Malignant Epithelioid Schwannoma Research Articles

Epithelioid malignant peripheral nerve sheath tumor arising in schwannoma

Epithelioid malignant peripheral nerve sheath tumor (EMPNST, malignant epithelioid schwannoma) is a rare variant of malignant peripheral nerve sheath tumor that has morphologic and immunophenotypic overlap with a variety of epithelioid neoplasms. Because of its rarity it may be potentially underrecognized. We describe a case arising in the subcutis of the thigh in a 25 year-old female, and discuss the pathologic features and differential diagnosis.

Hypopharyngeal Epithelioid Malignant Schwannoma Following Treatment of Hodgkin’s Lymphoma

The objective of this study is to present a patient with hypopharyngeal epithelioid malignant schwannoma as a secondary malignant neoplasm following treatment with MOPP and radiotherapy. The patient, presently a 43 year-old man, was diagnosed at clinical stage III Hodgkin’s lymphoma (HL) with mixed cellularity histopathology at the age of thirteen. He had received 40 Gy radiotherapy to the neck region and MOPP + maintenance MOPP (a total 10 cycles), while malignant schwannoma developed 30 years after the treatment. Although the patient further received four cycles of ifosfamide + adriamycin combination chemotherapy, he died with progression of his malignant schwannoma six months after diagnosis.

Malignant Epithelioid Schwannoma of the Oral Cavity in a Cat

ABSTRACTA malignant epithelioid schwannoma of the oral cavity was diagnosed in an 8-year-old domestic short-hair cat. The mass was located in the gingiva of the upper left premolar to molar region and showed multinodular growth patterns. The mass comprised epithelioid cells arranged in densely packed sheets. Tumor cells had large, round to oval nuclei with prominent nucleoli and an abundant eosinophilic cytoplasm. Immunohistochemically, most of the tumor cells were positive for S-100 protein, glial fibrillary acidic protein and vimentin, but all lacked melanoma-associated antigen and muscle and neuroendocrine markers. Stains for type IV collagen showed linear immunoreactivity around single cells and groups of cells. Ultrastructurally, tumor cells were separated by a well-defined basement membrane, and interdigitating cell processes were observed. To our knowledge, this is the first report of feline malignant epithelioid schwannoma.

Malignant peripheral nerve sheath tumour (malignant epithelioid Schwannoma) of the parotid gland

Malignant peripheral nerve sheath tumours are uncommon soft tissue tumours originating from Schwann cells or nerve sheath cells. Malignant epithelioid schwannoma is an aggressive variant of malignant peripheral nerve sheath tumour, and unfortunately is related with a high rate of recurrence and poor prognosis. In this study we present a rare case of malignant epithelioid schwannoma of the parotid gland and we discuss its origin, unusual presentation, and possible treatments options. Patient underwent total parotidectomy without neck dissection. Postoperative radiotherapy and chemotherapy management were not necessary. Patients with malignant epithelioid schwannomas typically present with pain and/or rapid enlargement of a pre-existing lesion but the diagnosis of these tumours remains difficult as it is based primarily on clinical suspicion. There is no recognized management for this tumour yet while the prognosis seems to correlate best with the extent of surgical resection (Fig. 4, Ref. 16).

Cutaneous epithelioid malignant schwannoma: review of the literature and case report

A case of malignant epithelioid schwannoma in the skin is reported. This was a rare variant of a malignant tumour that arose on the back of a 35-year-old male without neurofibromatosis. Clinically the nodule appeared to be a benign cyst but as it was painful it was decided to remove it. Ultrastructural and immunohistochemical features of the lesion were consistent with those of malignant epithelioid schwannoma so a radical excision was performed. Most ordinary malignant schwannoma are located in the deep soft tissue of the proximal portions of the upper and lower extremities and trunk; to the best of our knowledge only 26 cases of malignant epithelioid schwannoma in the skin and subcutis have been described in the literature. For the 14 male and 12 female patients reviewed, the median age was 43 years (range 19-84). Upper extremities were the most common sites (10 of 26). Seven patients developed local recurrences and four developed metastases: two to lung, one to lung and lymph node, and one to lung, liver and brain. All patients with local recurrence and all except one who developed metastases did not undergo wide local excision. We can conclude that malignant epithelioid schwannoma in the skin and subcutis is eminently curable if treated with wide local excision.

