bromochloroacetic-acid and Nerve-Sheath-Neoplasms

The authors present a case of clear cell sarcoma (CCS) in which the tumor originated in the S-1 nerve root and had been previously diagnosed as psammomatous melanotic schwannoma (PMS). This is the third case of a spinal nerve root origin for CCS reported in the English-language literature. The similar histogenesis of CCS and malignant melanoma supports the hypothesis that biological agents or immunotherapy are potentially important areas of investigation. The patient underwent S1-3 laminectomy and gross-total resection of the mass lesion. The border of the resection was extended 1 cm distal to the tumor margin. The postoperative period was uneventful. The new histopathological diagnosis was CCS (malignant melanoma of soft tissue). Despite total resection, the patient returned with disseminated disease at the 18-month follow-up visit. His follow-up magnetic resonance image of the lumbar spine revealed sacral L5-S3 involvement of the vertebral bodies along with disseminated cauda equina seeding. A CCS originating from peripheral nerves is quite rare. The histopathological and immunohistochemical appearance of CCSs resembles those of PMSs. Surgery should be the first choice of treatment.

Topics: Adolescent; Adult; Antigens, Neoplasm; Biomarkers, Tumor; Breast Neoplasms; Diagnosis, Differential; Diagnostic Errors; Female; Fibroadenoma; Humans; Keratins; Male; Melanins; Melanoma-Specific Antigens; Neoplasm Invasiveness; Neoplasm Proteins; Neoplasm Recurrence, Local; Neoplasms, Multiple Primary; Neoplastic Syndromes, Hereditary; Nerve Sheath Neoplasms; Neurilemmoma; Peripheral Nervous System Neoplasms; Pigmentation Disorders; Prognosis; S100 Proteins; Sacrococcygeal Region; Sarcoma, Clear Cell; Spinal Nerve Roots; Syndrome; Vimentin

In this report, we describe a highly unusual case of glandular malignant peripheral nerve sheath tumor presenting as a neck mass in a previously healthy 29-year-old man. Grossly, the tumor was found to arise from a swollen peripheral nerve trunk. The tumor was largely composed of spindle cells that demonstrated marked nuclear pleomorphism and numerous abnormal mitotic figures. In addition, histologically malignant glandular structures lined by simple nonciliated columnar cells with goblet cells were found clustered in the center of the tumor. Examination of the swollen peripheral nerve trunk revealed the presence of a plexiform neurofibroma. The spindle cells were positive for S100. The glands were negative for S100 but positive for keratin, epithelial membrane antigen, and neuroendocrine markers (somatostatin, chromogranin, Leu-7, and calcitonin). This patient was subsequently diagnosed as having von Recklinghausen disease and died of tumor metastasis to the lungs 34 months after the presentation. To our knowledge, only 3 similar cases have been previously described in the literature.

Topics: Adult; Biomarkers; Carcinoembryonic Antigen; Cell Nucleus; Epithelial Cells; Fatal Outcome; Head and Neck Neoplasms; Humans; Immunohistochemistry; Keratins; Lung Neoplasms; Male; Mitosis; Mucin-1; Nerve Sheath Neoplasms; Neurofibromatosis 1; S100 Proteins; Skin Neoplasms

The paper discusses morphologic and immunohistochemical characteristics of sclerosing perineurioma. Generally, it is well circumscribed and consists of tiny spindle-shaped plump epitheloid cells embedded in collagenous hyalinized matrix. Immunohistochemically, it was represented by EMA+, S-100, AE1/AE3, CAM 5.2, smooth muscle actin and desmin. Being benign, tumor was identified by differential diagnosis using fibroma of tendon sheath, sclerosing one, glomal tumor, giant cell tumor and sclerosing epitheloid cell sarcoma.

