Cases reported "Neurofibroma"

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1/9. Presacral solitary giant neurofibroma without neurofibromatosis type 1 presenting as pelvic mass--case report.

    A 35-year-old woman presented with a solitary neurofibroma in an unusual presacral location without neurofibromatosis manifesting as bilateral chronic sciatica for 2 years. She was initially considered as having a giant right ovarian mass, but was referred with a prediagnosis of solitary giant sacral nerve sheath tumor. The initial differential diagnosis was based on neuroimaging. A right-sided J incision with the extraperitoneal approach provided good exposure and handling of the tumor bed. Almost total excision without neurological deficit was possible. The histological diagnosis was neurofibroma. Benign retroperitoneal neural sheath tumors in patients without von Recklinghausen's disease are quite rare. Intrapelvic tumors are often diagnosed at a later stage. neuroimaging is very helpful to delineate this unusual site and the extent of tumor development, and to determine the appropriate surgical intervention. A clear understanding of retroperitoneal anatomy is essential for safe removal of such tumors. Complete resection is preferred to prevent local recurrence and malignant transformation. Although root section is inevitable, neurological deficit is unlikely.
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2/9. Intradermal spindle cell/pleomorphic lipoma of the vulva: case report and review of the literature.

    BACKGROUND: Spindle cell/pleomorphic lipoma (SC/PL) is a benign adipose tissue tumor that usually affects the subcutaneous tissues of shoulders, backs, and neck region of middle-aged male patients. Histologically, it is characterized by the presence of primitive CD34-positive spindle cells arranged in short fascicles, bizarre floret-like multinucleated giant cells, mature adipocytes, and a small number of lipoblasts. Recently, an intradermal subset has been described, which mainly affects female patients and presents a wider antomical distribution when compared to the classical variant of SC/PL. methods: We report a case of intradermal SC/PL affecting the labium majus of a 56-year-old female patient. RESULTS: The histological examination disclosed the typical histological features, however the lesion showed poorly demarcated and infiltrative borders, as well as involvement of dermal nerves. The immunohistochemical analysis according to streptovidin-biotin-peroxidase technique showed immunoreactivity for CD34 and vimentin in the spindle cells, as well as S100 protein and vimentin in the adipocytic cells. CONCLUSIONS: To the best of our knowledge, this is the first case of intradermal SC/PL affecting the vulvar region. Care must be taken not to misdiagnosis this rare tumor as well-differentiated liposarcoma, cellular angiofibroma, solitary fibrous tumor, and cutaneous neurofibroma.
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3/9. Bulky naevocytoma of the perineum: a singular variant of congenital giant pigmented naevus.

    We describe two instances of a previously unrecognized variant of congenital giant pigmented naevus (GPN), presenting as a bulky naevocytic tumour in the perineal region. In both cases the lesion was present at birth and attained massive dimensions. In addition to the characteristic histological patterns found in GPN, which included extensive areas with a neural appearance, these tumours presented an uncommon tendency to form pseudo-follicular structures lined by naevus cells. No features suggestive of malignant transformation were found. Because GPN may associate with an underlying malignancy, accurate diagnosis of this lesion is important in clinical practice.
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4/9. Giant cell fibroblastoma recurring as dermatofibrosarcoma protuberans.

    A case of giant cell fibroblastoma in an 8-year-old child recurred locally 15 years later with the typical picture of dermatofibrosarcoma protuberans. This sequence of events suggests that giant cell fibroblastoma is a juvenile form of dermatofibrosarcoma protuberans.
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5/9. Giant neurofibroma of the pectoral region including the breast. Case report.

    A case of a 56 years old female patient with a giant tumour of the left pectoral region developed in a 2 years time was reported. The tumour, free from skin and pectoral muscles, was after mammectomy 25 x 15 x 5 cm, 2.200 grs, multilobaed, albescent with hemorrhages zones and predominantly hard, the rest of the breast being extremely reduced in comparison with the volume of the tumour itself. The initial histological aspect was that of a benign spindle cell tumour and only after some neurohistological techniques the diagnosis established was neurofibroma. The literature data and the exceptional mammary localization of this tumour were reviewed.
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6/9. Giant pigmented tumour of the scalp--a diffuse neurofibroma or a congenital naevus showing neurofibromatous changes? Immunohistochemical and electron microscopic studies.

    A case of giant pigmented tumour of the scalp which developed in a 47-year-old woman is reported. Macroscopically, the tumour showed a peculiar two-layered structure, consisting of an upper non-pigmented and a lower pigmented portion. Histologically, it was composed of elongated neurofibromatous tumour cells with abundant collagen fibres in the non-pigmented portion and round naevus-like cells with abundant melanin pigment in the pigmented portion. S-100 protein and neurone-specific enolase were demonstrated in most of the tumour cells, but neurofilament and myelin basic protein were not detected. Electron microscopy revealed melanosomes in the tumour cells of the pigmented portion. These findings might support a melanocytic origin for the tumour, but the lack of superficial pigmentation and the associated hair loss were against this. The tumour may represent an example of duality of neural crest differentiation.
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7/9. Neurosarcomatous malignant melanoma arising in a neuroid giant congenital melanocytic nevus.

    We report a case of neuroid giant congenital melanocytic nevus (GCMN) in which a malignant schwannomalike tumor developed. literature review reveals that neurosarcomatous differentiation occurs among malignant tumors arising in GCMNs, apparently with greater incidence in those GCMNs showing benign neuroid differentiation. Although the differences between neuroid melanocytes and Schwann's cells may be more conceptual than real, we believe that the current tumor arising within a melanocytic nevus is likely of neuroid melanocytic origin and best designated as neurosarcomatous malignant melanoma.
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8/9. Giant neurofibromas of the labia.

    Two cases of giant neurofibroma of the vulva are described. The finding of a solitary neurofibroma in a patient who shows no other stigmata of multiple neurofibromatosis is unusual. Also unusual was the exceptional size of these tumors.
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9/9. A case of giant bathing trunk naevus with neurofibroma-like change.

    It is recognized that cells of melanocytic naevi may resemble neuroid structures and the term neurotization has been used to describe this phenomenon. This may result in difficulty in the histological differentiation between naevi and neurofibromas. We report the case of a patient with a giant bathing trunk naevus which has undergone marked neurotization and in whom there had been diagnostic difficulty.
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