Epithelioid Malignant Peripheral Nerve Sheath Tumor In Perineal Region

Epithelioid malignant schwannoma is an uncommon variant of malignant schwannoma. There are a few published articles about epithelioid malignant schwannoma in literature. A 38- year-old woman who had a necrotic and ulcerated, bad smelt mass with a size of 30x25x20 cm, at her right perineal region that invaded right labium majus was hospitalized. The patient was underwent large resection and ipsilateral inferior epigastric artery based vertical rectus abdominis musculocutaneous flap to reconstruct the defect. The patient referred to the Oncology Department for chemotherapy. She died within 3 months after the first operation. because of metastasis.

Malignant epithelioid cranial nerve sheath tumor: case report of a radiation response

Malignant epithelioid nerve sheath tumors (MESs) especially those involving intracranial cranial nerves are rare and thought to be radioresistant. We report a case of a MES involving the Vth and VIIth cranial nerves responsive to radiotherapy. A 41-year-old man with progressive left facial weakness underwent an MRI that disclosed an enhancing lesion involving both V3 cranial nerve and the distal VIIth nerve. Biopsy confirmed a malignant epithelioid schwannoma. The tumor was resected but residual tumor was present at the cut end of the infraorbital nerve, within the oral cavity and at the brainstem. Positive excision margins were irradiated to 60 Gy/30 fractions by a wedge pair technique extending from the inferior orbit to C2. Tumor was controlled for 38 months and then radiographic recurrence was resected from the infratemporal fossa outside the irradiated field.A new primary MES tumor developed at 69 months at the C1/C2 root levels at the lower edge of the previously irradiated field. It was subtotally resected but by 77 months residual tumor had grown inferiorly down to C5 so this area was re-irradiated with a complicated 7-field approach to spare spinal cord. Initially the tumor responded but ultimately progressed posteriorly where radiation dose was limited by spinal cord tolerance. New involvement of the Xth cranial nerve was noted. The patient expired from brainstem compression 7 years after initial radiotherapy. The long-term control of the original tumor despite positive margins argues for the use of radiotherapy in the treatment of these tumors.

Malignant epithelioid ovarian schwannoma: a case report

There are only a few cases reported in the literature about schwannoma originating from the ovary. The patient was registered to the hospital due to a painless solid tumor in her left axillary region. Histologic examination of the excised tumor showed reticulum cell sarcoma. Two years later, oophorectomy was performed due to a unilateral ovarian tumor. The results of the histologic examination with different staining were particularly the same as those found in the axillary tumor, finally diagnosed as a malignant epithelioid schwannoma of the ovary. Our case report may be of interest for three reasons: 1) malignant schwannoma of the ovary is a very rare tumor, 2) the epithelioid type of the tumor may cause a differential diagnostic problem, and 3) clinical appearance of the metastasis was quite unusual, also resulting in difficulties in the appropriate diagnosis.

Malignant epithelioid ovarian schwannoma: a case report

There are only a few cases reported in the literature about schwannoma originating from the ovary. The patient was registered to the hospital due to a painless solid tumor in her left axillary region. Histologic examination of the excised tumor showed reticulum cell sarcoma. Two years later, oophorectomy was performed due to a unilateral ovarian tumor. The results of the histologic examination with different staining were particularly the same as those found in the axillary tumor, finally diagnosed as a malignant epithelioid schwannoma of the ovary. Our case report may be of interest for three reasons: 1) malignant schwannoma of the ovary is a very rare tumor, 2) the epithelioid type of the tumor may cause a differential diagnostic problem, and 3) clinical appearance of the metastasis was quite unusual, also resulting in difficulties in the appropriate diagnosis.

