Cases reported "Pharyngeal Neoplasms"

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1/6. Hyalinizing spindle cell tumor with giant rosettes--a soft tissue tumor with mesenchymal and neuroendocrine features. An immunohistochemical, ultrastructural, and cytogenetic analysis.

    CONTEXT: Hyalinizing spindle cell tumor with giant rosettes is a recently described biphasic neoplasm of soft tissues that shares mesenchymal and neuroendocrine features. Its morphologic structure is distinctive, with the presence of hyalinized paucicellular foci that are termed rosettes. The cells around the latter display positive immunoreactivity for neuroendocrine markers. The small number of cases described to date indicates that they tend to be localized in the extremities. OBJECTIVE: To describe the clinicopathologic features of 2 unusual cases of hyalinizing spindle cell tumor with giant rosettes. methods AND RESULTS: One tumor was located in the prestyloid parapharyngeal space and the second in the left thigh. Both tumors were well circumscribed and surrounded by a thin capsule-like fibrous band without infiltrating projections. The rosettes were embedded in a spindle cell proliferation. Immunohistochemical stains showed positive results for S100 protein, synaptophysin, CD57, protein gene product 9.5, and neuron-specific enolase exclusively in the cells palisading the rosettes. These markers were negative in the spindle cell portions of the tumor. The latter were immunoreactive for factor xiiia, vimentin, HAM56, collagen IV, and CD68. vimentin was the only marker shared by the rosette-forming cells and the spindle cells. Ultrastructurally, the rosette-forming cells contained neurosecretory granules. This study describes the first cytogenetic analysis in this type of tumor revealing 2 cell lines, both containing a balanced translocation between chromosomes 7 and 16. Follow-up of the patients at 16 and 8 months did not disclose evidence of recurrence. CONCLUSIONS: These 2 new cases increase the awareness of hyalinizing spindle cell tumor with giant rosettes and demonstrate that it is a spindle cell neoplasm of unique cytogenetic rearrangements composed of dendritic, histiocytic, and fibroblastic cells admixed with cells that have neuroendocrine differentiation.
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2/6. Extranodal follicular dendritic cell sarcoma of the head and neck region: three new cases, with a review of the literature.

    Extranodal follicular dendritic cell (FDC) sarcoma of the head and neck region is uncommon, with 16 well-documented cases previously reported (four in the tonsil, four in the pharynx, two in the palate, five in the soft tissue, and one in the thyroid). We here report an additional three cases of extranodal FDC sarcoma in the tonsil (two cases) and pharynx (one case). In these new cases, the neoplastic cells were arranged in diffuse, fascicular, and vaguely whorled growth patterns. A background lymphocytic infiltrate was sprinkled throughout the neoplasms, with focal prominent perivascular cuffing. Scattered multinucleated giant cells were present. Immunohistochemically, tumor cells were strongly and diffusely positive for follicular dendritic cell markers CD21 and CD35. Tumor cells were diffusely positive for fascin and negative for leukocyte common antigen, S-100 protein, cytokeratin, and Epstein-Barr virus (EBV) latent membrane protein-1 (EBV-LMP). EBV was also not detected in the tumor cells by in situ hybridization for EBV-encoded RNAs. FDC sarcomas are probably an underrecognized neoplasm, especially when they occur in extranodal sites in the head and neck region. Two of the three new cases we report were initially misdiagnosed, and five cases of extranodal FDC sarcoma in the head and neck region reported in the recent literature were initially misdiagnosed. Our aim is to complement the current understanding of this neoplasm and alert pathologists to this rare entity in this region to avoid misdiagnosis. Recognition of extranodal FDC sarcoma requires a high index of suspicion, but this tumor has numerous distinctive histological features that should bring the neoplasm into the differential diagnosis. Confirmatory immunohistochemical staining with follicular dendritic cell markers such as CD21 and/or CD35 is essential for the diagnosis. Correct characterization of this neoplasm is imperative given its potential for recurrence and metastasis.
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3/6. Recurring giant-cell granuloma at the site of previous radiation therapy.

    A 15-year-old girl presented with an aggressive giant-cell granuloma (GCG) of the maxilla with local invasiveness and bony destruction. The tumor recurred twice and attained a diameter of 6 cm. Previously, this patient had had two hematologic malignancies for which she had received therapeutic doses of radiation to the site where the GCG occurred. It is therefore possible that this tumor was radiation induced.
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4/6. Giant epignathus.

    The case here described is interesting because of its giant size and because it had the morphology of a head. Immediate removal was followed by uneventful recovery. The term "epignathi" has been used since the last century for teratoid tumors growing from the upper jaw or the roof of the pharynx. These tumors tend to cause obstruction to respiration.
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5/6. Life-threatening presentations of fibrovascular esophageal and hypopharyngeal polyps.

    Giant fibrovascular polyps of the esophagus and hypopharynx are benign tumors of the upper digestive tract. Although a rare cause of asphyxiation, laryngeal impaction by a regurgitated polyp of the esophagus may be the initial symptom that brings the patient to request medical attention. Two new cases of giant fibrovascular polyps with dramatic and potentially life-threatening presentations illustrate the unpredictable behavior of these unusual tumors. Both patients presented to the emergency center with a history of coughing and eructation followed by temporary airway obstruction that was relieved by clenching a regurgitated fleshy mass between the teeth. Diagnostic and therapeutic intervention requires aggressive airway management, radiographic and endoscopic evaluation, and definitive surgical treatment. tracheotomy was required in one patient, and successful endotracheal intubation provided satisfactory airway control in the second. esophagoscopy revealed the origin of both tumors to be near the cricopharyngeal muscle. Complete surgical excision was curative in both cases.
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6/6. Giant cell tumour of the retropharynx.

    AIMS: Giant cell tumours of soft tissues are usually of tenosynovial origin and are benign, slow growing neoplasms that have the capacity for local recurrence. They occur most frequently in the fingers and only rarely in the head and neck region. Their occurrence in the retropharynx has not been reported. The differential diagnoses of head and neck lesions containing giant cells are discussed. methods AND RESULTS: We describe a case of a retropharyngeal giant cell tumour of tenosynovial type in a 48-year-old man. The clinical, radiological and histological features are reviewed. Immunohistochemical studies point to a possible histiocytic origin based on reactivity for CD68. CONCLUSIONS: Tenosynovial giant cell tumour should be considered in the differential diagnosis of tumours arising in the retropharyngeal soft tissues.
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