1/1631. Paraneoplastic pemphigus associated with follicular dendritic cell sarcoma arising from Castleman's tumor. Paraneoplastic pemphigus (PNP) is associated with various forms of underlying neoplasia. We report a patient with PNP associated with an intraabdominal follicular dendritic cell sarcoma that developed from a Castleman's tumor that had preexisted for 40 years. ( info) |
2/1631. MR imaging of complex tail-gut cysts. Retrorectal-cyst hamartomas (RCH) are rare developmental tail-gut cystic tumours of the retrorectal space, which occasionally undergo malignant transformation. We describe the magnetic resonance imaging (MRI) findings in two patients with RCH and in a third patient with unclassified sarcoma arising from a RCH. The RCH were hypointense or hyperintense on T1-weighted images and hyperintense on T2-weighted images; they did not enhance and they contained multiple septations. A solid component in the periphery of one cyst was markedly hypointense on T2-weighted images in keeping with fibrous material. The sarcoma arising from the wall of the RCH enhanced and was of intermediate signal intensity on all sequences. MR may help establish the diagnosis of RCH if an unenhanced cystic tumour is discovered in the retrorectal space and it can help detect those rare cases of malignant transformation of these developmental tumours. ( info) |
3/1631. Extranodal follicular dendritic cell sarcoma of the palate. Follicular dendritic cell tumors are uncommon and usually occur in lymph nodes. We report the case of a follicular dendritic cell tumor that occurred in the palate of a 14-year-old boy and manifested itself as a nodular mass. Histologically, the neoplasm consisted of spindle-shaped or oval-shaped cells with eosinophilic cytoplasms and nuclei with delicate, dispersed chromatin. The lesional cells were principally arranged in diffuse, fascicular patterns with vaguely whorled or storiform areas. Focal multinucleate tumor giant cells and lymphocytes were observed throughout the neoplasm. Immunohistochemically, tumor cells were positive for the follicular dendritic cell markers CD21, CD35, and CD23 and for S-100 protein, CD68, and muscle-specific actin. Tumor cells were negative for LCA, CD20, EMA, CK (AE1/AE3), HMB45, and CD34. lymphocytes were positive for LCA and CD45RO. Although follicular dendritic cell sarcoma is a very uncommon tumor, it should be included in the differential diagnosis of tumors in this location. ( info) |
4/1631. Epithelioid sarcoma of the vulva. We report a case of a 23-year-old woman diagnosed as having an epithelioid sarcoma of the vulva. She was treated by a clitoris-sparing hemivulvectomy and lymph node sampling of the ipsilateral groin. Vulvar reconstruction was performed with a rectus abdominis myocutaneous flap. Four years after the operation there is no evidence of disease and the patient has a normal sex life. The English literature on this subject is reviewed with special attention to the biological behavior and therapeutic approach. ( info) |
5/1631. Histochemical investigation into the molecular mechanisms of malignant transformation in a benign glomus tumour. A glomangiosarcoma arose in a benign glomus tumour. The histological and immunohistochemical characteristics of the tumour were investigated. Apoptotic cells were identified by terminal deoxynucleotidyl transferase (TdT) mediated dUTP-biotin nick end labelling (TUNEL). The proportion of apoptotic cells was found to be low and TUNEL positive nuclei were present in the benign part of the tumour. Bcl-2 protein, an inhibitor of apoptosis, was strongly expressed in the glomangiosarcoma with only weak staining in the benign area. The proliferation index of the glomangiosarcoma was almost 10-fold higher than that of the benign glomus tumour. Numerous nuclei in the glomangiosarcoma were intensely stained for the tumour suppressor protein p53. The results of the this study may contribute to an understanding of the molecular basis of malignant transformation in benign glomus tumours. ( info) |
6/1631. Myxoid variant of follicular dendritic cell sarcoma arising in the breast. Follicular dendritic cell sarcoma is a malignant tumor of the follicular dendritic cell which can arise in extranodal sites. We present here a case arising as a mass in the breast of a 41-year-old woman. The tumor was composed of mildly pleomorphic spindly cells with pale ovoid nuclei and cell processes intimately admixed with mature lymphocytes. In much of the lesion the cells were dispersed in cords in a myxoid stroma, and elsewhere there were solid sheets. The neoplastic cells were immunoreactive for CD21, CD35, EMA, and S100 protein, but not for other lymphoid markers or cytokeratin. Electron microscopy showed interdigitating cytoplasmic processes with junctions but no external lamina. The differential diagnosis includes carcinoma, lymphomas, and a variety of myxoid sarcomas. The tumor recurred within a few months and displayed increased nuclear pleomorphism and lymphatic invasion but the patient appears free of disease 3 years after the further excision. This case extends the spectrum of follicular dendritic cell sarcoma in soft tissue sites. ( info) |