Schwannoma maligno epitelioide en antebrazo asociado a melanoma

Tumours originating in peripheric nerves usually appear in patients with neurofibromatosis (NF) signs, presenting frequent combinations of tumours in nerves and cutaneous lesions. Nevertheless, this association is very rare in cases without NF. Therefore, the aim of the present article is to present a case of malignant melanoma and malignant schwannoma, without any described NF. A 69-year-old woman with antecedents of malignant melanoma diagnosed two years previously in the dorsum of the fifth finger of the left hand, treated by means of amputation of the finger. The patient presented a malignant epithelioid schwannoma adhered to the median nerve that required elbow amputation. One month later lung metastases appeared and the patient died. This case presented no known NF sign. However, a relationship must be searched for in the common origin of melanoma and schwannoma from the embryonic neural crest.

Primary Malignant Schwannoma of the Uterine Cervix: A Case Report and Literature Review

Primary malignant schwannomas are a very rare neural sheath tumor. A case of primary epithelioid-type malignant schwannoma of the uterine cervix in a 65-year-old woman is presented. This tumor is the first epithelioid malignant schwannoma documented within gynecologic tissues. Only two other cases of primary malignant schwannoma of the uterine cervix have been reported, and these cases are reviewed.

Malignant epithelioid schwannoma of the inferior alveolar nerve: a case report with a survey of the literature: K.-O. Henkel, H.-P. Putzke, B. Saka, K. K. H. Gundlach. (Mund Kiefer GesichtsChir 1998; 2: 163–166)

Malignant epithelioid schwannoma of the inferior alveolar nerve: a case report with a survey of the literature: K.-O. Henkel, H.-P. Putzke, B. Saka, K. K. H. Gundlach. (Mund Kiefer GesichtsChir 1998; 2: 163–166)

Malignant epithelioid schwannoma with melanocytic differentiation: a rare tumour with an unusual feature.

No Abstract available

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We present here a subcutaneous tumor in a 23-year-old female that had a 5 months history of agressively enlarging subcutaneous nodule of the scalp with histologic features of malignant rhabdoid tumor (MRT) . Histologically, the tumor consisted of varying cellularity, and mainly composed of cords of cells with deeply eosinophilic cytoplasm, clear cells reminiscent of clear cell sarcoma, solidly sheets somewhat reminiscent of primitive neuroectodermal tumor, discohesive eosinophilic cells of having features of the MRT and the aggregation of spindle cells containing peripheral nerve-like organoid structures in a small area. Immunohistochemistry revealed the presence of neuron specific enolase, S-100 protein and vimenin within the tumor cells. A few spindel or dendritic cells were stained positively for anti-keratin. The p53 supressor gene protein was also positive, especially in the rhabdoid cells. We speculat-ed the tumor cells in this case revealed the neuroectodermal associated differentiaion, mainly differentiated towards the schwann cell. We concluded that the histological diagnosis was epithelioid malignant schwannoma with rhabdoid features. She was alive and well 7 months with no evidence of disease.

Mediastinal Malignant Epithelioid Schwannoma

A case of mediastinal malignant epithelioid schwannoma (MES) is reported. The tumor probably arose in the vagal nerve, and the trachea was involved. A few months after excision of the primary tumor, multiple metastases appeared in lung, cervical spine, and neck lymph nodes. Microscopically, the tumor showed a highly cellular area resembling melanoma or carcinoma. Immunolabeling was done for S-100 protein, keratin, and melanoma-associated antigen. Examination of the entire lesion and in situ characteristics of the tumor involving the vagal nerve were helpful in making the correct diagnosis. Mediastinal MES, to our knowledge, has not been reported to date in the English-language medical literature.

