Cases reported "Sarcoma"

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1/63. Dendritic cell sarcoma of the thyroid.

    BACKGROUND: Follicular dendritic cell sarcoma (FDCS) arises from nonlymphatic antigen-presenting cells found in lymph node B-cell follicles. This extremely rare tumor, which usually arises in lymph nodes, does occur in extranodal head and neck sites such as the tonsil and soft palate. methods: A retrospective review of the patient followed at the University of Pittsburgh Medical Center from 1993 to the present was performed. CONCLUSIONS: This is the first reported case of an FDCS of the thyroid. A review of the literature provides useful information to aid in detection, treatment, and outcome of this unusual soft tissue malignancy.
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2/63. Primary epithelioid sarcoma of the scalp.

    This article retrospectively reviewed a case of epithelioid sarcoma of the scalp; a treatment plan for this type of neoplasm has not been well defined in the literature because of the rarity of sarcomas in general and sarcomas located in the head and neck in particular. No comparative results can be drawn when dealing with such lesion in the scalp. Early recognition with an aggressive approach to confirm the existence of an epithelioid sarcoma is mandatory; a high index of diagnostic awareness is needed to recognize this uncommon tumor. early diagnosis can only be auspicious. Early wide surgical resection is imperative to ensure better control of imperceptible tumor extension, and well-planned diagnostic and therapeutic intervention, rather than isolated management and referral for adjunctive treatment, is important.
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3/63. Follicular dendritic cell sarcoma of the neck: report of two cases complicated by pulmonary metastases.

    BACKGROUND: Follicular dendritic cell (FDC) sarcoma is an uncommon neoplasm occurring primarily in lymph nodes but also in extranodal sites. A correct diagnosis can be difficult to make, especially in the latter sites. methods: Two patients with FDC sarcoma of the cervical soft tissues that metastasized to the lungs are reported. Both were initially misdiagnosed as having CASTLE (carcinoma showing a thymus-like element). Additional immunohistochemical stains were performed. RESULTS: The primary tumors showed jigsaw puzzle-like lobulation resembling thymic epithelial tumor and consisted of spindly cells arranged in fascicles, whorls, and a storiform pattern. The spindly cells had indistinct cell borders, vesicular nuclei, and distinct nucleoli. Perivascular spaces were present. lymphocytes were sprinkled throughout the tumor in one case but were sparse in the other. The metastatic deposits in the lungs appeared 27 and 2 years, respectively, after the initial presentation and were histologically similar to the original tumors. The FDC nature of the primary and metastatic tumors was confirmed by positive staining with CD21/CD35 cocktail and CD23 and by negative staining for cytokeratin. In one case, in direct continuity with the main tumor, there was a lobulated lesion composed of small lymphocytes punctuated by large cells with vesicular nuclei, histologically reminiscent of thymoma. The large cells were shown by immunohistochemistry to represent FDCs forming complex interconnecting meshworks. It is unclear whether this contiguous mass represents a precursor lesion or an unusual-looking component of the neoplasm. CONCLUSIONS: FDC sarcoma can look deceptively like a thymic epithelial tumor histologically. A correct diagnosis requires a high index of suspicion and immunohistochemical evaluation. The tumor shows a propensity to metastasize to the lungs, which can be delayed until more than 20 years after initial presentation.
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4/63. Myxoid liposarcoma of the oral cavity with involvement of the periodontal tissues.

    BACKGROUND, AIMS: liposarcoma is the 2nd most frequent soft tissue sarcoma in adults, but it is extremely rare in the head and neck and, particularly, in the oral cavity. We report on a 25-year-old female who presented with a periodontal mass, extended from the right upper 3rd molar to the right upper 2nd premolar, covered by intact oral mucosa. The clinical differential diagnosis included peripheral giant cell granuloma, salivary gland neoplasms, squamous cell carcinoma of the gingiva, sarcoma and malignant lymphoma. methods: To accurately plan subsequent treatment, an excisional biopsy was performed and a myxoid liposarcoma was diagnosed. Consequently, the patient underwent wide excision of the neoplasm with maxillary en-block resection. RESULTS: The post-operative course was uneventful and the patient is alive and well 8 years after the original diagnosis. The authors stress the importance of considering soft tissue sarcomas in the diagnostic approach to patients with unusual periodontal neoplasms and to plan adequate surgical sampling of the lesion (i.e. excisional biopsy). CONCLUSIONS: This appears of pivotal importance as more limited specimens may result in inaccurate pre-operative diagnosis.
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5/63. Sarcoma of the thyroid region mimicking Riedel's thyroiditis.

