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An immunohistochemical evaluation of c-kit (CD-117) expression in malignant melanoma, and results of imatinib mesylate (Gleevec) therapy in three patients.

In order to determine whether imatinib mesylate (Gleevec), a tyrosine kinase inhibitor that binds the CD-117 (c-kit) receptor, may be of value in the treatment of malignant melanoma, an immunohistochemical analysis of 40 cases of primary and metastatic melanoma was undertaken. Thirty-five of the 40 cases showed 1+ or stronger labelling for CD-117 (up to a maximum of 4+). Three patients with neoplasms showing 4+ staining were selected for imatinib therapy. None responded. c-kit (CD-117) expression in melanoma appears to be common; however, the value of imatinib therapy remains to be proven.

Abdominal Neoplasms↗

Intraabdominal desmoplastic small round cell tumour.

Desmoplastic small round cell tumour (DSRCT) is an extremely rare neoplasm. Adolescent males and young adults are most frequently affected. It is highly malignant, with only 29% of patients surviving up to 3 years. This paper documents two cases, one of which, at 4 years old, is the second youngest case documented. Case 1, a 10-year old boy, presented with a 20-day history of choluria, acholia, asthenia, anorexia, and right abdominal pain. Laboratory values were altered, and imaging showed multiples masses in the liver and retroperitoneum. A minilaparotomy was carried out, and a biopsy showed a stage III DSRCT. He was treated with chemotherapy but died of hepatic failure. Case 2, a 4-year-old boy, presented with a 2-month history of abdominal distension. Several hard masses were palpated in the abdomen, and a right inguinal mass that compressed the right testis was observed. Biopsy of the inguinal tumour showed a DSRCT. After treatment with chemotherapy, two operations were carried out to resect different intraabdominal masses. The patient died with peritoneal carcinomatosis 2 months after the last operation. The first patient died due to the advanced stage of the disease, and the second died after chemotherapy, peripheral blood stem transplantation, and multiple operations. The occurrence of this type of tumour in the paediatric age group as well as its high malignancy is noteworthy. Until more effective forms of treatment are found, we recommend treatment with chemotherapy, surgery, and radiotherapy, with close monitoring of the patient.

Abdominal Neoplasms↗

Patterns of extension as a guide to radiation therapy in the management of ovarian neoplasms in children.

Sixteen cases of childhood malignant ovarian neoplasms of varied histology and stages were reviewed with special reference to the patterns of disease extension within the pelvis and abdomen. All patients had initial surgery elsewhere before referral to Memorial Hospital. Two patients had distant metastases (bone and lung). None of the other 14 girls had disease restricted to the pelvis. The first extension in 11 was to sites within the abdominal cavity as well as to the pelvis and in three to the abdomen without pelvic involvement. Total abdominal irradiation would have been required to encompass all known disease-bearing areas in these 14 girls.

Abdominal Neoplasms↗

Desmin positivity in primitive neuroectodermal tumors of childhood.

In this report, we describe two rosette-forming primitive neuroectodermal tumors that were found to contain desmin by both immunohistochemistry and Western blotting. Electron microscopy on both cases was consistent with primitive neuroectodermal tumors and revealed that the tumor cells contained cytoplasmic bundles of intermediate filaments. In both cases, studies for MyoD1 protein using immunohistochemistry and Western blotting were negative. Thus, the detection of desmin in a pediatric neoplasm does not absolutely exclude the diagnosis of primitive neuroectodermal tumor and should not be considered as prima facie evidence that a small-cell tumor is a rhabdomyosarcoma.

Abdominal Neoplasms↗

Epithelioid psammomatous melanotic schwannoma with osseous metaplasia.

Psammomatous melanotic schwannoma is a very rare soft tissue neoplasm, which is clinically, biologically, and histologically distinct from conventional schwannoma. A significant proportion of patients may present with Carney's heritable complex of myxomas, spotty pigmentation, and endocrine overactivity. Typically, the lesion is composed of spindled and epithelioid cells displaying abundant intracytoplasmic melanin pigment, together with the formation of psammoma bodies. We report an epithelioid example of this tumor arising in the anterior abdominal wall of a 23-year-old man. A unique feature was a peripheral rim of osseous metaplasia. The tumor was erroneously diagnosed as a metastatic malignant melanoma on initial examination owing to its immunohistochemical coexpression of S100 protein and HMB-45. Psammoma bodies have not to our knowledge been described in melanomas, and their presence serves as a useful clue to the diagnosis of psammomatous melanotic schwannoma.

Abdominal Neoplasms↗

Desmoplastic small round cell tumor: a clinicopathologic, immunohistochemical, and molecular study of 32 tumors.

Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young male patients and is characterized by a reciprocal translocation t(11;22)(p13;q12) associated with the EWS-WT1 gene fusion transcript. Clinical, histopathologic, immunohistochemical, and molecular genetics features were reviewed for 32 tumors. There were 29 male and three female patients, with ages from 6 to 54 years (mean, 25 years). The main clinical signs and symptoms included abdominal pain (eight patients), weight loss (five patients), and presence of umbilical hernia (four patients). Two tumors primarily involved the ethmoid sinus and the soft tissues of the scalp; the other tumors (mean size, 10 cm) involved the abdominal cavity (88%). One patient presented initially with an axillary lymph node metastasis. Generally, all tumors showed the typical histologic findings of variably sized clusters of small, round, or spindled cells lying in a desmoplastic stroma. The neoplastic cells in formalin-fixed, paraffin-embedded tissue sections were positive for desmin (dot pattern) (81% of the cases), WT1 (91%), keratin (87%), neuron-specific enolase (84%), CD99 (23%), and actin (3%). The EWS-WT1 gene fusion transcript was detected in 29 of 30 tumors. One tumor with typical clinicopathologic and immunohistochemical features did not show the gene fusion. Follow-up for 27 patients showed that 19 patients (70%) died of uncontrolled, local, or widespread metastatic disease 3-46 months (mean, 20 months) after diagnosis, and eight patients were alive with known evidence of disease. Occasionally, desmoplastic small round cell tumor lacks the classic clinical, histologic, and immunohistochemical features. This study emphasizes the utility of analysis of the EWS-WT1 gene fusion transcript, which was performed on paraffin-embedded tissues, to confirm the diagnosis.

Abdominal Neoplasms↗

Umbilical polyp: a report of two cases.

Two cases with umbilical polyp are reported. This rare malformation arises from remnants of the omphalomesenteric duct and may be clinically mistaken for a granuloma pyogenicum or other benign or malignant umbilical neoplasms. Histologically umbilical polyp shows branching glandular structures lined by intestinal mucosa in connection with the surface of the skin. When the umbilical polyp is not associated with any other types of underlying abnormalities, it may be treated by a simple surgical excision.

Abdominal Neoplasms↗

[Changes in the thoracic duct and lymphodynamics in neoplastic diseases].

X-ray contrast study of the thoracic duct was conducted in 35 patients with various neoplasms of the chest and upper abdomen, as well as in tumor involvement of lymph nodes, located proximally to the main lymphatic collector. Pathological changes in the thoracic Duct and central lymphodynamics, observed in neoplastic lesions concerned, are described.

Abdominal Neoplasms↗

Desmoplastic small round cell tumour: a description of two cases and review of the literature.

BACKGROUND: Desmoplastic small round cell tumour (DSRCT) is a recently described neoplasm, typically occurring in adolescent and young males. It usually shows an aggressive behaviour, presents in the abdomen, often with diffuse peritoneal implants. It has been demonstrated to be a chemosensitive tumour, generally with short-lasting response and poor survival gain from systemic chemotherapy. The authors report two additional cases of DSRCT and review the available medical literature. PATIENTS AND METHODS: Two young males with intra-abdominal DSRCT were treated with a first-line chemotherapy including carboplatin, doxorubicin and etoposide. RESULTS: Both of the patients obtained a partial response after first-line chemotherapy. The first patient started, subsequently, CD34+ stem cell mobilisation with high-dose cyclophosphamide (7 g/m(2)) in order to perform high-dose chemotherapy, but CD34+ cell count was insufficient to practice leukapheresis; he died 34 months after the diagnosis because of progression of the disease. The second patient underwent cytoreductive surgery, but progressed 2 months later despite second-line treatment; he died 16 months after the diagnosis. CONCLUSION: This experience confirms that DSRCT may be considered a chemosensitive tumour, highly aggressive, with short-lasting response to chemotherapy. Anyway, the recent literature suggests that multidisciplinary treatment including chemotherapy, surgery and radiation might be the proper approach to this rare malignancy.

Abdominal Neoplasms↗

[Does Bolande's malignant tumor exist?].

Congenital mesoblastic nephroma (CMN) is a rare renal neoplasm which Bolande differentiated from Wilms tumour given its benign nature. We describe 7 cases of CMN which have been treated over the last ten years, in order to highlight to what extent a tumour which is generally considered to be benign can have an aggressive behaviour. 5 patients were diagnosed during their neonatal period (3 of them prenatally) and 2 after the third month of life. Clinical presentation, simple abdominal radiology, echography and abdominal CT scan, which showed characteristic images, led us to a final diagnosis. Tumoral resection was carried out in the 6 typical CMN. The atypical or aggressive CMN was urgently operated under critical circumstances due to spontaneous tumoral rupture. The pathological study showed a CMN of cellular variety with a sarcomatous component. Despite adjuvant chemotherapy, there was a tumoral recurrence which required a surgical second-look and subsequent chemotherapeutical treatment. Bolande's tumour is generally benign, but there is an aggressive cellular variant that fits into the intermediate zone of a pathological spectrum. This aggressive variant have different clinical, radiological and anatomo-pathological features and therefore it involves radical surgical treatment and additional chemotherapy with a rigorous follow-up in the short and the long term.

