1/173. Excessive nodular hyperplasia of brunner glands associated with gastric hypersecretion and lipomatous atrophy of the pancreas.The case of a 34-year-old woman complaining of diarrhoea and abdominal pain is presented. Contrast radiography and endoscopy showed multiple polypoid tumours in the second part of the duodenum. Moreover, a severe fatty infiltration of the pancreas was shown by magnetic resonance and computed tomography scans. Due to pain, pancreatoduodenectomy (Whipple operation) was performed, and subsequent histopathologic examinations showed excessive Brunner gland hyperplasia of the duodenum and severe lipomatous atrophy of the pancreas. The occurrence of these two rare conditions in one patient has not been described previously, and it is conceivable that the lipomatous atrophy and exocrine insufficiency of the pancreas may have caused a compensatory stimulation of the submucosal structures of the duodenum.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/173. Acute leukemia following prolonged cytotoxic agent therapy.1. Nine patients in whom acute non-lymphoblastic leukemia (ANLL) developed following prolonged alkylating agent therapy are described. Five of the patients received no radiotherapy. The conditions treated were: Hodgkin's disease (four patients), primary amyloidosis, primary macroglobulinemia, malignant lymphoma, multiple myeloma, and carcinoma of the tonsil. 2. Prior to the advent of chemotherapy, this complication was not observed in large series of patients with lymphoproliferative disorders and multiple myeloma. However, the medical literature now contains at least 125 other detailed reports of ANLL developing after prolonged cytotoxic agent therapy. 3. multiple myeloma and Hodgkin's disease, both of which commonly have good responses to chemotherapy, predominate as the underlying diseases. However, 35% of the case reports involve patients with other illnesses, including 12 patients who did not have neoplasms. 4. More than half of the patients developing ANLL have received chemotherapy alone without radiotherapy. 5. At least half of the patients developing ANLL experienced long periods of significant cytopenia during therapy, often with documentation of bone marrow dysplasia. 6. The wide variety of drugs associated with this complication suggests that any cytotoxic agent may be leukemogenic. However, alkylating agents overwhelmingly predominate as the class of compounds which are most often associated with terminal ANLL. 7. The vast majority of patients reported in the literature with ANLL complicating underlying malignancies have received cytotoxic drugs for prolonged periods (median 3 1/2 years) and leukemia developed most commonly 3 to 5 years after the diagnosis of the underlying disease. Most of these patients benefited from therapy and survived longer (median 5 years) than historical control of untreated patients. 8. The leukemogenic potential in man of prolonged cytotoxic agents therapy, especially with alkylating agents, seems to be well established. This evidence admonishes against the prolonged use of these drugs in non-fatal disorders. 9. More accurate assessment of risk: benefit ratios awaits the results of prospective controlled studies. The results of these studies could also lead to significant modifications in recommendations for long-term maintenance therapy with cytotoxic agents.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
3/173. Cutaneous involvement in lymphoblastic lymphoma.Lymphoblastic leukemia/lymphoma (LBL) is a malignant neoplasm of precursor lymphocytes of B- or T-cell phenotype. Involvement of the skin is relatively uncommon. We examined retrospectively the clinicopathologic, immunophenotypic, and molecular genetic features of six patients with cutaneous involvement of LBL (B-LBL=5; T-LBL=1). patients presented clinically with solitary, large tumors located on the head (3 cases) or the back (1 case), or with generalized tumors (2 cases). Ulceration was uncommon. In two patients the onset of skin lesions was concomitant to the diagnosis of lymphoblastic leukemia. Histopathologic examination showed in all cases a dense, diffuse, monomorphous infiltrate located in the entire dennis and subcutaneous fat. A typical "starry sky" pattern was observed in the majority of the lesions. In some areas neoplastic cells were aligned in a "mosaic-like" fashion. Cytomorphologically, medium sized lymphoid cells with round or convoluted nuclei, inconspicuous nucleoli and scant cytoplasm predominated. There were no significant differences in the histopathologic features of skin lesions in T- and B-LBL. In B-LBL, CD79a was more useful than CD20 in determining the phenotype of neoplastic cells (4/5 cases positive for CD79a as compared to 2/5 cases positive for CD20). TdT, CD10 and CD43 were positive in 4 cases, CD34 in 2. The case of T-LBL revealed positivity for CD1a, CD3, CD43 and TdT, and negativity for CD34 and for B-cell markers. All neoplasms were positive for CD99 and bcl-2, and showed a high proliferation rate. Molecular genetic analysis of J(H) and T-cell receptor (TCR) genes performed using a polymerase chain reaction technique revealed a monoclonal rearrangement of J(H) genes in all five B-LBLs. One of these cases showed also a concomitant TCR-gamma gene rearrangement. A monoclonal rearrangement of the TCR-gamma gene was detected in the case of T-LBL. Our study shows that skin lesions of LBL present characteristic clinicopathologic and molecular features allowing the differentiation from other cutaneous lymphomas, even in cases without clinical history of previous precursor lymphoblastic leukemia/lymphoma.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
