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Neurotropic melanoma invading the median nerve.

We report a case of acral lentiginous melanoma of the right thumbnail bed which demonstrated characteristic intraneural invasion and extension along the median nerve. Four years after amputation of the involved thumb, a melanotic tumor recurred on the right thenar. Radiation therapy was given. The tumor invaded the median nerve, however, causing progressive pain and paralysis of the right hand. Despite right arm amputation, the tumor extensively metastasized, and the patient died three years later. Histopathologically, the tumors were characterized by extensive proliferation of spindle-shaped cells forming neuroid fascicles especially prominent in the metastatic region. Tumor cells were positive immunohistochemically with S-100 protein antisera.

Amputation, Surgical↗

Usefulness of epithelial membrane antigen (EMA) to discriminate between perineural invasion and perineural indentation in prostatic carcinoma.

AIMS: Perineural invasion (PNI) is one of the few unequivocal criteria to diagnose adenocarcinoma of the prostate. Distinguishing PNI from perineural indentation (PNIn), however, is sometimes difficult. The aim of this study was to discriminate between PNI and PNIn using EMA immunohistochemistry. METHODS AND RESULTS: We selected representative sections from 87 prostatectomies with prostatic adenocarcinoma. Normal peripheral nerves were continuously encircled with perineurium, which was immunoreactive for EMA. We identified 1319 PNI by carcinomas, 368 PNIn by carcinomas, and 303 PNIn by benign glands. We categorized the EMA immunoreactivity patterns into three classes: samples that displayed discontinuity or complete loss of the perineurium (Type A), samples where there were carcinomas or benign glands in the perineural space or peripheral nerves (Type B) and samples that showed no changes in the perineurium (Type C). For PNI we observed Type A, Type B, and Type C patterns in 55.3%, 24.8% and 19.9% of carcinomas, respectively. The incidence of each of those patterns in PNIn by carcinomas was 32.1%, 14.9% and 53.0%, respectively. Cases of PNIn by benign glands showed Type A or Type C patterns. They did not, however, exhibit Type B patterns. CONCLUSION: EMA immunostaining will aid the diagnosis of prostatic adenocarcinoma.

Adenocarcinoma↗

Neuroblastoma. Evaluation of 60 cases.

60 cases of neuroblastoma from the departments of paediatrics and paediatric surgery, Rigshospitalet, are reviewed. The overall cure rate was 35%. In localized disease (stages I--II) the prognosis was favourable (cure rate 78%), while the prognosis for disseminated disease (stages III--IV) was poor (cure rate 10.8%). Neuroblastomas in stage IV-S had a favourable prognosis in spite of metastases to the liver, skin and bone marrow. The clinical stage at the time of diagnosis was the only independent prognostic factor in this study.

Age Factors↗

April 2004: woman in her early fifties with a cervical intraspinal and extraspinal mass lesion.

April 2004: A 53-year-old woman presented with a large club-shaped intra- and extraspinal tumor of the C6 nerve root which had intradurally grown through the enlarged C5-6 neural foramen and focally infiltrated the dura mater. Microscopy revealed a melanin-pigmented tumor with spindle-shaped and epithelioid cells and scattered psammoma bodies, a so-called psammomatous melanotic schwannoma (PMS). More than half of the patients with PMS have Carney complex, a genetically heterogeneous multiple neoplasia syndrome of autosomal-dominant inheritance, which in our patient, however, could not be detected unequivocally. Prognosis of all melanotic schwannomas (MSs) is not good with local recurrences or metastases in over 40% of cases. In our case, frequent versicular nuclei with distinct nucleoli, occasional mitoses and apoptoses and increased focal MIB-1 labeling indices prompted us to diagnose a malignant PMS. However, histologic criteria for malignancy in MSs are not clearly defined and there is no reliable histopathological indicator of malignant clinical behavior in MSs. Therefore, designation of "PMS with malignant histologic features" may be more appropriate. Since tumor recurrences and metastases im MSs may occur after more than 5 years, long-term follow-up of affected patients is required. One year after operation our patient showed no signs of tumor recurrence or metastases.

