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Lymphoma in cardiac allotransplant recipients. Clinical and histological features and immunological phenotype.

To characterize further lymphomas which arise in chronically immunosuppressed allotransplant patients, we studied the clinical and histological features of seven cases of nonHodgkin's lymphoma and determined the immunological phenotype in four cases, which developed in a series of 182 patients who underwent cardiac transplantation at Stanford University Hospital. Clinical features which were correlated with the development of lymphoma included patient age, pretransplant diagnosis, and the number of transplants. All cases of nonHodgkin's lymphoma presented as solitary or multiple localized extranodal lesions while none presented in lymph nodes. Extranodal sites of involvement included brain, lung, and soft tissues of the thigh at the site of antilymphocyte serum injections. Histologically, five of seven lymphomas were classified as high grade, large cell, immunoblastic and two of seven as intermediate grade, large noncleaved cell. The immunological phenotype of the neoplasm from four patients was determined by reaction with immunoglobulin light and heavy chain reagents and monoclonal antibodies to T cell and B cell antigens. All lymphomas from four patients which were tested stained for Ia antigen (HLA-DR) but did not stain for immunoglobulin or the T cell antigens detected in this study.

Abdominal Neoplasms↗

Familial neuroblastoma - different histological manifestations in a family with three affected individuals.

Neuroblastoma is one of the most common malignant neoplasms in childhood. These tumours represent a heterogeneous group both in terms of clinical course and histological appearance, ranging from benign, slowly growing, often asymptomatic ganglioneuromas to malignant, highly aggressive neuroblastomas. Most cases occur sporadically, but in rare cases several individuals in the same family present with ganglioneuroblastomatous tumours. We report a case of familial neuroblastoma, occurring in a mother and her two daughters, with very different clinical presentation, outcome and tumour histology. The mother had recurrent, fully mature, benign ganglioneuromas, predominantly located in the retroperitoneum. The two daughters both developed malignant abdominal neuroblastomas, at the age of 2 and 8 years, respectively. Both died in spite of intensive therapy. Hereditary neuroblastoma appears to be as heterogeneous as the sporadic form of the disease. Since no consistent predisposition gene has been located in affected families, several different genetic or epigenetic events may account for the different histological and clinical presentations.

Abdominal Neoplasms↗

Desmoid tumor arising in a laparoscopic trocar site.

Desmoid tumors are fibrotic neoplasms of low metastatic potential. These tumors have long been associated with major laparotomy incisions. However, to the best of our knowledge, they have not been previously associated with laparoscopic trocar placement. This report involves the case of a patient who developed a desmoid tumor at a trocar site. A 35 year-old premenopausal woman presented with an enlarging infraumbilical mass 10 years after a laparoscopic tubal ligation. The mass was immediately subjacent to the skin incision for the laparoscopic procedure. It was approximately 6 cm in diameter and fixed to the underlying abdominal wall. It was evaluated with sonographic and CT imaging, which revealed a solid mass arising from the fascia with no associated hernia. A biopsy of the mass confirmed a diagnosis of a desmoid tumor. This was subsequently excised with a wide margin and reconstructed with Marlex mesh. The patient is without recurrence 2 years following surgery. This case represents what we believe to be the first report of a desmoid tumor arising from a incision made for the placement of a laparoscopic port. Rapidly increasing laparoscopic applications make it likely that these lesions will increase in frequency. In this setting, resection with wide margins remains the mainstay of treatment for desmoid tumors.

Abdominal Neoplasms↗

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.

Abdominal Neoplasms↗

Intraabdominal desmoplastic small round cell tumor: report of a case with fine needle aspiration, cytologic diagnosis and molecular confirmation.

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a recently described neoplasm. This entity is well defined, with distinct clinical, pathologic and immunohistochemical features. Molecular studies have shown a specific reciprocal translocation t(11;22)(p13;q12). To our knowledge, no report of DSRCT with molecular confirmation on cytologic material has been reported before. CASE: Fine needle aspiration (FNA) was performed on an intraabdominal mass in a 37-year-old man. A May-Grunwald-Giemsa-stained preparation showed clusters of small round tumor cells associated with desmoplastic stromal cells, highly suggestive of DSRCT. FNA of a supraclavicular node showed cytologic features similar to those of the primary abdominal mass, including a prominent desmoplastic reaction of the stroma. Immunocytochemical studies showed myogenic and epithelial differentiation. Molecular analysis was performed on FNA, revealing the EWS/ WT1 chimeric transcript and thus confirming the cytologic diagnosis. CONCLUSION: Cytomorphologically, a definitive diagnosis of DSRCT may be difficult, as this tumor bears considerable resemblance to other small round cell tumors. The diagnosis can be confirmed by ancillary techniques, such as immunocytochemistry, and particularly by molecular analysis, which may also be performed on cytologic material.

