1/135. Cytology of ascitic fluid in a patient with granulocytic sarcoma (extramedullary myeloid tumor). A case report.BACKGROUND: Granulocytic sarcoma (GS) is the rare extramedullary manifestation of acute myeloid leukemia that may precede or be concurrent with leukemic infiltration of bone marrow or herald blastic transformation of a chronic myeloproliferative disorder. It has been found in most body sites and shows no age or sex predilection, necessitating its inclusion in the differential diagnosis of undifferentiated neoplasms. CASE: A 36-year-old female presented with a three-year history of abdominal pain, jaundice and fluctuating abdominal girth. Cytology of the ascitic fluid revealed myeloid cells of eosinophilic lineage at all stages of differentiation, with many undifferentiated cells. Immunohistochemical studies on a cell block confirmed the diagnosis of granulocytic sarcoma, which excluded the differential diagnoses of Hodgkin's disease, non-Hodgkin's lymphoma and Langerhans histiocytosis. CONCLUSION: Granulocytic sarcoma may present as a serous effusion and can be diagnosed on a cytologic specimen.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/135. The expanding clinical spectrum of desmoplastic small round-cell tumor: a report of two cases with molecular confirmation.desmoplastic small round cell tumor (DSRCT) is an aggressive neoplasm characterized by a consistent histological appearance, a unique immunohistochemical profile, and a specific chromosomal translocation. DSRCT is also hallmarked by distinctive clinical features. Most tumors arise in adolescent or young adult males, present as bulky abdominal masses, and diffusely spread along the peritoneal surface. We report two cases of DSRCT that do not fit this typical profile. One case involved the abdominal cavity of a 76-year-old woman. The other case arose in the parotid of a 22-year-old man. Histologically, the tumors showed the characteristic features of DSRCT. Immunohistochemically, the tumors showed positivity for cytokeratin, desmin, and neuron-specific enolase. Genetically, the tumors expressed the EWS-WT1 chimeric transcript. These two cases expand the differential diagnosis for poorly differentiated small-cell tumors that involve elderly patients or arise in the parotid. Moreover, they challenge the popular notion that DSRCT is a "blastomatous" tumor derived exclusively from the primitive mesothelium.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/135. Intra-abdominal embryonal rhabdomyosarcoma in an adult.rhabdomyosarcoma is an uncommon neoplasm in the adult population. Sporadic cases of primary rhabdomyosarcoma arising in the abdomen have been reported, but these cases are limited almost exclusively to the pediatric population. We report a well-documented case of primary intra-abdominal rhabdomyosarcoma in a 57-year-old woman. The patient presented with a pelvic mass and an elevated serum CA 125 and was referred to gynecologic oncologists at our institution for a presumed primary gynecologic malignancy. Intraoperatively, amorphous gelatinous tumor comprised a large portion of the peritoneal cavity. Surgical exploration of the abdomen failed to implicate any specific organ as the site of origin of the tumor. The overall histologic pattern of the resected tumor was most consistent with embryonal type rhabdomyosarcoma. To our knowledge this is the first well-documented case report of non-hepatobiliary, adult, intra-abdominal embryonal rhabdomyosarcoma in the English language literature. The presentation of a rare adult sarcoma mimicking a gynecologic malignancy was an unusual feature that complicated the diagnosis in this case.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/135. Fine needle aspiration cytology of desmoplastic small round cell tumor. A case report.BACKGROUND: Intraabdominal desmoplastic small round cell tumor (DSRCT) is a recently recognized type of primitive sarcoma characterized by a predilection for young males, a usually very aggressive course and generally unsuccessful therapy. A primitive histologic appearance with prominent desmoplasia and striking divergent multilineage differentiation are well-described morphologic features of this tumor, along with a consistent fusion of the EWS and WT1 genes at the molecular level. The cytologic literature contains only scattered references to this type of neoplasm. Detailed information on the clinical and fine needle aspiration (FNA) biopsy and the immunocytochemical and ultrastructural findings in a patient with DSRCT is presented. CASE REPORT: A 23-year-old male had a firm abdominal mass with multiple secondary lesions of the liver. An FNA biopsy was performed under ultrasonographic guidance. CONCLUSION: FNA of the liver nodules showed cohesive groups of small cells with hyperchromatic nuclei and inconspicuous nucleoli; immunocytochemically vimentin and desmin showed characteristic perinuclear globular positivity. FNA cytology is an effective means of diagnosing deeply located lesions. The cytologic features of DSRCT need to become familiar to pathologists and must be considered in the differential diagnosis of liver metastasis.