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Massive congenital intracranial teratoma with skull rupture at delivery.

The majority of case reports concerning intracerebral tumors are almost equally divided between teratomas and gliomas. We report a case of congenital intracranial teratoma with suggested management. An 18-year-old woman at 30 weeks' gestation in active labor because of severe fetal hydrocephalus and polyhydramnios was delivered by cesarean section. Spontaneous rupture of the neonatal skull led to delivery of a stillborn infant; a congenital teratoma was found. Although intracranial teratoma is a well-recognized entity in the differential diagnosis of pediatric brain tumors, massive congenital teratoma replacing cerebral hemispheres of a fetus has been seldom reported. Antenatal diagnosis of the neoplasm and abdominal delivery may not improve outcome.

Adolescent↗

Are complications in intraoperative radiation therapy more frequent than in conventional treatment?

To evaluate whether intraoperative radiation therapy (IORT) results in higher complication rates than conventional radiotherapy, 119 patients were studied who entered four prospectively randomized clinical trials that compared IORT with conventional therapy. Malignant neoplasms included 33 gastric carcinomas, 35 retroperitoneal sarcomas, 22 resectable pancreatic cancers, and 29 unresectable pancreatic cancers. One hundred thirty-six complications developed among 66 patients who received conventional therapy, and 108 complications developed among 53 patients who received IORT. There was no statistical significance between treatment groups with respect to the overall incidence of complications. Analysis of types of complications by tumor type using Fisher's exact test revealed only one significant complication: an increased rate of sepsis among the patients with retroperitoneal sarcoma who received conventional therapy compared with their IORT cohorts. The overall complication rate associated with IORT was equivalent to conventional radiotherapy in the treatment of these malignant neoplasms and supported the use of IORT where clinically indicated.

Abdominal Neoplasms↗

The ultrastructural and immunohistochemical heterogeneity of CD-30-positive neoplasms: so-called anaplastic large cell Ki-1 lymphomas.

Anaplastic large cell lymphoma (ALCL), also referred to as Ki-1 lymphomas, was first recognized as an entity with characteristic light microscopic appearance in 1985. This tumor is composed of variably cohesive cells, often with large, markedly atypical, and multinucleated cellular forms. The recognition of ALCL resulted from the development of a monoclonal antibody in Kiel, Germany, named Ki-1, which was initially believed to be a putative marker for Reed-Sternberg cells. This antibody was later found to be specific against the epitope CD-30. Attempts to create strict criteria to preserve this neoplasm as a specific entity have undergone evolution. However, it is now clear that included in this group are a variety of pleomorphic neoplasms with CD-30 immunoreactivity. Some of these neoplasms are nonlymphoid and show marked heterogeneity in their immunohistochemical and ultrastructural profiles. This article aims to highlight the ultrastructural spectrum of neoplasms exhibiting CD-30 positivity that are within the spectrum of ALCL. It remains to be determined if there are subgroups of these CD-30-positive neoplasms that can be segregated on the basis of ultrastructural and immunohistochemical criteria with corresponding clinical correlates that may impact on their management, treatment, and prognosis. We review here the heterogeneity of CD-30-positive neoplasms (so-called anaplastic large cell Ki-1 lymphomas).

Abdominal Neoplasms↗

Results of multimodal treatment for desmoplastic small round cell tumors.

PURPOSE: Desmoplastic small round cell tumors (DSRCTs) are rare aggressive neoplasms that frequently present with large symptomatic intraabdominal masses. We examined the effects of multimodal therapy including induction chemotherapy, aggressive surgical debulking, and external beam radiotherapy on patients with DSRCT. METHODS: Institutional Review Board permission was obtained. Sixty-six patients were diagnosed by histology, immunohistochemistry, and or cytogenetics as having DSRCT at our institution from July 1, 1972, to July 1, 2003. Data were collected on patient demographics, presenting symptoms, tumor location and extent, treatment regimen, and overall survival. RESULTS: A majority of patients were male (91%), Caucasian (85%), and with a median age of 19 (7-58) years old at diagnosis. The most common presenting complaint was an intraabdominal mass (64%). In 63 patients (96%), the primary tumor was located in the abdomen or pelvis. Thirty-three (50%) had positive lymph nodes and 27 (41%) had distant parenchymal metastases at diagnosis. Overall, 3- and 5-year survivals were 44% and 15%, respectively. Twenty-nine of these patients (44%) underwent induction chemotherapy (P6), surgical debulking, and radiotherapy. Three-year survival was 55% in those receiving chemotherapy, surgery, and radiotherapy vs 27% when all 3 modalities were not used (P < .02). Gross tumor resection was highly significant in prolonging overall survival; 3-year survival was 58% in patients treated with gross tumor resection compared to no survivors past 3 years in the nonresection cohort (P < .00001). Ten patients (15%) have no evidence of disease with a median follow-up of 2.4 years (range, 0.4-11.2 years). CONCLUSIONS: Multimodal therapy results in improved survival in patients with DSRCT. Aggressive surgical resection of these extensive intraabdominal neoplasms correlates with improved patient outcome.