Abdominal Neoplasms↗

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.

Abdominal Neoplasms↗

Abdominal tuberculous lymphadenopathy: MR imaging findings.

BACKGROUND: The purpose of this study was to evaluate the utility of magnetic resonance imaging (MRI) as a diagnostic tool in abdominal tuberculous lymphadenopathy. METHODS: MRI studies of 11 patients with histologically proven abdominal tuberculous lymphadenopathy were reviewed with regard to anatomic distribution, size, shape, degree, and pattern of enhancement and relation of the lesions to adjacent structures. RESULTS: The most common site of involvement was the periportal area (n = 6), followed by the peripancreatic (n = 5), mesenteric (n = 1), and paraaortic (n = 1) areas. Eight patients were readily diagnosed as having tuberculous lymphadenopathy on abdominal computed tomography. Three patients had a heterogeneously enhancing masslike lesion adjacent to the pancreas and were initially diagnosed as having cystic tumor of the pancreas. On MRI, 11 lesions showed T1 iso- or hypointensity and central T2 hyperintensity. Two lesions showed T1 iso- or hypointensity and central T2 hypointensity. The lesions with different T2 signal intensities showed different patterns of enhancement on contrast-enhanced dynamic studies. The relations between the enlarged lymph nodes and adjacent bile ducts or vascular structures were well depicted on MRI. CONCLUSION: MRI was useful in differentiating enlarged lymph nodes abutting the pancreas initially diagnosed as cystic neoplasms on abdominal computed tomography.

Abdomen↗

Oncologic diagnosis with 2-[fluorine-18]fluoro-2-deoxy-D-glucose imaging: dual-head coincidence gamma camera versus positron emission tomographic scanner.

PURPOSE: To compare the performance of a dual-head single photon emission computed tomographic (SPECT) Anger camera operated in coincidence mode with that of a dedicated positron emission tomographic (PET) scanner in the imaging of cancer with 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG). MATERIALS AND METHODS: Thirty-one patients with known or suspected malignant neoplasms underwent imaging with both methods, and the images were read blindly. Diagnostic performance on a lesion-by-lesion basis was compared with attenuation-corrected PET as the standard of reference. RESULTS: Of a total of 109 discrete lesions depicted at PET, 60 (relative sensitivity, 55%) were identified on the coincidence-mode images. Of the nodules or masses depicted at PET, 13 (93%) of 14 lung nodules or masses, 20 (65%) of 31 mediastinal lymph nodes, five (71%) of seven lesions in the neck, five (55%) of nine axillary lymph nodes, 11 (50%) of 22 bone metastases, and six (23%) of 26 abdominal tumor deposits were correctly identified on the coincidence gamma camera images. CONCLUSION: These preliminary findings indicate FDG imaging with a modified dual-detector gamma camera operating in coincidence mode can depict many of the lesions depicted with a PET scanner, particularly in the lungs. Sensitivity for lesions detected at dedicated FDG PET was poor in the abdomen and in all locations outside the lungs for tumor deposits generally less than 1.5 cm in short-axis diameter.

Abdomen↗

Brain tumor as a second malignant neoplasm following neuroblastoma stage IV S.

A rare brain tumor (spongioblastoma polare) occurring 7 years after treatment of neuroblastoma stage IV S is reported. The literature concerning the occurrence of a second cancer in children exposed to mutagenic therapy for their initial tumor is reviewed, and genetic and environmental factors are discussed. Diminishing aggressiveness of the treatment in childhood cancer with good prognosis should be considered. Continuous follow-up of children cured of cancer is warranted.

Abdominal Neoplasms↗

Palliation of malignant ascites by the LeVeen peritoneo-venous shunt.