Abdomen↗

[Magnetic resonance in oncology].

The physical and biological background to magnetic resonance imaging (MRI) in patients with neoplasms is reviewed. The clinical indications for this method of diagnosis, staging, and follow-up of malignant neoplasms are discussed on the basis of the guidelines given by the MRI Consensus Conference on April 25-26, 1989, in Berne. MRI is the modality of choice in patients with neoplasms of the central nervous system and the musculoskeletal system. Further emerging indications for MRI are neoplasma of the ENT region, liver, and pelvic organs. Finally, the foreseeable developments in MRI and in magnetic resonance spectroscopy (MRS) are mentioned.

Abdominal Neoplasms↗

Extra-adrenal phaeochromocytoma: report of three interesting cases.

The clinical, laboratory and operative findings from three cases of extra-adrenal phaeochromocytoma are reviewed. Some of the difficulties encountered in localizing such a neoplasm are highlighted. Data from intraoperative catecholamine monitoring on the three patients are also presented. Adequate alpha and beta blockade produced stable cardiovascular parameters during surgery in all three individuals.

Abdominal Neoplasms↗

CT findings of regression in intraabdominal desmoplastic small-cell tumor.

We report the computed tomographic (CT) findings in a patient with intraabdominal desmoplastic small-cell tumor before and after 10 weeks of chemotherapy. This tumor is a rare, frequently fatal neoplasm of the peritoneum, seen predominantly in young males. Initial CT demonstrated large intraperitoneal masses, hepatic metastases, retroperitoneal and right axillary lymphadenopathy, ascites, and pleural effusion. Follow-up CT showed marked decrease of the main tumor bulk and complete regression in the axillary nodes.

Abdominal Neoplasms↗

Digital subtraction angiography in the evaluation of neoplasms.

Intravenous and intraarterial DSA studies were performed in patients being evaluated for possible neoplastic disease. DSA studies were obtained of the head, neck, chest, abdomen and extremities to evaluate the potential use of this technique in the cancer patient's workup. The results of this study suggest that many cancer patients can have the extent of tumor involvement defined using intravenous digital subtraction angiography. However, due to the number of false negative examinations, this study should not be relied upon as the only screening procedure. The current best application of DSA appears to be its use in conjunction with intraarterial injections as a faster and more economical alternative to angiography and in following up these neoplasms with intravenous DSA after appropriate therapy to evaluate possible recurrence or metastatic spread. Hybrid subtraction techniques and/or the development of new contrast media may greatly facilitate the use of intravenous DSA in conjunction with cancer diagnosis.

Abdominal Neoplasms↗

Isotropic CT examination of abdomen and pelvis diagnostic quality of reformat.

RATIONALE AND OBJECTIVES: To evaluate the image quality of axial and coronal reformats obtained from isotropic resolution abdomino-pelvic computed tomography (CT) examinations. MATERIALS AND METHODS: Thirty consecutive patients with intravenous contrast-enhanced abdomino-pelvic CT examinations (Brilliance 40, Philips Medical Systems, Cleveland, OH) were enrolled for the study. The raw data were reconstructed into two sets of source axial images: 0.9-mm slice widths with 0.45-mm reconstruction interval (isotropic resolution) and 4-mm slice widths with 3-mm reconstruction interval (anisotropic resolution: group A). Isotropic data set was reformatted into axial and coronal stacks (groups B and C, respectively) with 4-mm slice width and 3-mm interval. Three independent readers evaluated stacks A to C using a 3-point scale for resolution of hepatic vessels, edge sharpness of kidneys, respiratory motion artifact, reconstruction artifact, noise, and overall image quality. RESULTS: There was no statistical difference among the groups A to C for vessel resolution, motion artifact, noise, and overall quality. The scores given to group C were significantly lower than those to groups A and B for reconstruction artifacts. There was no difference among groups A to C for overall impression of image quality. The interreader agreements were excellent for axial images (groups A and B) and moderate for coronal reformats. CONCLUSION: Isotropic scanning of the abdomen and pelvis allows creation of reformats with similar image quality as similar thickness axial source images. These reformats are of sufficient quality to form the basis of clinical interpretation.