Antigens, Neoplasm↗

Malignant mesenchymoma of ulnar nerve: combined sarcoma of nerve sheath and rhabdomyosarcoma.

Malignant mesenchymoma within the right ulnar nerve of an 8 year old boy is described. The patient did not have stigmata of von Recklinghausen's neurofibromatosis. The growing and painful tumour was excised five and a half detection, and recurred five months later. Mingling of the nerve sheath sarcoma and rhabdomyosarcoma was noted within the same mass which was separated from the adjacent striated muscles. It is suggested that this mesenchymoma arose from mesenchymal cells or cells of mesenchymal type comprising the peripheral nerve sheath which is derived from ectomesenchyme of the neural crest.

Child↗

Nerve and muscle microvasculitis in peripheral neuropathy: a remote effect of cancer?

In a series of 50 cases in which nerve and/or muscle microvasculitis was seen on biopsy, seven were associated with malignancy. In two cases, the cancer was found after the discovery of microvasculitis. All patients exhibited sensory-motor neuropathy, which was often painful and asymmetrical, with a progressive course. ESR and CSF protein levels were always elevated. Motor conduction velocity was slightly reduced in three cases, unmeasurable in one case, and normal in three. Cancers involved were adenocarcinoma in five cases (three prostate and two lung), Hodgkin's disease in one and immunoblastic lymphadenopathy in one. A thorough search for cancer should be performed when microvasculitis is seen in nerve or muscle biopsy specimens, especially when ESR and CSF protein levels are elevated.

Adenocarcinoma↗

Computed tomography with the EMI scanner in the diagnosis of primary and metastatic intracranial neoplasms.

The findings and diagnostic results in 600 examinations of primary and metastatic intracranial neoplasms performed at one hospital with the EMI scanner are presented and the effectiveness of this method is compared with that of radionuclide imaging, cerebral angiography, and pneumoencephalography. The computed scan proved to be highly reliable in the diagnosis of glioma, cerebral metastases, meningioma, and acoustic neuroma.

Adenocarcinoma↗

An approach to contrast enhancement in computed tomography of the brain.

A total of 450 brain examinations were performed using computed tomography before and after intravenous infusion of methylglucamine diatrizoate 30%. In 110 cases involving a documented intracranial lesion, the scans were analyzed to determine the degree and usefulness of contrast enhancement. Enhancement was achieved in a wide variety of neoplastic and non-neoplastic lesions and was found to be of considerable help in detection of abnormality as well as in defining tumor morphology.

Adenoma, Chromophobe↗

Soft-tissue sarcoma involving bone or neurovascular structures: MR imaging prognostic factors.

PURPOSE: To determine if magnetic resonance (MR) imaging findings of soft-tissue sarcoma involving bone or neurovascular structures allow prediction of local recurrence, distant metastasis, or disease-specific survival. MATERIALS AND METHODS: Preoperative MR images of 46 patients with soft-tissue sarcoma were reviewed for tumor involving bone or major vessels or nerves. MR imaging findings were correlated with local recurrence, distant metastasis, and disease-specific survival after surgery and chemotherapy and/or radiation therapy. Primary tumors were predominantly in the lower extremity (n = 35 [76%]), deep (n = 44 [96%]), and high-grade (n = 35 [76%]). RESULTS: On MR images, bone invasion occurred in 12 patients (26%), major-vessel encasement in five patients (11%), and major-nerve encasement in seven patients (15%). In patients with (n = 12) and those without (n = 34) bone invasion, frequencies of disease-related death (in nine [75%] and 12 [35%] patients, respectively) were significantly different (P = .02); frequencies of local recurrence or distant metastasis were not significantly different. In patients with and those without major-vessel or major-nerve encasement, there were no significant differences between frequencies of local recurrence, distant metastasis, or disease-specific survival. CONCLUSION: In soft-tissue sarcoma, bone invasion on MR images was predictive of decreased disease-specific survival. MR imaging findings of bone or neurovascular involvement otherwise appear to be more important for tailoring surgery than for predicting local recurrence, distant metastasis, or survival.

