Search results for "Peripheral Nerve Sheath"
showing 6 items of 6 documents
Malignant Peripheral Nerve Sheath Tumor With Osseous Heterologous Differentiation in Uncommon Locations (Heart and Retropharynx)
2016
We report two cases of malignant peripheral nerve sheath tumor (MPNST) in an uncommon location (heart and retropharynx) both with divergent osseous heterologous differentiation. We present the pathological and immunohistochemical studies that confirmed the neurogenic origin. The histopathology of the tumor arising in the retropharynx showed a transition from a neurofibroma to MPNST, making this a new report of an MPNST arising from a plexiform neurofibroma without neurofibromatosis. Primary cardiac MPNST with osseous differentiation has never been reported before. In conclusion, the histology of MPNSTs is very heterogeneous, showing no specific diagnostic immunoprofile or genetic alteration…
Desmoplastic melanoma may mimic a cutaneous peripheral nerve sheath tumor: Report of 3 challenging cases
2017
Desmoplastic melanoma (DM) and cutaneous malignant peripheral nerve sheath tumors (MPNST) reveal histological and immunohistochemical similarities, including S100 positivity and negative staining for conventional melanocytic markers. We present 3 cases of cutaneous S100-positive spindle cell tumors in elderly patients, in which first findings led to initial misdiagnoses as cutaneous MPNST and benign peripheral sheath nerve tumor (neurofibroma). The identification of adjacent atypical melanocytic hyperplasia in the overlying skin along with tumor cell proliferation, also in the superficial dermis, the neurotropic component and the absence of any relationship between the tumor and a major ner…
Oral manifestations of Type I Neurofibromatosis in a family
2011
Neurofibroma is a benign peripheral nerve sheath tumor. It is one of the most frequent tumors of neural origin and its presence is one of the clinical criteria for the diagnosis of neurofibromatosis type I (NF-I). Neurofibromatosis type I is an autosomal dominantly inherited disease due to an alteration in the long arm of chromosome 17. About 50% of NF-I patients have no family history of the disease. NF-I patients have skin lesions (café au lait spots and neurofibromas) as well as bone malformations and central nervous system tumors. Diagnosis is based on a series of clinical criteria. NF-I presents with certain definite oral manifestations which confers to the dentists a major responsibil…
Malignant peripheral nerve sheath tumor of the maxila
2005
Summary We present a case of a malignant peripheral nerve sheath tumor [MPNST] of the maxilla corresponding to a 12 year old male who had a painful, ulcerated rapid growing tumor. Histology revealed the presence of a tumor consisting of fusiform cells with abundant mitosis, with negative cytokeratins, actin, desmin, myoglobin and factor VIII. In contrast, the protein S-100 was positive. MPNST was not associated with signs of Neurofibromatosis Type 1. The patient was surgically intervened and received radiotherapy, then died within 10 months posterior to surgical intervention after suffering recurrence.
Giant schwannoma of the foot: a case report and literature review
2017
A schwannoma is a rare, benign tumor originating from Schwann cells of peripheral nerve sheath. It commonly occurs in subjects between 20 and 50 years of age, and its malignant transformation is exceptional. While schwannomas usually affect the head and neck region, localization in the lower extremity is exceptionally rare, and even fewer cases have described schwannomas occurring in the foot. We report a case of a giant schwannoma of the foot diagnosed in a 65-year-old woman. A giant schwannoma of the foot is an extremely rare soft tissue tumor. MRI may allow an earlier diagnosis and provide valuable information about the size and possible bone invasion. This case report noted that a compl…
Teratoma with a malignant somatic component in pediatric patients: The Associazione Italiana Ematologia Oncologia Pediatrica (AIEOP) experience
2010
Background Teratoma with a malignant somatic component (TMSC) is rare but described in adults, whereas information on pediatric presentation is sparse. Procedure The Associazione Italiana Ematologia Oncologia Pediatrica identified 14 cases of TMSC. Clinical files and pathology specimens were reviewed. Results The series (9 female, 5 male) showed the following disease: testis (2), sacrococcygeal (3), ovary (3), retroperitoneum (3), mediastinum (2), and foot soft tissue (1). Distribution of the somatic component was: carcinoma (4), pancreatic neuroendocrine tumor (1), neuroblastoma (3), rhabdomyosarcoma (3), rhabdomyosarcoma plus liposarcoma, chondrosarcoma, neurogenic sarcoma (1), chondrosar…