Immunohistochemical Diagnosis of a Rare Case of Epithelioid Malignant Peripheral Nerve Sheath Tumor with Multiple Metastases

ArticleinJapanese Journal of Ophthalmology 48(6):565-9 · November 2004with21 Reads
DOI: 10.1007/s10384-004-0121-8 · Source: PubMed
The epithelioid variant of malignant peripheral nerve sheath tumor (MPNST) is a rare tumor with poor prognosis that sometimes involves the head and neck. The diagnosis is based principally on the histological examination, and it is generally very difficult to reach the correct diagnosis. An 84-year-old Japanese woman presented with a tumor mass of 2 week's duration in the right medial canthal region. Although the tumor was excised surgically, metastases occurred three times on her face and head, and the patient died of distant systemic multiple metastases. In the histopathological analysis, the tumor showed a composite pattern comprising spindle or polygonal cells arranged in irregular bands, and a population of larger epithelioid cells in solid sheets and nests. In the immunohistochemical analysis, the tumor cells were positive for S-100 protein, vimentin, and nerve growth factor receptor (NGFR), and negative for cytokeratin and HMB 45 (melanoma-associated antigen). These findings confirmed the diagnosis of MPNST. Epithelioid MPNST has complex histopathological findings and histopathological features similar to other epithelioid tumors, especially malignant melanoma. Immunohistochemical examination using NGFR and HMB-45 is important in the differential diagnosis.
    • "The diagnostic challenge, including differential diagnoses and value of IHC in its objective diagnosis, is discussed herewith. Malignant peripheral nerve sheath tumors in soft tissues generally occur in adulthood, typically between the ages of 20 and 50 years of age, as in the present case that was referred to us with a diagnosis of an undifferentiated sarcoma [7] . They may be associated with multiple neurofibromatosis or occur de novo [1], as in our case. "
    [Show abstract] [Hide abstract] ABSTRACT: A 45-year-old P3L3, referred to us with abdominopelvic mass for further management. Vaginal examination was suggestive of uterine mass. Magnetic resonance imaging. (MRI) of abdomen.pelvis disclosed a uterine mass with equivocal invasion of the fat plane with the sigmoid colon. Coelomic antigen. (CA) 125 was 120.2 U/ml. (normal range, 0-35 U/ml). On exploratory laparotomy entire pelvic cavity was filled with a mass that was seen arising from the uterus and involving the sigmoid colon. Hence, a total abdominal hysterectomy with bilateral salpingo-oopphorectomy. (TAH BSO) was performed, along with resection anastomosis of the rectosigmoid and excision of omental and pelvic peritoneal nodules. Histopathology and immunohistochemical analysis, including S100-P positivity confirmed diagnosis of a malignant peripheral nerve sheath tumor. (MPNST), with tumor deposits in the right parametrium, omentum, sigmoid colon, and pelvic peritoneum. This case is presented in view of its rarity and associated diagnostic and therapeutic implications. © 2015 Journal of Cancer Research and Therapeutics | Published by Wolters Kluwer - Medknow.
    Full-text · Article · Feb 2016
    • "Maintenance of excisional margins at the time of surgery has been associated with a significant reduction or prevention of local recurrence [5]. Although the optimal excisional margins have not been established, extended resection is the treatment of choice for malignant soft tissue tumors [2, 4, 5] . The efficacy of radiotherapy and chemotherapy has not yet been established. "
    [Show abstract] [Hide abstract] ABSTRACT: This report describes a patient with a rare huge epithelioid malignant peripheral nerve sheath tumor (MPNST) in the left axilla. A male in his 70s was admitted to our hospital for evaluation of a growing tumor in his left axilla. The tumor was solid and immovable. Examination of a biopsy specimen resulted in a diagnosis of epithelioid MPNST. Two weeks after the biopsy was performed, the tumor grew to 20 cm and became painful, and the patient was unable to feel pressure on his upper arm. Immediately before surgery to remove the tumor, computed tomography suggested the presence of lung metastases. The patient and his family were informed of his disease state, and they elected surgical treatment to ease the symptoms associated with tumor enlargement. Systemic metastases appeared soon after the surgery, and the patient died within 11 weeks. Comparative genomic hybridization (CGH) analysis showed that this tumor was chromosomally unstable, with impairments in gene expression.
    Full-text · Article · Sep 2015
  • [Show abstract] [Hide abstract] ABSTRACT: The malignant schwannoma is a neoplasia whose origin is given by peripheral nerve tissue. It rarely appears in the head and neck, and even less frequently in maxillofacial territory. Here is a case report of a male patient, 9 years old, with a rapidly growing mass, expansive growth, located in the body and right mandibular ramus diagnosed as a malignant neurosarcoma or malignant schwannoma of the mandible, with mandibular canal, mental canal and foramen, soft tissue and bone involvement of the region.
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