Topics: Adult; Antiporters; Biomarkers; Biomarkers, Tumor; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Nerve Sheath Neoplasms; Peripheral Nervous System Neoplasms; S100 Proteins; Sclerosis

As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in the differential diagnosis, and analyzed the data with cluster analysis. Stain intensity was scored as none, weak, or strong. For CD99, tumors with membranous accentuation were independently categorized. Cluster analysis sorted five groups, with like tumors clustering together. Synovial sarcoma clustered into two groups: one cytokeratin and EMA positive (n = 11), the other mostly cytokeratin negative, EMA positive, bcl-2 positive and mostly CD56 positive (n = 9). Malignant peripheral nerve sheath tumor clustered into two groups: one S100 positive, with nestin and NGFR positivity in most (n = 10), the other mostly S100 negative, and variably but mostly weakly positive for nestin and NGFR (n = 11). Ewing sarcomas clustered into a single group driven by membranous CD99 staining. Thirteen cases failed to cluster (outliers), while three Ewing sarcomas clustered into groups of other tumor types. Paired antibodies for each tumor type determined by visual assessment of cluster analysis data and statistical calculations of specificity, sensitivity, and predictive values showed that EMA/CK7 for synovial sarcoma, nestin/S100 for malignant peripheral nerve sheath tumor, and membranous CD99/Fli-1 for Ewing sarcoma yielded high specificity and positive predictive values. Cluster analysis also highlighted aberrant staining reactions and diagnostic pitfalls in these tumors. Hierarchical cluster analysis is an effective method for analyzing high-volume immunohistochemical data.

Topics: 12E7 Antigen; Antigens, CD; Biomarkers, Tumor; Bone Neoplasms; CD56 Antigen; Cell Adhesion Molecules; Cluster Analysis; Diagnosis, Differential; Humans; Immunohistochemistry; Keratins; Mucin-1; Nectins; Nerve Sheath Neoplasms; Nerve Tissue Proteins; Proto-Oncogene Protein c-fli-1; Receptors, Nerve Growth Factor; S100 Proteins; Sarcoma, Ewing; Sarcoma, Synovial

Ectopic hamartomatous thymoma (EHT) is a rare benign tumor. We present a case of EHT, which was seen as subcutaneous mass on the left supraclavicular area in a 19-year-old man. The tumor consisted of spindle cells, epithelial cells, adipose cells, and a small amount of lymphocytes, as described previously. Immunohistochemically, spindle cells were positive for keratin, a-smooth muscle actin, CD34 and vimentin, but negative for desmin and S-100 protein. Lymphocytes were positive for CD45RO but negative for CD20, CD1a, and CD99. Approximately, 5% of cells were positive for MIB-1 and no cells stained for p53 and bcl-2. Recognition of EHT is important and needs to be differentiated from high-grade sarcomas such as synovial sarcoma or glandular malignant peripheral nerve sheath tumor.

Topics: 12E7 Antigen; Adult; Antigens, CD; Antigens, CD1; Antigens, CD20; Antigens, CD34; Cell Adhesion Molecules; Choristoma; Diagnosis, Differential; Hamartoma; Humans; Keratins; Leukocyte Common Antigens; Male; Nerve Sheath Neoplasms; Sarcoma, Synovial; Soft Tissue Neoplasms; Thymoma; Thymus Neoplasms; Vimentin

We present a rare case of primary extracranial meningioma in a 36-year-old man, who had a solitary multinodular mass located in the plantar muscle of the foot. The histology of specimens from simple excision was typical of meningioma, showing bland spindle cell proliferation with a whorl pattern. Immunohistochemical analysis demonstrated that the tumor cells showed diffuse and strong positivity for epithelial membrane antigen as well as moderate reactivity for cytokeratin and vimentin. Ultrastructurally, the tumor cells were characterized by thin bipolar cytoplasmic processes and joined by multiple small desmosomes. There were frequent pinocytotic vesicles and a distinct external lamina on the cell surface. These findings suggest that this primary ectopic meningioma, arising in the soft tissue, may have been derived from perineurial cells of the peripheral nerve, but was morphologically distinguishable from perineurioma. Primary extracranial meningioma should be included in the differential diagnosis of soft-tissue spindle cell tumors, especially those of peripheral nerve origin.

Topics: Adult; Diagnosis, Differential; Foot Diseases; Humans; Immunohistochemistry; Keratins; Male; Meningioma; Nerve Sheath Neoplasms; Soft Tissue Neoplasms; Vimentin

