomas are usually found in older patients or those with longstanding disease. Most of these tumors secrete peptide hormones that cause specific clinical syndromes. These syndromes, however, may have an insidious onset and a slow progression, making their diagnosis difficult and in many cases delayed. The robust acid production may cause esophagitis, duodenal ulcers throughout the duodenum, ulcers involving the proximal jejunum, and diarrhea. The ulcer diathesis is commonly refractory to conservative therapy such as antacids. The diagnosis is made by finding increased gastric acid secretion, elevated basal gastrin levels in the serum [generally >115 pmol/L (200 pg/mL)], and an exaggerated response of serum gastrin to either secretin or calcium. Other causes of elevated serum gastrin levels, such as achlorhydria, treatment with H2 receptor antagonists or proton pump inhibitors, retained gastric antrum, small-bowel resection, gastric outlet obstruction, and hypercalcemia, should be excluded. The diagnosis can be suggested by documenting hypoglycemia during a short fast with simultaneous inappropriate elevation of serum insulin and C-peptide levels. More commonly, it is necessary to subject the patient to a supervised 12- to 72-h fast to provoke hypoglycemia (Chap. The trend to earlier diagnosis of, hence, smaller tumors has reduced the usefulness of octreotide scanning, which is positive in a minority of these patients. The glucagonoma syndrome may represent a complex interaction between glucagon and ghrelin overproduction and the nutritional status of the patient. The Verner-Morrison, or watery diarrhea, syndrome consists of watery diarrhea, hypokalemia, hypochlorhydria, and metabolic acidosis. The diarrhea can be voluminous and is almost always found in association with an islet cell tumor, prompting use of the term pancreatic cholera. However, the syndrome is not restricted to pancreatic islet tumors and has been observed with carcinoids or other tumors. These secondary interactions add complexity to the diagnosis and management of these tumor syndromes. Pancreatic islet cell tumors are diagnosed by identification of a characteristic clinical syndrome, hormonal assays with or without provocative stimuli, or radiographic techniques. One approach involves annual screening of individuals at risk with measurement of basal and meal-stimulated levels of pancreatic polypeptide to identify the tumors as early as possible; the rationale of this screening strategy is the concept that surgical removal of islet cell tumors at an early stage will be curative. These tumors can exhibit aggressive behavior and local invasiveness that make them difficult to resect (Chap. Adrenal cortical tumors are found in almost one-half of gene carriers but are rarely functional; malignancy in the cortical adenomas is uncommon. Due to its rarity, screening for these tumors is only indicated when there are suggestive symptoms. Mediastinal carcinoid tumors (an upper mediastinal mass) are more common in men; bronchial carcinoid tumors are more common in women. The closed circles show the relative distribution of mutations, mostly inactivating, in each exon. Mutation data are derived from the Human Gene Mutation Database from which more detailed information can be obtained ( The major value of genetic testing in a kindred with an identifiable mutation is the assignment or exclusion of gene carrier status. It is presumed that these mutations are somatic and occur in a single cell, leading to subsequent transformation. Most develop hyperparathyroidism, 80% develop pancreatic islet cell tumors, and more than half develop pituitary tumors. For most of these tumors, initial surgery is not curative and patients frequently require multiple surgical procedures and surgery on two or more endocrine glands during a lifetime. For this reason, it is essential to establish clear goals for management of these patients rather than to recommend surgery casually each time a tumor is discovered. There is less agreement Subcutaneous or visceral lipomas and cutaneous leiomyomas may also be present but rarely undergo malignant transformation. In the first, all parathyroid tissue is identified and removed at the time of primary operation, and parathyroid tissue is implanted in the nondominant forearm. Thymectomy should also be performed because of the potential for later development of malignant carcinoid tumors. Second, performance of a total pancreatectomy to prevent malignancy causes diabetes mellitus, a disease with significant long-term complications that include neuropathy, retinopathy, and nephropathy. These features make it difficult to formulate clear-cut guidelines, but some general concepts appear to be valid. Treatment with H2 receptor antagonists (cimetidine or ranitidine) or proton pump inhibitors (omeprazole, lansoprazole, esomeprazole, etc. Hepatic artery embolization, radiofrequency ablation, or chemotherapy (5-fluorouracil, streptozocin, chlorozotocin, doxorubicin, or dacarbazine) may reduce tumor mass, control symptoms of hormone excess, and prolong life; however, these treatments are never curative. Consideration should be given to participation in clinical trials of new agents that target specific molecular pathways. Surgical resection of a prolactinoma is rarely curative but may relieve mass effects. As a result, other neoplastic manifestations that develop later in the course of this disorder, such as carcinoid syndrome, are now seen with increased frequency. Multiple Endocrine Neoplasia Type 2A could lead to death or serious morbidity from pheochromocytoma in an affected kindred member. This tumor usually develops in childhood, beginning as hyperplasia of the calcitonin-producing cells (C cells) of the thyroid. Measurement of the serum calcitonin level after calcium or pentagastrin injection makes it possible to diagnose this disorder at an early stage in its development (see below). About half of the tumors are bilateral, and >50% of patients who have had unilateral adrenalectomy develop a pheochromocytoma in the contralateral gland within a decade. A second feature of these tumors is a disproportionate increase in the secretion of epinephrine relative to norepinephrine. The manifestations of hyperparathyroidism do not differ from those in other forms of primary hyperparathyroidism (Chap. Diagnosis is established by finding hypercalcemia, hypophosphatemia, hypercalciuria, and an inappropriately high serum level of intact parathyroid hormone. Multiglandular parathyroid hyperplasia is the most common histologic finding, although with longstanding disease adenomatous changes may be superimposed on hyperplasia. Metastatic disease has been described prior to 1 year of age, and death may occur in the second or third decade of life. However, the prognosis is not invariably bad even in patients with metastatic disease, as evidenced by a number of multigenerational families with this disease. The mucosal neuromas and marfanoid body habitus are the most distinctive features and are recognizable in childhood. Neuromas are present on the tip of the tongue, under the eyelids, and throughout the gastrointestinal tract and are true neuromas, distinct from neurofibromas. The most common presentation in children relates to gastrointestinal symptomatology including intermittent colic, pseudoobstruction, and diarrhea. The first is a cysteinerich extracellular domain; point mutations in the coding sequence for one of six cysteines (codons 609, 611, 618, 620, 630, or 634) cause amino acid substitutions that induce receptor dimerization and activation in the absence of its ligand. The first affects a cysteine-rich (Cys-Rich) region in the extracellular portion of the receptor. Each germline mutation changes a cysteine at codons 609, 611, 618, 620, or 634 to another amino acid. Uncommon mutations (<5% of the total) include those of codons 533 (exon 8), 666, 768, 777, 790, 791, 804, 891, and 912. These findings mirror results in other malignancies where germline mutations of cancer-causing genes contribute to a greater percentage of apparently sporadic cancer than previously considered. Establishment of the specific germline mutation facilitates the subsequent analysis of other family members. Individuals in a kindred with a known mutation who have two normal analyses can be excluded from further screening. In children with codon 611, 618, 620, 630, 634, and 891 mutations, thyroidectomy should be performed before the age of 6 years because of reports of local metastatic disease in children this age. However, in other kindreds there are examples of metastatic disease occurring early in life. For example, metastatic disease prior to the age of 6 years has been described with codon 609 and 804 mutations and before the age of 14 years in a patient with a codon 912 mutation. Patients should be warned before pentagastrin injection of epigastric tightness, nausea, warmth, and tingling of extremities and reassured that the symptoms will last ~2 min. The goal is to identify a pheochromocytoma before it causes significant symptoms or is likely to cause sudden death, an event most commonly associated with large tumors. Women should be tested during pregnancy because undetected pheochromocytoma can cause maternal death during childbirth. Total thyroidectomy with a central lymph node dissection should be performed in children who carry the mutant gene. Incomplete thyroidectomy leaves the possibility of later transformation of residual C cells. The goal of early therapy is cure, and a strategy that does not accomplish this goal is short-sighted.
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