Primary epithelioid malignant schwannoma of the lung

This is the first documented report of a case of primary malignant nerve sheath tumor of the lung showing an epithelioid malignant schwannoma. A 55 year old Japanese female underwent lobectomy of the middle lobe of the right lung because of a large, rounded tumorous shadow found on chest X-ray film. Light microscopic examination of the neoplasm disclosed an epithelioid malignant schwannoma with foci having a spindle-cell sarcomatous pattern. The neoplastic cells were positive for S-100 protein and neuron specific enolase by the immunoperoxidase method. Transmission electron microscopy showed basement membrane-like material surrounding the neoplastic cells and long spacing collagens in the interstitial spaces. The patient died of a recurrent tumor in the right hemithorax 5 months after undergoing lobectomy. An autopsy revealed no primary site of the neoplasm outside the lung. Furthermore, there was no evidence of neurofibromatosis.

Primary Mesenchymal Tumors of the Urinary Bladder: A Histological and Immunohistochemical Study of 30 Cases

The light microscopic and immunohistochemical features of 30 primary mesenchymal neoplasms of the urinary bladder are reported. Half of the cases represented smooth and striated muscle tumors (five leiomyomas, seven leiomyosarcomas including epithelioid and myxoid subtypes, one rhabdomyoma, one embryonal rhabdomyosarcoma and one alveolar rhabdomyosarcoma). One third of the tumors were of fibrohistiocytic origin (one fibrous histiocytoma and eight malignant fibrous histiocytomas including fascicular and storiform, inflammatory and pleomorphic subtypes). In addition, a malignant epithelioid schwannoma, a round cell liposarcoma, two hemangiomas and two mixed mesodermal tumors were observed. The morphology of the vesical mesenchymal tumors was identical to that of their counterparts known to occur in other sites, particularly in the soft tissue. Muscle-specific actin, alpha-1-antichymotrypsin, S-100-protein and neuron-specific enolase proved to be useful and reliable immunomarkers for differential diagnosis of poorly differentiated leio- and rhabdomyosarcomas, malignant fibrous histiocytomas and malignant schwannomas. Since some tumors coexpressed several classes of intermediate filaments, diagnostic immunocytochemistry should only be used considering a larger panel of antibodies and in close correlation with the histological and cytological features of the neoplasms.

Malignant Epithelioid Schwannoma of the Skin Showing Partial HMB-45 Positivity

A malignant epithelioid schwannoma occurred on the right second toe of a 30-year-old Japanese man. It was a firm, flesh-colored, benign-appearing nodule and measured 13 x 9 mm in diameter and 6 mm in height. To our knowledge, this is the first case of malignant epithelioid schwannoma occurring on the toe. Histopathology was characterized by a circumscribed nodule in the dermis that predominantly consisted of atypical large epithelioid cells with some spindle cells whose proliferation was similar to that of the Verocay bodies seen in ordinary schwannoma. Fontana-Masson staining demonstrated no melanin pigment in the tumor at the light microscopic level. The eosinophilic cytoplasm contained abundant glycogen and was positive for S-100 protein and HMB-45, as usually seen in melanomas. Electron microscopy revealed that there was an abundance of long-spacing collagen in the extracellular matrix, and the cells contained numerous dense-cored granules. But no definite melanosomes were observed in any stage. As far as we are aware, this is the first case of a malignant epithelioid schwannoma showing HMB-45 positivity.

An Unusual Presentation of Clear Cell Sarcoma

An Unusual Presentation of Clear Cell Sarcoma

Cerebral Metastasis from an Epithelioid Malignant Schwannoma: Case Report

Abstract The authors present a case of brain metastasis from an epithelioid malignant schwannoma. The patient previously had undergone a surgical resection of the primary tumor in the right forearm. The neoplasm was composed of nests of cells with an entirely epithelioid appearance without spindle cell areas. Immunohistochemically, the tumor cells stained positive for S100 protein and negative for cytokeratin, neuron-specific enolase, and anti-melanoma antiserum. To our knowledge, this is the first reported case of cerebral metastasis from an epithelioid malignant tumor of the peripheral nerve sheath.