Monophasic synovial sarcoma (MSS) and malignant peripheral nerve sheath tumor (MPNST) are spindle cell sarcomas with overlapping histologic features, and their immunophenotypes may overlap, since MPNSTs express S-100 protein in only 50% to 60% of cases and rarely express epithelial markers, whereas MSSs can express S-100 protein in up to 40% of cases. We immunostained 29 cases of MSS and 29 cases of MPNST with antibodies to AE1/AE3, CAM 5.2, epithelial membrane antigen (EMA), S-100 protein, and cytokeratin subsets 7 and 19. Inclusion criteria for MSS included a consistent histology with expression of at least 1 epithelial marker. Inclusion criteria for MPNST included a tumor with a consistent histology arising in a patient with neurofibromatosis type 1 and/or in a plexiform neurofibroma, or ultrastructural confirmation of clear-cut schwannian differentiation. By definition, all cases of MSS were positive for at least 1 epithelial marker. Ten cases showed focal S-100 protein immunoreactivity, and 26 cases stained for cytokeratins 7 and 19. Twenty-three cases stained for both antigens, whereas only 2 cases were negative for both cytokeratins. Twenty-two MPNSTs demonstrated immunoreactivity for S-100 protein, and 11 stained focally for AE1/AE3 or EMA. Two cases of MPNST stained for cytokeratin 7, and only 1 case stained for cytokeratin 19. No cases of MPNST stained for both cytokeratins. Antibodies to cytokeratins 7 and 19 are useful adjuncts for the separation of MSS from MPNST. The majority of MSSs stain for one or both of these antigens, whereas most MPNSTs, including those that are EMA- or AE1/AE3-positive, do not express these cytokeratin subsets.

Topics: Biomarkers, Tumor; Diagnosis, Differential; Humans; Immunoenzyme Techniques; Keratin-7; Keratins; Nerve Sheath Neoplasms; Sarcoma, Synovial; Soft Tissue Neoplasms

Although benign epithelioid peripheral nerve sheath tumors have been described, they are rare, and benign epithelioid schwannomas have not yet been established as a specific histologic variant. We present four cases of tumors which we believe would meet criteria to be classified as benign epithelioid schwannomas. Biopsy specimens obtained from four different patients were examined with routine and immunohistochemical staining. All the tumors were well-circumscribed lesions that were surrounded by a capsule containing EMA-positive cells. The cellular component was composed of epithelioid cells, in which there was a lack of mitotic activity. Immunohistochemical studies showed the tumor cells were S-100 protein and Leu 7 positive and HMB-45 negative. In addition, type IV collagen encircled individual cells within the tumor, indicating a continuous basal lamina. We report a group of cutaneous epithelioid schwannomas. Although the presence of such tumors is not unexpected, this diagnosis may not be initially considered because of this rare cytologic feature.

Topics: Adult; Antigens, Neoplasm; Collagen; Female; Humans; Immunohistochemistry; Keratins; Male; Melanoma-Specific Antigens; Mucin-1; Neoplasm Proteins; Nerve Sheath Neoplasms; Neurilemmoma; Peripheral Nervous System Neoplasms; S100 Proteins; Schwann Cells; Skin Neoplasms; Vimentin

Synovial sarcoma is a relatively common sarcoma in adults, which in its classic bimorphic form infrequently poses a diagnostic problem. Monomorphic spindled variants, as well as the less common poorly differentiated variants, may be confused with other soft-tissue sarcomas; the poorly differentiated variant (PDSS), in particular, may be histologically indistinguishable from other small, blue, round cell tumors, including primitive neuroectodermal tumors (PNETs). Detection of the synovial sarcoma-associated t(X;18) by either cytogenetic or molecular genetic approaches may be necessary to confirm the diagnosis of synovial sarcoma in difficult cases. We evaluated 10 cases of PDSS from eight patients using a panel of antibodies (including those to intermediate filament proteins, nerve-sheath associated markers, and neuronal and neuroectodermal associated markers) in order to better establish the immunophenotype of this tumor and to help distinguish it from the tumors with which it may be confused, particularly PNETs and high-grade malignant peripheral nerve sheath tumors (MPNSTs). Our results showed PDSS to have significant immunophenotypic overlap with both PNETs and MPNSTs. In most instances these three entities may be differentiated by a panel of antibodies that should include those to both low and high molecular weight cytokeratins, epithelial membrane antigen, type IV collagen, CD99, CD56, and S-100 protein. Our results also suggest that synovial sarcoma may be a tumor showing combined neuroectodermal and nerve sheath differentiation--perhaps because of translocation-associated expression of specific proteins--rather than a carcinosarcoma of soft tissues or a tumor of specialized arthrogenous mesenchyme.