Bone Neoplasms↗

Chest wall tumors: radiologic findings and pathologic correlation: part 1. Benign tumors.

Benign chest wall tumors are uncommon lesions that originate from blood vessels, nerves, bone, cartilage, or fat. Chest radiography is an important technique for evaluation of such tumors, especially those that originate from bone, because it can depict mineralization and thus indicate the diagnosis. Computed tomography (CT) and magnetic resonance (MR) imaging are helpful in further delineating the location and extent of the tumor and in identifying tumor tissues and types. Although the radiologic manifestations of benign and malignant chest wall tumors frequently overlap, differences in characteristic location and appearance occasionally allow a differential diagnosis to be made with confidence. Such features include the presence of mature fat tissue with little or no septation (lipoma), the presence of phleboliths and characteristic vascular enhancement (cavernous hemangioma), evidence of neural origin combined with a targetlike appearance on MR images (neurofibroma), well-defined continuity of cortical and medullary bone with the site of origin (osteochondroma), or fusiform expansion and ground-glass matrix (fibrous dysplasia). Both aneurysmal bone cysts and giant cell tumors typically manifest as expansile osteolytic lesions and occasionally show fluid-fluid levels suggestive of diagnosis.

Adult↗

Chest wall tumors: radiologic findings and pathologic correlation: part 2. Malignant tumors.

Malignant chest wall tumors are classified into eight main diagnostic categories: muscular, vascular, fibrous and fibrohistiocytic, peripheral nerve, osseous and cartilaginous, adipose, hematologic, and cutaneous. However, there are malignant tumors that arise in the chest wall and that do not fit well in any of these categories (eg, Ewing sarcoma and synovial sarcoma). Malignant chest wall tumors typically manifest as painful, rapidly growing, large palpable masses. Chest radiography, the technique most often used for initial evaluation, can be helpful for detecting cortical destruction. However, computed tomography is more sensitive than chest radiography for detecting calcified tumor matrix and cortical destruction. Magnetic resonance imaging often allows more accurate delineation and localization of the tumor and is helpful for determining the presence and extent of tumor invasion and for tissue characterization. Although the imaging features of many malignant chest wall tumors are nonspecific, knowledge of the typical radiologic manifestations of these tumors often enables their differentiation from benign chest wall tumors and occasionally allows a specific diagnosis to be suggested. The article reviews the clinical and imaging features of the most common malignant chest wall tumors and presents images collected at a single cancer referral center.

Adult↗

From the archives of the AFIP: benign musculoskeletal lipomatous lesions.

Benign lipomatous lesions involving soft tissue are common musculoskeletal masses that are classified into nine distinct diagnoses: lipoma, lipomatosis, lipomatosis of nerve, lipoblastoma or lipoblastomatosis, angiolipoma, myolipoma of soft tissue, chondroid lipoma, spindle cell lipoma and pleomorphic lipoma, and hibernoma. Soft-tissue lipoma accounts for almost 50% of all soft-tissue tumors. Radiologic evaluation is diagnostic in up to 71% of cases. These lesions are identical to subcutaneous fat on computed tomographic (CT) and magnetic resonance (MR) images and may contain thin septa. Lipomatosis represents a diffuse overgrowth of mature fat affecting either subcutaneous tissue, muscle or nerve, and imaging is needed to evaluate lesion extent. Lipoblastoma is a tumor of immature fat occurring in young children, and imaging features may reveal a mixture of fat and nonadipose tissue. Angiolipoma, myolipoma, and chondroid lipoma are rare lipomatous lesions that are infrequently imaged. Spindle cell and pleomorphic lipoma appear as a subcutaneous lipomatous mass in the posterior neck or shoulder, with frequent nonadipose components. Hibernoma appears as a lipomatous mass with serpentine vascular elements. Benign lipomatous lesions affecting bone, joint, or tendon sheath include intraosseous lipoma, parosteal lipoma, liposclerosing myxofibrous tumor, discrete lipoma of joint or tendon sheath, and lipoma arborescens. Intraosseous and parosteal lipoma have a pathognomonic CT or MR appearance, with fat in the marrow space or on the bone surface, respectively. Liposclerosing myxofibrous tumor is a rare intermixed histologic lesion commonly located in the medullary canal of the intertrochanteric femur. Benign lipomatous lesions may occur focally in a joint or tendon sheath or with diffuse villonodular proliferation in the synovium (lipoma arborescens) and are diagnosed based on location and identification of fat. Understanding the spectrum of appearances of the various benign musculoskeletal lipomatous lesions improves radiologic assessment and is vital for optimal patient management.