7/1631. Delay in the diagnosis and treatment of primary bone sarcoma of the pelvis. BACKGROUND: Symptoms arising from primary bone sarcoma of the pelvic girdle are often insidious in onset and nonspecific in nature. To make the subtle initial signs and symptoms of these tumors more apparent to clinicians, we studied a cohort of patients who had a primary bone sarcoma of the pelvic girdle. Our purpose was to describe the initial clinical findings and to evaluate the duration, frequency, and implications of delays in the treatment of these tumors. methods: We retrospectively analyzed the data on sixty-eight patients who had a primary bone sarcoma of the pelvic girdle. The data that we reviewed included demographic characteristics; histological diagnosis; anatomical location, size, and stage of the tumor; characteristics of the biopsy specimen; duration and description of symptoms before an accurate diagnosis was made; delay before recognition of the tumor on radiographs; results of diagnostic imaging; inaccurate diagnoses; type of intervention based on these inaccurate diagnoses; and outcome with regard to survival. There were forty high-grade sarcomas and twenty-eight low-grade sarcomas. RESULTS: Excluding two asymptomatic patients in whom the sarcoma was noted incidentally, the average duration of symptoms before an accurate diagnosis was made was ten months (median, six months; range, one month to four years). Common symptoms and findings on physical examination included pain in the buttock (twenty-three patients; 35 percent), a mass (twenty patients; 30 percent), sciatica (nineteen patients; 29 percent), pain in the hip (seventeen patients; 26 percent), pain in the groin (thirteen patients; 20 percent), and low-back pain (fourteen patients; 21 percent). In thirty (44 percent) of the sixty-eight patients, the pelvic sarcoma was not recognized initially and an inaccurate diagnosis was made. The misdiagnoses included a herniated lumbar disc, spinal stenosis, spondylolisthesis, tendinitis, bursitis, an inguinal hernia, a stress fracture, a pilonidal cyst, a recurrent urinary-tract infection, and degenerative arthritis of the spine, hip, and knee. Inappropriate treatment for these misdiagnoses included seven operative procedures (two laminectomies, two debridements, one hip arthrotomy, one total knee replacement, and one inguinal herniorrhaphy), six courses of nonsteroidal anti-inflammatory medications, five chiropractic adjustments, four trials of physical therapy, and three local injections of steroids. It took an average of seven months for the clinicians to arrive at the diagnosis of primary pelvic sarcoma. With the numbers available, no significant association between the duration of symptoms before an accurate diagnosis was made and the grade or the stage of the tumor could be detected. In addition, no association between the duration of symptoms and the survival of the patient (p = 0.54) could be determined, with univariate analysis. The grade and the stage of the tumor were strongly associated with the outcome, with a low tumor grade proving to be a favorable prognostic indicator for survival (p = 0.006). CONCLUSIONS: patients who have a primary bone sarcoma of the pelvis often have had symptoms for a long duration that mimic those of more commonly encountered non-neoplastic musculoskeletal conditions. When a patient has symptoms that appear to be out of the ordinary, particularly refractory pain or pain at rest, physicians should include the pelvic girdle in the evaluation and should carefully examine a high-quality radiograph of the entire pelvis. ( info) |
8/1631. Low-grade fibromyxoid sarcoma: clinicopathologic case report with review of the literature. Low-grade fibromyxoid sarcoma is a rare, benign-appearing soft tissue neoplasm with an aggressive clinical course characterized by multiple local recurrences over several years, with ultimate spread to lung and occasionally to bone. Thus far, a total of 24 cases of low-grade fibromyxoid sarcoma have been reported in the literature. The authors present an additional case that grossly and microscopically emphasizes a pronounced lobular pattern of contrasting areas of cellularity showing high proliferative activity, as demonstrated by a proliferation marker, Ki 67 with MIB-1, and hypocellular areas with prominent myxoid component and abundant collagen fibrils. There was predominance of delicate capillary-sized stromal vessels with collagenized walls in both cellular and myxoid areas. The unusual features in this case were osseous metaplasia, prominent intranuclear pseudoinclusions, dna tetraploidy, and membrane-bound intracytoplasmic fat vacuoles. The immunoprofile and cytologic and ultrastructural features are described. After the excision of the tumor, the patient was treated with radiotherapy without chemotherapy. The patient has been observed for 26 months and is alive without the evidence of disease. The postoperative follow-up with axial computed tomography at 24 months showed no evidence of disease, except postsurgical fibrotic changes. ( info) |
9/1631. Molecular assessment of clonality leads to the identification of a new germ line TP53 mutation associated with malignant cystosarcoma phyllodes and soft tissue sarcoma. Cystosarcoma phyllodes (CSP) is a rare breast neoplasm composed of stromal and epithelial elements. It usually runs a benign course but it may metastasize. In a 31-year-old patient with recurring CSP, a mesenchymal tumor in the leg developed. The question arose whether the latter tumor could be a metastasis from the CSP, which would have major treatment consequences. The problem was addressed using molecular methods, i.e., comparison of the pattern of polymorphic repeat markers on chromosome 17p as well as single strand conformation polymorphism analysis and sequencing of exons 5 to 8 of the TP53 gene in both tumor and normal tissue. An identical pattern of loss of heterozygosity in both breast tumors was demonstrated, but a different pattern was shown in the tumor in the leg. This led to the conclusion that the latter tumor had to be a new primary tumor. A mutation in codon 162 of the TP53 gene was found in the tumor tissue as well as in the normal tissue of this patient. This germ line mutation leads to the replacement of isoleucine by asparagine and most likely has functional consequences. In all four examined tumors of this patient, the normal TP53 allele was lost. This is strong evidence that this germ line TP53 mutation causes the genesis of these two rare primary mesenchymal tumors in this young patient. The current study exemplifies the power of molecular diagnostic methods in investigating the specific clinical problem of clonal relation between two separate tumors. The germ line mutation found in codon 162 of the TP53 gene and the association with cystosarcoma phyllodes have not been described previously. ( info) |
10/1631. 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. ( info) |