    Because sarcomas of the anterior lower neck region occur so infrequently, they are not usually considered in the differential diagnosis of Riedel's thyroiditis. Riedel's thyroiditis itself may be confused on clinical grounds alone with malignant neoplasms because of its invasive features. Sarcomatoid carcinoma is the main entity to be discarded in this regard. This is accomplished through histological examination by the finding of carcinomatous areas and/or reactivity with epithelial markers. These features also set apart sarcomatoid carcinoma from true sarcomas. This report concerns a patient with a sarcoma of the anterior lower neck region which was initially suspected to be Riedel's thyroiditis or sarcomatoid carcinoma on clinical and radiological grounds. A peroperative biopsy was interpreted by two independent pathologists as consistent with Riedel's thyroiditis. The subsequent clinical course and postmortem examination demonstrated a high grade sarcoma with metastasis to both lungs and the pleura, and invasion of adjacent neck structures. Nevertheless, some areas of the postmortem material showed a microscopic pattern similar to mediastinal fibrosis, raising the possibility of the malignant transformation of a fibrosclerotic lesion.
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6/63. Malignant triton tumor of the nasal cavity.

    BACKGROUND: Malignant triton tumor is usually an aggressive sarcoma consisting of a malignant schwannoma with rhabdomyoblastic differentiation. However, malignant triton tumor of the nasal cavity is very rare. methods: A case report of a 38-year-old woman with polypoid mass, which bled easily in the right nasal cavity, and nasal obstruction is presented with a review of the literature pertaining to this unusual case. RESULTS: The malignant triton tumor was treated with wide surgical excision followed by radiation therapy. Histopathological diagnosis of the malignant triton tumor was made on the surgical specimen. The patient's postoperative course was unremarkable, and follow-up at 5 years reveals no evidence of disease. CONCLUSIONS: A malignant triton tumor in the nasal cavity is a rare disease. head and neck surgeons should recognize the possibility of malignant triton tumor occurring in the nasal cavity.
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7/63. 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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keywords = neck
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8/63. Primary epithelioid sarcoma of the dura: case report.

    OBJECTIVE AND IMPORTANCE: Epithelioid sarcomas are rare mesenchymal neoplasms that occur most often in the extremities of young adults. Despite isolated reports of epithelioid sarcomas arising in the head and neck region, these lesions have not been described previously, to our knowledge, in the central nervous system. CLINICAL PRESENTATION: We present the case of an 18-year-old woman with a unique dural sarcoma that arose in the right frontotemporal region. As visualized on magnetic resonance imaging studies, the 4.5-cm tumor focally traversed the cranium to penetrate the galea, the temporal muscle, and subcutaneous tissue. No brain invasion was noted. INTERVENTION: Despite gross total removal and postoperative radiotherapy (59 Gy), a large recurrence was noted 5 months after surgery. Histologically, the partly necrotic tumor consisted of epithelioid and spindle cells showing widespread vimentin and variable cytokeratin as well as epithelial membrane antigen immunoreactivity. Ultrastructurally, the cohesive cells featured various organelles, intermediate filaments, junctions, and filopodia-containing intercellular spaces. CONCLUSION: With the inclusion of epithelioid sarcoma, the spectrum of central nervous system sarcomas continues to expand.
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9/63. Spindle cell lesions of the head and neck mimicking rhabdomyosarcoma in children.

    Malignancies of the head and neck are uncommon among children. The most common solid tumors to occur in this region are rhabdomyosarcoma, hodgkin disease, and non-Hodgkin lymphoma. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to have benign spindle cell lesions. These rare lesions should be part of the differential of children with a head and neck mass.
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10/63. Proliferative myositis: a rare pseudosarcoma of the chest wall.

    Proliferative myositis is a rare, inflammatory tumor that is often misdiagnosed as sarcoma. The clinical course of proliferative myositis is benign, and local recurrence after simple excision is uncommon. Typically, the lesion presents in the extremities or the head and neck. We present an unusual case of proliferative myositis with involvement of the anterior chest wall.
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