Abdominal Cavity↗

Intraabdominal desmoplastic small round cell tumor: results of ifosfamide-based chemotherapy.

Intraabdominal desmoplastic small round cell tumor (IDSRT) is a rare neoplasm, which is malignant and typically occurs in young adults. Although IDSRT is chemotherapy-sensitive, most cases do not achieve a complete response (CR). Even if some cases achieve a CR, they often relapse and result in poor prognosis. We report two cases of IDSRT, in a 24-year-old man and a 23-year-old man, who were treated with ifosfamide-based chemotherapy. Case 1, a 24-year-old man, presented with an abdominal mass, and the diagnosis of the biopsy specimen at laparoscopy was IDSRT. He received 7 courses of ifosfamide-based chemotherapy, which obtained a partial response (PR). He had no surgical resection, except for the biopsy procedure, and he underwent a total of 21 courses of chemotherapy. He died 36 months after diagnosis because of tumor regrowth and peritoneal bleeding. Case 2, a 23-year-old man, whose biopsy material allowed a diagnosis of IDSRT, was treated with 18 courses of chemotherapy, using ifosfamide, that resulted in a PR. This was followed by resection of the abdominal lesions, and he received an additional 2 courses of adjuvant chemotherapy. Four months after, completion of these 2 courses, the tumor recurred, and he died of diffuse pulmonary metastasis 36 months after diagnosis. This case report examines the results of ifosfamide-based chemotherapy in our two patients.

Abdominal Neoplasms↗

Fine needle aspiration appearance of extragastrointestinal stromal tumor. A case report.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) rarely develop outside the digestive tract and in the soft tissues of abdomen and retroperitoneum. Such tumors are designated extra-GISTs (EGISTs). Cytologic and immunocytochemical features of a case of EGIST are reported. CASE: A 54-year-old woman presented with a peritoneal mass, diameter 22 cm, adherent to the omentum and without a connection to the digestive tract. Fine needle aspiration biopsy (FNAB) of the excised tumor showed high cellularity in two patterns: monotonous spindle cells were intermingled with a mildly atypical epithelioid component. Immunocytochemistry performed on cytospins revealed reactivity for c-kit (CD117), CD34 and smooth muscle actin and negativity for S-100. The findings were concordant with a histologic diagnosis of EGIST. CONCLUSION: EGISTs are infrequent neoplasms and can be diagnosed in FNAB samples. The clinical/radiologic setting must be considered together with the cytologic features. Immunocytochemistry is a clue to the diagnosis when it detects c-kit reactivity.

Abdominal Neoplasms↗

Prediction rule for etiology of vague abdominal pain in the emergency room: utility for imaging triage.

RATIONALE AND OBJECTIVES: To determine the predictive value of clinical parameters in patients with nonspecific abdominal pain undergoing computed tomography (CT) evaluation of the abdomen and pelvis in the emergency room (ER). MATERIALS AND METHODS: A cross-sectional study of a total of 164 sequential abdominal CT exams of the abdomen and pelvis during a 4 month period for nonspecific abdominal pain in the ER setting identified 100 abnormal scans in 164 patients (61 men, 103 women) of average age 46 years (range 4-97). Patient demographic characteristics (age, sex, temperature, white blood cell (WBC) count, and presence of peritoneal signs) were recorded at the time of CT examination. Results of the CT studies were correlated with the clinical data and discharge diagnosis to assess their positive predictive value using ordinal logistic regression. RESULTS: There were 17 cases of appendicitis, 9 cases of diverticulitis, 3 neoplasms, 3 abdominal abscesses, 2 pancreatitis, 2 duodenitis, 5 with fluid collections, 1 buttocks abscess, and 1 epiploic appendagitis were diagnosed with CT, 57 patients had unrelated findings on CT (common but not usually associated with vague pain). A diagnosis of appendicitis correlated with; elevated WBC count (>11.5) ( = 0.002), male sex ( = 0.001), and younger age (<25 years old) ( = 0.002). A positive CT correlated with an elevated WBC >11.5 (OR, 7.7; 95% CI, 3.3-18). The presence of peritoneal signs and fever did not correlate with a positive CT finding and diverticulitis had no predictive variables. Alternative diagnoses were correlated with female sex ( = 0.014). The combination of; age, sex, and WBC count allowed for a prediction rule with Area under the receiver operator curve of 0.92 to be generated. CONCLUSION: An elevated white blood cell (WBC) count is strong evidence of the presence of an inflammatory process. Alternative diagnostic considerations should be entertained in the context of a normal WBC count without strong clinical suspicion, particularly in women. The use of these factors alone allowed the construction of a prediction rule that can be used for CT protocol optimization.

Abdominal Pain↗