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
5/135. Extraskeletal myxoid chondrosarcoma of the knee.Extraskeletal myxoid chondrosarcoma is an uncommon neoplasm, accounting for less than 2% of all soft tissue sarcomas. It affects adult males with a median age in the fifth decade at the time of diagnosis. The tumor usually arises in the deep soft tissues, especially in the lower extremities. patients present with a gradually enlarging mass that may or may not be associated with pain. This report describes a 25-year-old man who initially presented with a 4- to 5-year history of right knee pain and an enlarging mass in the right knee. Evaluation revealed a cartilaginous neoplasm with no evidence of metastatic disease. The tumor was widely excised and an allograft reconstruction was performed. The patient was closely followed with an eventual above the knee amputation for recurrent myxoid chondrosarcoma. At 34 months, retroperitoneal metastases were noted on abdominal CT. The patient underwent a left radical nephrectomy, renal vein thrombectomy and enucleation of the mass in the right kidney, distal pancreatectomy, and splenectomy. The patient received postoperative chemotherapy. Forty-eight months after initial diagnosis, the patient was found to have recurrent abdominal and retroperitoneal lesions. At 64 months, the patient died from complications of extraskeletal myxoid chondrosarcoma.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
6/135. Cytokeratin-negative desmoplastic small round cell tumor: a report of two cases emphasizing the utility of reverse transcriptase-polymerase chain reaction.desmoplastic small round cell tumor (DSRCT) is a unique, highly aggressive neoplasm that chiefly affects male adolescents and young adults. This tumor is characterized by nests of small undifferentiated cells that show immunohistochemical evidence of epithelial, mesenchymal, and neural differentiation. We report two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation, but were found to have the fusion transcripts characteristic of this tumor. Both patients (a 41-year-old male and a 31-year-old female) presented with large intra-abdominal masses. After diagnostic biopsy, both were treated with multi-agent chemotherapy. One patient expired 18 days after diagnosis, and the other is currently alive 28 months later. Histologically, both tumors had the characteristic features of DSRCT and were composed of small round cells with hyperchromatic nuclei and scanty cytoplasm. In one of the cases, perinuclear intracytoplasmic hyaline inclusions were seen. Immunohistochemically, neither case expressed any of the epithelial markers tested, including AE1/AE3, CAM 5.2 and EMA. Both tumors were diffusely immunoreactive for desmin with a prominent globoid "dot-like" pattern of staining in one case. Both tumors stained for vimentin, neuron specific enolase, and synaptophysin, but were negative for CD99, muscle-specific actin, and myogenin. Reverse transcriptase-polymerase chain reaction revealed EWS-WT1 fusion transcripts characteristic of this neoplasm. In conclusion, we describe two cases of DSRCT that lacked immunohistochemical evidence of epithelial differentiation but had histologic and other immunohistochemical features which suggested this diagnosis. The ability to confirm the diagnosis of this rare tumor using molecular genetic techniques is particularly useful in those cases with unusual histologic or immunophenotypic features.- - - - - - - - - - ranking = 2keywords = neoplasm (Clic here for more details about this article) |
