1/32. A rapidly growing benign intrathoracic neurofibroma after lung lobectomy.A 67-year-old male underwent a right upper lung lobectomy for lung cancer in January 1993. Follow-up chest x-rays revealed a progressive and rapidly growing intrathoracic mass in the right thorax. The mass, however, did not resemble a tumor recurrence, and the patient complained only of shortness of breath. Computerized tomography and magnetic resonance imaging confirmed the presence of the intrathoracic mass and its associated compression of the residual lung. A right thoracotomy was performed in January 1998, and a mass found arising from the sympathetic nerve trunk was resected. Microscopic examination revealed stellate or spindle-shaped cells in myxoid stroma with sparsely distributed collagen fibers. Immunohistochemically, the cells were positive for neuron-specific enolase, and the tumor was identified as neurofibroma. The patient did not suffer from von Recklinghausen's disease, and there was no family history of the disease. After resection of the neurofibroma, the compressed lung was able to re-expand, and the patient's shortness of breath disappeared. At one year postoperative, the patient remains well, and there is no evidence of recurrence.- - - - - - - - - - ranking = 1keywords = spindle (Clic here for more details about this article) |
2/32. Pigmented neurofibroma: report of two cases and literature review.Two cases of pigmented neurofibroma of the skin are reported. In case 1, the tumor was removed from the back of a 55-year-old man with no associated neurofibromatosis. In case 2, the tumor was removed from the abdominal wall of a 21-year-old woman with neurofibromatosis. Both tumors consisted of benign, short spindle cells and multiple foci of scattered melanin-laden cells. In case 1, the spindle cells were arranged in a storiform pattern, resembling features of dermatofibrosarcoma protuberans. Immunohistochemically, the spindle cells of both cases were demonstrated to be positive for S-100 protein and CD34. The melanin-laden cells stained positively for HMB-45. This report describes an additional two cases of pigmented neurofibroma that conform to the new diagnostic criteria for this disease.- - - - - - - - - - ranking = 13.794453901404keywords = spindle cell, spindle (Clic here for more details about this article) |
3/32. 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.- - - - - - - - - - ranking = 27.588907802807keywords = spindle cell, spindle (Clic here for more details about this article) |
4/32. Solitary neurofibroma of the oral mucosa: a previously undescribed variant of neurofibroma.We report a distinct morphologic type of neurofibroma, lipomatous neurofibroma, arising in the oral mucosa, which has not been described previously in the literature. A 25-year-old female patient presented with a solitary mucosal mass on the palatal gingiva. Although the limited biopsy material was diagnosed as a spindle cell lipoma, characteristic light microscopic neurofibromatous areas, intricately admixed with mature fat, were found in the entire resection specimen. Immunohistochemically, many of the spindle cells were positive, either diffusely or focally, for common neural markers, with patchy staining for CD34 and epithelial membrane antigen. S-100 protein was also positive in adipocytes. Ultrastructural examination confirmed the diagnosis of neurofibroma and suggested an intimate relationship between neoplastic neural cells and adipocytes.- - - - - - - - - - ranking = 9.1963026009358keywords = spindle cell, spindle (Clic here for more details about this article) |
5/32. Pediatric pigmented dermatofibrosarcoma protuberans (Bednar tumor): case report and review of the literature with emphasis on the differential diagnosis.dermatofibrosarcoma protuberans (DFSP) is a fibrous tumor of intermediate malignant potential that usually affects the trunk of young to middle-aged adults. On histological examination, it is characterized by a monomorphous population of spindle cells arranged in a storiform or cartwheel pattern. Bednar tumor (BT), formerly known as storiform pigmented neurofibroma, is currently considered the pigmented variant of DFSP due to the histological and cytogenetic similarities between these two lesions. There are very few reports on BT affecting pediatric patients. We describe a case of BT affecting the dorsal aspect of the left forearm of a 6-year-old-male patient and emphasize the diagnostic clues to distinguish this unusual cutaneous neoplasm from other pigmented lesions, including pigmented (melanotic) neurofibroma (PMN), psammomatous melanotic schwannoma (PMS), neurocristic cutaneous hamartoma (NCH), and desmoplastic malignant melanoma (DMM). We would like to stress that surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of BT as there is the risk of misdiagnosing it either as pigmented tumors associated with neurocutaneous syndromes, such as PMN and PMS, or as a highly malignant melanocytic neoplasm (DMM).- - - - - - - - - - ranking = 4.5981513004679keywords = spindle cell, spindle (Clic here for more details about this article) |