A LeVeen peritoneo-venous shunt was placed in each of 5 patients under local anesthesia to relieve symptomatic malignant ascites. All patients had marked ascites due to histologically documented intraabdominal carcinomatosis, extensive hepatic neoplasm, or a combination of malignant ascites and severe parenchymal liver disease. Cases included metastatic breast carcinoma, pancreatic carcinoma, melanoma, and primary cholangiocarcinoma. Prompt relief of respiratory distress and discomfort associated with tense ascites was achieved in all patients; however, survival was short (one week to seven months) due to advanced disease. The LeVeen shunt can provide effective palliation of malignant ascites in carefully selected symptomatic patients.

Abdominal Neoplasms↗

Pancreatic resection for locally advanced primary and metastatic nonpancreatic neoplasms.

We conducted a retrospective review of our single-institution experience with pancreas resection for locally advanced primary malignancy or metastases from other organs. From January 1989 through April 2001 35 patients underwent pancreatic resection for locally advanced primary (17) and recurrent nonpancreatic (18) tumors. Patient records were examined for recurrence and survival. Seventeen patients with locally advanced primary tumors presented with pancreatic extension either into the head/body (six) or tail (11). Pancreatic resections were completed as en bloc procedures with the primary disease of stomach (five), colon (four), sarcoma (five), adrenal gland (one), or spleen (one). Procedures performed included pancreaticoduodenectomy for proximal lesions and distal pancreatectomy for disease limited to the pancreatic tail. Median overall survival was 56 months. Fourteen of 17 patients remain alive: three with disease and 11 without evidence of recurrence. Eighteen patients presented with recurrent tumor from a previously resected right upper quadrant tumor (nine) or metastases from an intra-abdominal source (nine). The primary source was colon (eight), biliary (three), sarcoma (three), melanoma (two), ovary (one), and unknown primary (one). Patients underwent pancreaticoduodenectomy, distal pancreatectomy, or resection of residual pancreas. Overall median survival was 46 months. In this group of 18 patients there was no increased survival in those patients with a time to recurrence from their primary tumor resection greater than 2 years. We conclude that pancreatic resection for locally advanced nonpancreatic or recurrent intra-abdominal malignancies is possible in properly selected patients. The ability to obtain disease-free margins through en bloc resection is a key component of therapy.

Abdominal Neoplasms↗

Malignant mesenchymoma arising from the prostate in Gardner's syndrome.

The first case of a primary malignant mesenchymoma arising from the prostate in Gardner's syndrome is reported. The neoplasm occurred in a 40-year-old man with a chief complaint of urinary retention. Biopsy findings were primary malignant mesenchymoma. Total cystectomy and lymphadenectomy were performed, but the patient died due to multiple metastases within 6 months. He had undergone a colectomy for polyposis coli 17 years earlier and had mandibular osteomas. This was the first case of malignant mesenchymoma arising from the prostate in Gardner's syndrome.

Abdominal Neoplasms↗

Pancreatic endocrine tumor with signet ring cell features: a case report with novel ultrastructural observations.

The case of a malignant pancreatic endocrine neoplasm with an unusual signet ring cell appearance is reported. The tumor was resected from a 30-year-old man with a 4.0-cm tumor in the body of the pancreas diagnosed by computerized tomographic (CT) scan. The resected tumor had a unique morphology characterized by numerous mucin-negative, signet ring cells, which were argyrophilic and immunoreactive for cytokeratin (CAM 5.2), chromogranin, synaptophysin, neuron specific enolase, and gastrin. Dense-core neurosecretory-type granules and numerous cytoplasmic lamellar inclusions were identified by electron microscopy. These inclusion bodies consisted of multilayered concentric osmiophilic lamellae (myelin figures), which most likely represent an abnormal accumulation of degenerating organelles. Two years later, the patient developed an abdominal recurrence of the tumor, confirming its malignant behavior. This case expands the spectrum of pancreatic endocrine tumors to include an aggressive signet ring cell tumor with a novel ultrastructural basis.

Abdominal Neoplasms↗

Cytology of the liver, spleen and peritoneum obtained by sheathed brush during laparoscopy.