Abdominal Neoplasms↗

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.

Abdominal Neoplasms↗

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.

Abdominal Neoplasms↗

[Extramedullary lymphosarcoma, erythroblastopenia and gastric adenocarcinoma. Apropos of a case].

The authors report the case of a patient with lymphosarcoma and erythroblastopenia who died following a surgical operation for gastric carcinoma. The erythroblastopenia, corrected by radiotherapy of the lymphosarcoma, reappeared during the relapse. The link between these two diseases seems undoubted. The later appearance of a gastric carcinoma suggested the predisposing role of the first neoplasm, and/or its treatment, through an immunological disorder.

Abdominal Neoplasms↗

Unsuspected carcinoma of the gall bladder: case report of trocar-site metastasis following laparoscopic cholecystectomy.

Cancer of the gall bladder is a rare malignant neoplasm with an unfavourable prognosis. Laparoscopic surgery has brought about the emergence of possible neoplastic diffusion along trocar tracts in cases where unrecognized carcinoma of the gall bladder is present. The authors present a case of neoplastic abdominal diffusion discovered 4 months after laparoscopic cholecystectomy in which histologic examination of the surgical specimen revealed the presence of unrecognized carcinoma of the gall bladder.

Abdominal Neoplasms↗

Multiple fetuses in fetu:imaging findings.

Fetus in fetu is an extremely rare developmental abnormality secondary to abnormal embryogenesis in a diamniotic monochorionic pregnancy. It occurs when a vertebrate fetus is enclosed within the abdomen of a normally developing fetus. This report describes the prospective diagnosis of fetus in fetu by findings on a plain radiograph and CT scan. At surgery, two fetus in fetu were discovered.

Abdominal Neoplasms↗

Pediatric fine-needle aspiration biopsy.

A total of 135 fine-needle aspiration (FNA) biopsies from varying sites were performed in 123 children (mean, 10.5 years; range, one day to 18 years) over a five-year period. One hundred thirty (96.3%) biopsy specimens were satisfactory for evaluation. Seventy-nine cases were nonneoplastic (60.8%); among these cases, a specific diagnosis of infectious disease was made in 17 (13.1%). A diagnosis of neoplastic disease was made in 50 (38.5%) cases, of which 14 (10.8%) were benign, 28 (21.5%) were malignant, and 8 (6.2%) were neoplasms of uncertain biologic potential. The sensitivity of pediatric FNA biopsies was 90.6%, specificity 100%, positive predictive value 100%, negative predictive value 94.7%, and efficiency of the test 96.5%. There were no false-positive diagnoses and there were four false-negative diagnoses, three of which involved aspirates of the central nervous system (CNS). Ancillary studies, including immunocytochemistry (20 cases), electron microscopic examination (18 cases), microbiologic culture (8 cases), cytogenetic studies (7 cases), and flow cytometry (3 cases), were performed on the aspirated material, enabling a more specific diagnosis or supplying additional information in many cases. Definitive diagnosis by FNA biopsy enabled radiation therapy and/or chemotherapy to be administered for unresectable malignant neoplasms, provided material for culture of infectious lesions, identified benign lesions not needing surgery, and aided the surgeon in planning the extent of surgery in resectable malignant neoplasms. These results support the greater use of FNA biopsy in the pediatric population.

Abdominal Neoplasms↗

CT of the mesenteric vascular anatomy.

