Type B1 thymoma in multiple endocrine neoplasia type 1 (MEN-1) syndrome.

AIMS AND BACKGROUND: Multiple endocrine neoplasia (MEN) syndromes include a group of disorders characterized by the neoplastic transformation of two or more endocrine tissues. In type 1 syndrome (MEN-1), pituitary, parathyroid and pancreatic islet tumors are most frequently represented. Thymic neoplasms are also rarely associated, and thymectomy during subtotal or total parathyroidectomy should always be considered. STUDY DESIGN: The authors describe a rare case of a 22-year-old male who presented a type B1 thymoma without myasthenia gravis associated to hyperparathyroidism, corticoadrenal adenoma and three neuroendocrine pancreatic lesions (somatostatinoma, glucagonoma and insulinoma).

[Arterial aneurysms associated with cystic hepato-renal disease]. / Association anévrismes artériels multiples et maladie kystique hépato-rénale.

BACKGROUND: Ehlers Danlos syndrome (type IV) and kidney and liver cyst disease can present a common factor: anomalous biosynthesis of structural collagen and elastic tissue. We present an exceptional case. CASE REPORT: A 62-year-old man complained of pain in the upper left quadrant of the abdomen. Ultrasonography, magnetic resonance imaging and arteriography evidenced an aneurysm of the splenic artery, an aneurysm of the hypogastric artery, multiple cysts in the kidney and liver, and mitral valvulopathy. Treatment was splenectomy with resection of the splenic aneurysm and resection of the iliac aneurysm and iliac-iliac bypass. DISCUSSION: The association of kidney and liver cyst disease with venous gastrointestinal and cardiovascular complications is well known. The simultaneous presence of cysts and peripheral and visceral aneurysms with anomalies of the arterial wall resembling Ehlers Danlos syndrome (type IV) would suggest that these two diseases might result from a common connective tissue anomaly. The underlying mechanism(s) remain unknown.

[Malignant nodular hidradenoma. A clinical case]. / Idradenoma nodulare maligno. Caso clinico.

Malignant clear cell hidradenoma is an uncommon tumor of eccrine sweat gland origin. In contrast to the benign form, malignant clear cell hidradenoma tends to invade the surrounding tissue and has a high incidence of distant metastases. For these reasons a wide surgical excision associated to a locoregional lymph node dissection should be performed alter the initial diagnosis. The case of a 17 year old female with this rare lesion is reported; the literature is reviewed and surgical treatment discussed.

Cystic peritoneal mesothelioma: report of a case.

Peritoneal mesothelioma is a rare disease, especially when it arises in a cystic form with tardive and often nonspecific symptoms. While diffuse neoplasms have an unfavorable prognosis, cystic forms are usually benign. An accurate diagnosis can only be made only with electron microscopy and immunohistochemical studies. A 92-year-old woman with an ultrastructurally ascertained cystic peritoneal mesothelioma was admitted to the hospital's emergency ward, and was considered to be unusual because of the size of the mass and the patient's age. A review of the literature is made, and the clinical and diagnostic aspects of this disease are also discussed.

Baroreflex failure syndrome after bilateral excision of carotid body tumors: an underestimated problem.

Carotid body tumors (CBTs) are relatively rare paragangliomas that develop from neural crest cells at the bifurcation of the common carotid artery. They are generally slow growing and benign. Excision is currently considered the treatment of choice, although vascular and especially neural injuries are still relatively frequent in patients with large or bilaterally resected tumors. The baroreflex failure syndrome (BFS) has recently been identified as a severe, rarely recognized, and certainly underestimated complication after the bilateral excision of CBTs. The present report describes a case of a bilateral CBT followed by BFS and reviews the experiences reported in the literature. In light of the low incidence of malignancy of these tumors, their biologic behavior, their very high rate of cranial nerve palsy, and the occurrence of BFS in bilaterally resected paragangliomas, the current practice of bilaterally removing these tumors is questioned.

[Isolated mesenteric fibromatosis. A clinical case]. / Fibromatosi mesenterica isolata. Caso clinico.

The authors report a case of isolated mesenteric fibromatosis un associated with familial adenomatous polyposis or Gardner's syndrome or prior abdominal surgery. These neoplasms are usually asymptomatic until when the compression of the small or large bowel or the ureter causes symptoms; although they are benign lesions without metastases, local recurrences are very frequent. Surgical removal is the primary treatment; until now no satisfactory results have been obtained with other therapeutic modalities.

Obstructive biliary symptomatology as the first sign of HIV-infection.