A sheathed cytology brush, fashioned to be used during laparoscopy, is described for brushing the surface of the liver, spleen and peritoneum. The method has had no morbidity or complication in 100 consecutive patients. and has been highly successful in obtaining diagnostic cytological material from malignant neoplasms involving the liver (94%) and peritoneum (100%). In two cases, biopsies of tumor nodules were nondiagnostic, while brush cytology definitively documented malignancy. Because the brushing induces minimal trauma and is quick and simple to perform, it is suggested that brush cytology be considered a supplement to needle and forceps biopsies during laparoscopy. The stainless steel sheath on the brush eliminates concern over loss of cytologic material during brush withdrawal.

Abdominal Neoplasms↗

The utility of ancillary studies in pediatric FNA cytology.

We evaluated the diagnostic contribution of adjunct studies performed on aspirated material in the work-up of pediatric fine-needle aspiration (FNA) biopsies. Ancillary studies were performed on 54 of 136 (39.7%) pediatric FNA biopsies during a 5-year period. In 23 (16.9%) cases, immunocytochemical (ICC) studies, consisting of immunoperoxidase staining of direct smears and/or cell blocks or flow cytometric immunophenotyping, were performed. The studies were adequate in 14 cases (60.9%), suboptimal in five cases (21.7%), and inadequate in four cases (17.4%). Of the adequate and suboptimal cases, the ICC data helped to narrow the differential diagnosis or classify the disease process in eight cases (42.1%), confirmed cytologic impression in nine cases (47.4%), and gave contradictory results in two cases (10.5%). Adequate material for electron microscopy (EM) was obtained in 14/19 cases (73.7%). Ultrastructural studies were diagnostic, or helped classify the disease process in five cases (35.7%), confirmed the cytologic impression in four cases (28.6%), helped exclude diagnostic considerations in three cases (21.4%), and were judged to be non-contributory in two cases (14.3%). Cytogenetic studies revealed six of seven cases (all neoplasms) to have abnormal karyotypes. Special stains for organisms performed on smears from 25 cases including Ziehl-Neelsen, Gomori methenamine silver (GMS), Gram, and Warthin-Starry (WS) were negative except for 1/16 GMS and 4/9 Gram stains. In summary, we found that with appropriate case selection, ancillary studies performed on aspirated material can provide useful information in pediatric FNA cytology.

Abdominal Neoplasms↗

CT of malignant melanoma in the chest, abdomen, and musculoskeletal system.

Malignant melanoma is an aggressive neoplasm that can involve virtually every organ system. This article provides a review of the various appearances on computed tomographic (CT) scans of melanoma involving the chest, abdomen, and musculoskeletal system. Specific emphasis is placed on the typical and atypical CT manifestations of disease as well as the similarity of these findings to those for other disease entities. The importance of accurate staging of melanoma is stressed.

Abdominal Neoplasms↗

Intraabdominal desmoplastic small round cell tumour in an 11-year-old boy.

A case of desmoplastic small round cell tumour (DSRCT) is presented. This aggressive and rare neoplasm predominantly affects males and is almost exclusively intraabdominal in location. It is unique in that neural, mesenchymal and epithelial markers are co-expressed. Despite multi-modal therapy, the prognosis is extremely poor. The present report details the clinical features and typical pathological findings of DSRCT in an 11-year-old boy, who succumbed to the disease 16 months after diagnosis despite multiple chemotherapeutic regimes.

Abdominal Neoplasms↗

Extranodal non-Hodgkin's lymphoma presenting as an abdominal wall mass. A case report and review of the literature.

Soft tissue lymphoma is a very rare clinical entity with varying presentation characteristics and atypical clinical and imaging features. The present report describes a patient who presented with a painless soft tissue mass on the posterolateral surface of the abdominal wall, simulating a neoplasm of mesenchymal origin. After complete surgical excision, the tumor was diagnosed as a diffuse large B-cell lymphoma. No B-symptoms were present and clinical staging did not reveal other sites of disease (stage I EA). The International Prognostic Index score was equal to 1 and classified the patient to the good risk group. Post-operatively the patient was treated with immuno-chemotherapy consisting of rituximab plus cyclophosphamide, epirubicin, vincristine and prednisolone and is currently free of disease for 10 months. The case is discussed with a brief review of the literature on the diagnosis, treatment and outcome of soft tissue lymphomas.

Abdominal Neoplasms↗