7/135. Recurrent Epstein-Barr virus-associated post-transplant lymphoproliferative disorder: report of a patient with histologically similar but clonally distinct metachronous abdominal and brain lesions.A liver transplant patient developed a single central nervous system (CNS) intraparenchymal lesion 5 months after the diagnosis of an intraabdominal diffuse large B-cell post-transplant lymphoproliferative disorder (PTLD). biopsy of the new CNS lesion showed a diffuse large B-cell PTLD morphologically and immunohistochemically indistinguishable from the abdominal lesion. In addition, both lesions were positive for Epstein-Barr virus (EBV) dna by polymerase chain reaction (PCR) and for EBV-encoded rna by in situ hybridization. Although these results were consistent with a metastatic origin for the CNS lesion, the finding of an intraparenchymal lesion without leptomeningeal or dural spread was suggestive of a new primary CNS lymphoma. Proof that the brain lesion was a second primary and not a metastasis was obtained by immunoglobulin gene rearrangement studies and assessment of EBV clonality. Multiple primary lymphoid neoplasms arise at higher frequency in the setting of immunosuppression, and molecular investigations of tumor clonality can provide clinically relevant staging and prognostic information.- - - - - - - - - - ranking = 1.0128441839682keywords = neoplasm, central nervous system, nervous system (Clic here for more details about this article) |
8/135. Metastatic neuroblastoma with ganglioneuromatous differentiation and mandibular involvement.Neuroblastic tumors comprise neuroblastoma (NB), ganglioneuroblastoma, and ganglioneuroma (GN). NBs have shown remarkable differentiation capabilities, which include spontaneous regression. In the last 3 decades, the prognosis for metastatic NB has improved significantly. The ability to distinguish prognostic subtypes based on clinical and biologic features allows for understanding of this disease process and development of management procedures. NBs are often asymptomatic and remain undetected until a large abdominal mass or metastasis is found. GN, the most differentiated variant, is a benign neoplasm with significant growth potential and ability to cause clinical complications. GN of the mandible has seldom been reported in the literature in English. We present a case of NB metastatic to the mandible exhibiting ganglioneuromatous differentiation. We discuss the current understanding of the biology, grading, classification, and prognostic implications of NB.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/135. Angiomatoid (malignant) fibrous histiocytoma as a second tumour in a child with neuroblastoma.Neuroblastoma occurring as a disseminated disease in children has a poor prognosis. Haematogenous metastases usually involve the marrow, bone, liver and skin. A second neoplasm may also develop. We describe a child with retroperitoneal neuroblastoma (stage 3) who developed a nodular mass in the inguinal area which was suspected to be a metastasis. Histopathology disclosed an angiomatoid (malignant) fibrous histiocytoma, and excision was curative. The occurrence of angiomatoid (malignant) fibrous histiocytoma as a second tumour in a patient with neuroblastoma has not previously been reported.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/135. Renal angiomyolipoma with epithelioid sarcomatous transformation and metastases: demonstration of the same genetic defects in the primary and metastatic lesions.angiomyolipoma (AML) is a benign neoplasm that occurs either sporadically or in patients with tuberous sclerosis complex (TSC) and shows frequent allelic losses at chromosome arm 16p. It has been suggested recently that the melanogenesis marker-positive perivascular epithelioid cell (PEC) has been found consistently in AML. The authors report a 50-year-old woman without evidence of TSC affected by classic renal AML containing an area composed of atypical epithelioid cells with the same morphoimmunophenotypic characters of PEC. After 7 years from surgical removal of the lesion, the patient developed a local recurrence and successive lung and abdominal metastases that showed morphologic and immunohistochemical features overlapping those of the epithelioid area of the previously removed AML. Genetic analysis showed that the classic AML and its epithelioid area as well as the pulmonary and abdominal metastases shared the same allelic loss on chromosome arm 16p. Based on these findings, the authors view this case as evidence of a malignant transformation of a classic AML with morphologic, immunophenotypic, and genetic demonstration of its clonal origin.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
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