6/32. A corneal diffuse neurofibroma as a manifestation of von recklinghausen disease.PURPOSE: To report a case of a primary corneal diffuse neurofibroma in a patient with von Recklinghausen disease (NF-1). methods: Case report. A physical examination and histopathology were performed. The immunohistochemical studies were performed using an avidin-biotin-peroxidase complex technique on formalin-fixed and paraffin-embedded tissue. Histologic sections from corneal tissue were incubated with primary antibodies against vimentin and S-100 protein. A complementary ultrastructural study of the same formalin-fixed and paraffin-embedded tissue was made. RESULTS: The ophthalmologic examination revealed a yellowish-white elevated mass that involved the supratemporal cornea but not the limbus. Histologic study showed a tumor of the peripheral nerve sheath, a diffuse neurofibroma in the corneal stroma, and proliferation of spindle cells with markedly elongated nuclei. Cells comprising the tumor reacted with vimentin and S-100 protein, and the ultrastructural studies revealed myelinated nerve fibers confirming the diagnosis. CONCLUSION: The development of a primary diffuse neurofibroma in the cornea of patients with von Recklinghausen disease is possible. The present case supports the statement that neurofibromas arising from the peripheral nerve sheath may involve any part of the body.- - - - - - - - - - ranking = 4.5981513004679keywords = spindle cell, spindle (Clic here for more details about this article) |
7/32. 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.- - - - - - - - - - ranking = 9.8677106493234keywords = naevus (Clic here for more details about this article) |
8/32. Metastatic acinic cell carcinoma in a neurofibroma mistaken for carcinosarcoma.BACKGROUND: Tumor-to-tumor metastasis is a rare, but well-recognized, entity most commonly involving metastatic carcinoma to a mesenchymal neoplasm. We report a case of acinic cell carcinoma of the parotid gland metastatic to a neurofibroma. methods AND RESULTS: A 55-year-old man with a history of a high-grade acinic cell carcinoma of the parotid was seen with a mass at the surgical site and metastatic foci in the scalp 10 months postoperatively. The resection specimen revealed a spindle cell lesion with metastatic foci of high-grade adenocarcinoma, initially diagnosed as a carcinosarcoma. The bland morphology and S-100-positive expression of the spindle cell lesion confirmed the diagnosis of neurofibroma. The high-grade features of the carcinomatous foci and their similarity to the primary tumor confirmed the presence of a tumor-to-tumor metastasis. CONCLUSION: To our knowledge, this is the first reported case of acinic cell carcinoma metastatic to a neurofibroma, an important entity in the differential diagnosis of biphasic tumors of the head and neck.- - - - - - - - - - ranking = 9.1963026009358keywords = spindle cell, spindle (Clic here for more details about this article) |
9/32. A solitary laryngeal neurofibroma ina pediatric patient.A neurofibroma of the larynx is a rare disease that usually involves the arytenoids and the aryepiglottic fold. Pediatric patients with a laryngeal neurofibroma often present with progressive dyspnea. A definitive diagnosis of a laryngeal neurofibroma is based on the histopathologic demonstration of the characteristic spindle cells, and a positive result in immunohistochemical staining for S-100 protein. Tumor excision of laryngeal neurofibromas by an external approach was formerly common. We present a case of a solitary laryngeal neurofibroma in a 4-year-old child that was completely excised using direct laryngoscopy and a CO2 laser. There has been no recurrence after a follow-up of 4 years. For pediatric patients with an isolated laryngeal neurofibroma, endoscopic surgery provides an alternative approach due to its safety, effectiveness, and minimal invasiveness.- - - - - - - - - - ranking = 4.5981513004679keywords = spindle cell, spindle (Clic here for more details about this article) |
10/32. Unusual rapidly growing gastric myxoid neurofibroma: a case report.A 62-year-old man was first found to have a gastric tumor in the fornix during a routine health examination 16 months previously. The case was not associated with von Recklinghausen's disease. Initially, the tumor was approximately 4 mm in diameter, but grew very rapidly to 20 mm in 13 months, and to 30 mm in the following month, without manifesting clinical symptoms. Malignancy was suspected because of the rapid growth and the endoscopic findings: a rough-granular elevated lesion with a wide stalk, and with erosions and mucus on its surface. Although endoscopic biopsy was done several times, the routine histological examinations were inconclusive. Endoscopic resection was therefore carried out. Examination of the resected specimen showed that the main elements consisted of wavy, long-spindled cells, which crossed irregularly, and whose interstitium was myxomatous. Immunohistochemically the specimen showed strongly positive reaction for S-100 protein and positive reaction for neuron specific enolase (NSE). Based on these immunohistochemical findings, a final diagnosis of gastric myxoid neurofibroma was made, which showed rapid growth due to myxomatous change. The patient showed a good postoperative course and no recurrence has been recognized by follow-up endoscopy.- - - - - - - - - - ranking = 1keywords = spindle (Clic here for more details about this article) |
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