Adolescent↗

Micrographic surgery ('slow Mohs') in cutaneous sarcomas.

BACKGROUND: Micrographic surgery (MS) results in very low local recurrence rates in all sorts of skin tumours that grow by extensive subclinical infiltration. Therefore, MS should prove useful in the treatment of cutaneous sarcomas. OBJECTIVE: To treat cutaneous sarcoma with MS in order to minimize local recurrence rates. METHODS: We treated 5 cases of dermatofibrosarcoma protuberans and 5 cases of cutaneous sarcomas of different origin (atypical fibroxanthoma, malignant fibrous histiocytoma, malignant peripheral nerve tumour) with micrographic surgery using paraffin-embedded sections. In primary cutaneous sarcomas, tumour extensions were readily detected in HE sections. In recurrent tumours, special stains were needed to distinguish tumour extensions from scar tissue. RESULTS: All patients were treated successfully and have remained free of local recurrences as of yet. Solitary pulmonary metastasis occurred in 1 patient with high-grade malignant peripheral nerve tumour. CONCLUSION: MS is an excellent procedure to minimize local recurrence in cutaneous sarcomas. Cutaneous sarcomas with low metastatic potential can be cured with MS.

Adult↗

Myxopapillary ependymoma of the conus medullaris and filum terminale in the pediatric age group.

Myxopapillary ependymoma of the conus medullaris and filum terminale is a relatively common spinal intradural neoplasm in adulthood. However, only a reported 8-12% of such tumors affect this site in children, and the ideal management remains controversial. Three children with myxopapillary ependymomas of the conus medullaris and filum terminale were treated by the author over a 2-year period with an at least 24-month follow-up. These children, ages 7, 8 and 13 years, included 1 male and 2 females. Their salient presentation was an acute exacerbation of chronic lower back pain. The duration of symptoms prior to diagnosis ranged from 16 to 18 months. A preoperative MRI, with and without contrast, was available for every case. All children underwent replacement laminoplasty with gross total tumor resection. Somatosensory evoked potentials were used in all surgeries. An early postoperative MRI of the entire neural axis was available for all cases. No permanent complications were noted. Self-catheterization for 6 weeks was required in 1 child with preoperative urinary incontinence. One child received radiation therapy following a recurrence. Clinical and surgical results were compared to the only 2 other reported series (11 patients) addressing this type of tumor in children. Based on this review, the authors propose that: (1) unexplained and intractable lumbar pain in childhood should be thoroughly investigated with an MRI scan: (2) the gross feature of myxopapillary ependymoma allowing for complete resectability appears to be the key prognostic factor; (3) radiotherapy appeared to have no proven value in completely resected tumors in children; (4) postoperative baseline MRI and regular sequential imaging studies are essential for long-term follow-up, and (5) replacement laminoplasties may be of value in preventing future spinal deformities, musculoskeletal pain and allowing for an 'easier' resection in the event of a recurrence.

Adolescent↗