Topics: 12E7 Antigen; Adolescent; Adult; Antigens, CD; Biomarkers, Tumor; Cell Adhesion Molecules; Child; Collagen; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Karyotyping; Keratins; Male; Middle Aged; Mucin-1; Nerve Sheath Neoplasms; Neuroectodermal Tumors, Primitive; Neurofilament Proteins; Peripheral Nervous System Neoplasms; Sarcoma, Synovial

Intracranial nervous-system tumours were diagnosed in three of 1092 bovine necropsy specimens submitted to the Department of Veterinary Pathology, Obihiro University between April 1983 and March 1996. A fourth case was a referral from the Department of Veterinary Pathology, Rakuno Gakuen University. Histopathological examination revealed four types of tumour: intracranial malignant peripheral nerve sheath tumour (MPNST), choroid plexus papilloma, differentiated fibrillary astrocytoma and anaplastic (malignant) astrocytoma. Immunohistochemically, the intracranial MPNST was strongly positive for S-100 protein and vimentin, and in places weakly positive for glial fibrillary acid protein (GFAP). The choroid plexus papilloma was strongly positive for epithelial membrane antigen (EMA), keratin, S-100 protein and vimentin, and positive for GFAP in places. The cytoplasm and fibrous component in the differentiated fibrillary astrocytoma were strongly positive for S-100 protein and GFAP. The anaplastic (malignant) astrocytoma was strongly positive for vimentin, S-100 protein and keratin in the cytoplasm and fibrous processes, and weakly positive for GFAP and EMA in places. Myelin basic protein (MBP) and synaptophysin showed a weak positive reaction in the marginal areas of the tumour.

Topics: Animals; Astrocytoma; Brain Neoplasms; Cattle; Cattle Diseases; Glial Fibrillary Acidic Protein; Glioblastoma; Glioma; Immunohistochemistry; Keratins; Mucin-1; Myelin Basic Protein; Nerve Sheath Neoplasms; S100 Proteins; Vimentin

Topics: Cell Differentiation; Epithelioid Cells; Humans; Immunohistochemistry; Keratins; Microscopy, Electron; Nerve Sheath Neoplasms; Peripheral Nervous System Neoplasms

A primary malignant peripheral nerve sheath tumor (MPNST) of the pleura that clinically mimicked a malignant mesothelioma in a 57-year-old man is described. Histologically, the tumor had features similar to those described in cases of the so-called epithelioid MPNST. A unique finding in this case was the demonstration of keratin expression in the epithelioid component of the tumor, as well as the presence of rhabdomyoblasts. This is the first example of an MPNST with heterologous elements arising in the pleura. Immunohistochemical and ultrastructural studies were important in differentiating this tumor from other malignancies with sarcomatoid and epithelioid features involving the pleura.

Topics: Actin Cytoskeleton; Biomarkers, Tumor; Cell Differentiation; Diagnosis, Differential; Fatal Outcome; Glycogen; Humans; Keratins; Male; Mesothelioma; Middle Aged; Neoplasm Recurrence, Local; Nerve Sheath Neoplasms; Peripheral Nervous System Neoplasms; Pleural Neoplasms; Rhabdomyosarcoma

The glandular peripheral nerve sheath tumor is a rare variant of nerve sheath neoplasms in which the focally occurring glands are lined by cells showing divergent differentiation. The vast majority of the reported nerve sheath tumors harboring these glands have been malignant. We herein present a case of benign glandular peripheral nerve sheath tumor in a 43-year-old woman who had no evidence of von Recklinghausen's disease. Histologically, the tumor is composed of spindle cell component and collections of glandular component. The glandular component occupied the central two-thirds of the lesion and was lined by a single layer of nonciliated cuboidal or columnar cells. No mitotic figures were recognized in the spindle cell area. This spindle cell area had neurofibroma-like features rather than schwannoma. Many of the spindle cells had positive reaction products for S-100 protein. The glandular lining epithelium were positive for cytokeratins (CAM 5.2, AE1/AE3, PKK1) and EMA. Some epithelial cells were immunoreactive for CEA, chromogranin, somatostatin and Leu-7. These immunohistochemical findings support the neuroendocrine differentiation of the epithelial element from the schwannian component.

Topics: Adult; Carcinoembryonic Antigen; Chromogranins; Female; Humans; Immunohistochemistry; Keratins; Membrane Glycoproteins; Mucin-1; Mucins; Neoplasms, Nerve Tissue; Nerve Sheath Neoplasms; S100 Proteins; Somatostatin