4/173. Nonocular cancer in retinoblastoma survivors.From a review of the records of 2,302 patients with retinoblastoma collated from CPMC and AFIP, it was found that retinoblastoma patients who survived their original eye cancer have a high incidence of second nonocular malignancies. The second neoplasms occur almost exclusively (97.5%) in patients who have had bilateral retinoblastoma, although retinoblastoma is much more commonly unilateral. The second neoplasms have appeared between 1 and 42 years after the successful treatment of retinoblastoma and have been fatal in approximately 85% of cases. Seventy-one percent of patients develop tumors in the field of the radiation beam; many of these were following treatment with low doses of radiation and after short latent periods. Nineteen percent of patients develop tumors clearly out of the field of radiation (eg, osteogenic sarcoma of the femur). retinoblastoma patients appear to be unusually radio-sensitive to low doses of radiation and develop tumors in the field of radiation following treatment of 3,500 rads. They have a high incidence of nonocular tumors clearly distant from the radiation beam develop nonocular malignancies even if no radiation has been given. The incidence of multiple malignancies in patients successfully treated for retinoblastoma appears to be higher than for any other primary malignancy whether they receive radiation or not.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
5/173. choriocarcinoma arising in a squamous cell carcinoma of the esophagus.Extragonadal germ cell tumors are rare neoplasms with histologic features comparable to those of gonadal origin. Squamous cell carcinoma of the esophagus was diagnosed in a 53-year-old male patient, and was palliated for a short period by cisplatin plus 5-fluorouracil. Clinical deterioration and development of gynecomastia led to diagnosis of hormone-secreting choriocarcinoma that originated within the squamous cell tumor of the esophagus. Salvage chemotherapy affected the markers but not the tumor. Extragonadal choriocarcinoma is a chemosensitive tumor, but when arising within squamous cell carcinoma of the esophagus it may be chemoresistant, and lead to a fatal outcome.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
6/173. Diffuse neonatal haemangiomatosis.A newborn girl with severe diffuse neonatal haemangiomatosis is described. She was treated with high dose systemic corticosteroids and high dose interferon-alpha-2a, but with fatal outcome. A review of the current literature is presented.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
7/173. Two cases of segmental multiple glomangiomas in a family: type 1 or type 2 segmental manifestation?Several autosomal dominant skin diseases may manifest cutaneous mosaicism. Two types of segmental arrangement can be distinguished: type 1 is characterized by segmental lesions with similar severity to that observed in the diffuse phenotype, the remaining skin being normal; type 2 is characterized by segmental lesions showing a major degree of severity and milder lesions diffusely arranged. Multiple glomus tumours have recently been included in the group of genodermatoses showing type 2 segmental involvement. A family with 2 cases of multiple glomangiomas arranged in a segmental fashion is reported. A 12-year-old girl presented multiple nodular glomangiomas on her right buttock and thigh, in a band-like distribution. A sister of her paternal grandfather showed plaque-like multiple glomangiomas on her left thigh and various glomangiomas on her right buttock and arm. No other family members were known to be affected. Two new cases of familial segmental multiple glomangiomas are reported, with the particularity that one of these exhibited type 2 segmental manifestation and the other type 1 from a clinical point of view.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
8/173. Hepatic angiomyolipoma with concomitant hepatocellular carcinomas.angiomyolipoma is a rare lipomatous tumor in the liver. Definitive preoperative diagnosis is becoming easier by the use of ultrasonography, computed tomography, and magnetic resonance imaging techniques. Nonsurgical treatment has been advocated for its benign nature. However, recently we encountered one case of hepatic angiomyolipoma with two concomitant hepatocellular carcinomas on a hepatitis b carrier. Although his serum alpha-fetoprotein was normal, under the above impression these lesions were resected. The pathologic findings showed a typical angiomyolipoma and two well-differentiated hepatocellular carcinomas with marked fatty metamorphosis. This is the first report of angiomyolipoma with concomitant hepatocellular carcinomas in the literature. Nonsurgical treatment of angiomyolipoma in an endemic area for hepatocellular carcinoma should proceed with caution because cases of fat-rich minute hepatocellular carcinomas will make the diagnosis difficult.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
9/173. Inheritance of Hippel-Lindau disease: a rare case of maxillary bone metastasis.A rare case of familial Hippel-Lindau disease, which is an autosomal dominant disorder with inherited susceptibility to various forms of cancer, is reported in a 46-year-old father and his sons. The father complained of a malignant hypernephroma, which metastasized in the upper jaw 6 months later. As in this patient, renal cell carcinomas are fatal and are exhibited at an early age. Consequentially, patients with familial von hippel-lindau disease exhibiting cases of renal cell carcinoma should be examined very carefully in the head and neck region to detect and treat metastases as early as possible.- - - - - - - - - - ranking = 3keywords = fat (Clic here for more details about this article) |
10/173. Lipoma of the liver associated with hepatocellular carcinoma and polycystic liver disease.BACKGROUND/AIMS: We report on a patient with lipoma of the liver associated with hepatocellular carcinoma (HCC) and polycystic liver disease. methods: Clinical features of a patient with lipoma, HCC and polycystic liver disease are presented. A right liver lobectomy was performed. RESULTS: Histological examination revealed a poorly differentiated multicentric HCC, as well as bile duct cysts and an encapsulated tumor of adipose tissue. The postoperative course was uncomplicated. The patient died 4 years after surgery with local recurrence of HCC. CONCLUSION: We find the report to be of interest because of the unusual nature of this association and the low prevalence of lipoma of the liver. The association of liver polycystic disease with other types of tumor is discussed and the literature dealing with lipomas of the liver reviewed.- - - - - - - - - - ranking = 83.97494331406keywords = adipose (Clic here for more details about this article) |
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