BACKGROUND: To evaluate the appearance of the arrangement of the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) on computed tomography (CT) in normal patients and in patients with abdominal masses. METHODS: One hundred seventy-seven consecutive abdominal CT examinations of 143 adults and two children were reviewed. The relationship of the SMV to the SMA was recorded at four locations: the beginning of the mesenteric vessels and levels 3 cm, 6 cm, and 9 cm caudad to the beginning. The relationship of the SMV to the SMA was divided into four quadrants in relation to the SMA: I, ventral right or directly ventral; II, dorsal right or directly right; III, dorsal left or directly dorsal; and IV, ventral left or directly left. RESULTS: In the beginning of the SMV-SMA complex and levels 3 cm, 6 cm, and 9 cm caudal to the beginning, the SMV was located in quadrant I in 146, 84, 69, and 43 examinations, in quadrant II in 31, 93, 71, and 27 examinations, in quadrant III in zero, zero, five, and three examinations, and in quadrant IV in zero, zero, nine, and 15 examinations, respectively. The cases with SMV inversion had neither malrotation nor adjacent tumor compression. All the cases with an adjacent tumor-induced compression of the SMV-SMA complex had a normal SMV-SMA relationship. CONCLUSION: In the first 3 cm, the SMV is always to the right of the SMA. Caudal to the level of 6 cm, the SMV may be located to the left of the SMA without evidence of malrotation. A midgut nonrotation is more likely to be present when a proximal SMV inversion is coexistent with a rightward direction of the proximal jejunal vessels. A hypothetical depiction of the step-by-step change of the SMV-SMA relationship during embryologic development may explain the arrangement patterns of the mesenteric vessels in normal rotation and midgut nonrotation.

Abdominal Neoplasms↗

Computed tomography of fluid collections within the abdomen.

Computed tomography is ideally suited for detecting mass lesions and for distinguishing "solid" from "cystic" masses. In the majority of cases, the recognition of fluid collections is unequivocal and lead to direct management decisions. Subphrenic, intrahepatic, and subhepatic abscesses can be recognized, aspirated, and drained. Using CT control, pancreatic pseudocysts can be managed similarly. Renal cysts can be accurately diagnosed with CT, especially with contrast enhancement, and then even the occasional case in which hydronephrosis may be a problem can be distinguished. Very occasionally, solid tumors containing mucinous material or lipoid can appear as cystic lesions, especially in the suprarenal, ovary, liver, or lymph nodes, and, conversely, abscesses may have higher tissue attenuation values, suggesting a solid lesion. It is most important to ensure that bowel is adequately labeled with contrast so that is is not misdiagnosed as a mass lesion.

Abdominal Neoplasms↗

Cytology of ascitic fluid in a patient with metastasizing malignant Brenner tumor of the ovary. A case report.

BACKGROUND: Transitional cell ("Brenner") tumors represent about 2% of all ovarian neoplasms. Brenner tumors are almost always benign. Malignant Brenner tumors of the ovary resemble urothelial carcinomas and are extremely rare. CASE: A 77-year-old, white female presented with malignant Brenner tumor in both ovaries as well as lung and abdominal metastases. The cytology of the ascitic fluid revealed many activated mesothelial cells and three-dimensional cell clusters arranged in a papillary pattern. The round to oval nuclei displayed mild anisokaryosis and hyperchromasia but had a quite evenly dispersed opaque or finely granular nucleoplasm. Enfoldings of the nuclear membrane gave them the appearance of so-called coffee bean nuclei. The cytoplasm stained light bluish. CONCLUSION: Knowledge of the cytologic features of ascitic fluid might allow a preoperative diagnosis of malignant, or at least proliferating, ovarian Brenner tumor.

Abdominal Neoplasms↗

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.

Abdominal Neoplasms↗

Routine helical CT of the abdomen: image quality considerations.

PURPOSE: Both helical and nonhelical abdominal computed tomographic (CT) scans were obtained to compare image quality, study the effect of patient size and collimation, and compare the frequency of visualization of normal abdominal structures. MATERIALS AND METHODS: The study group consisted of 60 consecutive patients with clinically suspected metastatic malignancy. RESULTS: Both helical and nonhelical image quality was excellent, with equal mean image quality scores of 4.1 on a 5-point scale. In patients weighing more than 175 lb (79 kg), both helical and nonhelical image quality degraded equally when 5-mm collimation was used; 10-mm collimation resulted in excellent image quality, regardless of patient size. Small in-plane structures (eg, renal arteries, renal veins, pancreatic duct) were seen best on helical scans. With the addition of retrospectively reconstructed overlapping images, improvement in visualization of these structures was statistically significant. CONCLUSION: Helical CT scanning should be the preferred means of acquiring routine abdominal CT images.

Abdominal Neoplasms↗

[Soft tissue sarcoma as a result of uterine cervix cancer radiotherapy].

The secondary neoplasms due to previous radiotherapy occur rarely. They develop within the radiation field or on its border. In this paper we present the case of soft tissue sarcoma that evolved 11 years after uterine cervix cancer radiotherapy. The long-term survival of patients subjected to radiotherapy is linked with the risk of secondary neoplasms appearance. That is why the repeated regular observation after radical treatment is recommended.

Abdominal Neoplasms↗