A 41-year-old white homosexual man presented with epigastric pain and jaundice. Physical examination showed enlargement of bilateral axillar and left inguinal lymph node, while ERCP and a CT scan suggested interruption of bile flow in the intrapancreatic tract of the common bile duct. An endoprosthesis was positioned in the common bile duct during the ERCP. Blood tests (both ELISA and Western blot techniques) showed positivity for anti-HIV antibodies and a CD4 count of 780/mmc (normal: 900-1,200/mmc). A few days later, a dramatic increase of the size of a lymph node in this right axilla occurred, rapidly reaching 5 cm of diameter. A biopsy was performed at this level, and histological examination revealed a high grade B-cell Burkitt type lymphoma. Bone marrow biopsy was negative, as well as lumbar puncture. Aggressive chemotherapy with adriamycin, cyclophosphamide, bleomycine, eldesine and prednisone, together with intratechal administration of methotrexate, was attempted. However, after a marginal and transient regression, the NHL rapidly progressed and the patient eventually died seven months after the diagnosis of NHL. A post mortem examination confirmed the diagnosis of Burkitt lymphoma of the peripancreatic and axillar lymph nodes, with diffusion to the leptomeninges, subaracnoideal spaces and encephalus. No signs of lymphoma were detected in other nodal or extra nodal areas.

Microscopic endoluminal tumorectomy.

PURPOSE: We herein report our experience with transanal endoscopic microsurgery. The new technique combines an endoscopic view and access of the rectum under gas insufflation via a stereoscopic telescope with all conventional surgical maneuvers such as tissue preparation, coagulation and control of bleeding, irrigation, suction, and, finally, suturing of the parietal defect. METHODS: The main indication of transanal endoscopic microsurgery is the removal of broad-based sessile polyps and excision of early rectal cancers. We performed local excision of pT2, G1-2 adenocarcinomas and excision of advanced rectal cancer in high-risk patients. The reported series includes 35 consecutive patients, who have been enrolled in a prospective clinical trial. Five patients were excluded for different reasons. The patients were submitted to 29 total wall excisions with or without perirectal fat and one mucosectomy. RESULTS: Postoperative histologic examination showed 9 adenomas and 21 adenocarcinomas. Morbidity included 2 (5.6 percent) perioperative and 2 (5.6 percent) late complications. There was no operative mortality and the mean postoperative hospital course was six days. All patients are in follow-up observation with a mean time of 10.3 months. In the group of adenomas and adenocarcinomas, we did not observe local recurrence. CONCLUSIONS: Considering our experience with the overall results reported by other authors, we believe that transanal endoscopic microsurgery is the procedure of choice for the treatment of rectal polyps and early rectal cancers provided strict patient selection criteria are met.

The importance of heterogeneity and of multiple site sampling in the prospective determination of deoxyribonucleic acid flow cytometry.

Multiple fresh specimens from 59 nonsmall cell carcinomas of the lung, 38 carcinomas of the gastric tract and 55 carcinomas of the colon and rectum were analyzed by deoxyribonucleic acid (DNA) flow cytometry (FC) after radical resection to evaluate tumor ploidy as an independent prognostic factor. The minimum follow-up period was five years (range of five to ten years). Aneuploidy was observed in 98.0 percent of carcinomas of the lung, in 70.9 percent of carcinomas of the colon and rectum and in 63.1 percent of carcinomas of the gastric tract. FC DNA heterogeneity, in terms of different number of DNA stem lines or different DNA indices between core and periphery, or both, was found in 50.0 percent of carcinomas of the lung, 47.0 percent of carcinomas of the colon and rectum and in 34.5 percent of carcinomas of the gastric tract. A diploid pattern was more frequently observed in less advanced stages of the gastrointestinal tract. By univariate analysis (Kaplan-Meier), patients with carcinoma of the lung with hypodiploid or hypertetraploid peaks, or both, and aneuploid gastric tumors had poorer prognosis. These differences were only marginally significant. Cox analysis demonstrated that the single most important prognostic variable for predicting the overall survival rate was the stage of disease. Tumor DNA content can be considered a marker of advanced stages, particularly in tumors of the gastrointestinal tract, but there is no evidence that it is an independent prognostic variable able to predict long term survival in patients who have been radically resected.

[Massive metachronous inguinal metastases of carcinoma of the anal margin. A clinical case report]. / Metastasi inguinale massiva metacrona da carcinoma del margine anale. Presentazione di un caso clinico.

The authors report a case of epidermoid carcinoma of the anal margin with repeated metachronous metastases in the inguinal lymph nodes. The treatment of synchronous ad metachronous metastases is briefly discussed through a literature review.

Local recurrence after curative resection for colorectal cancer: frequency, risk factors and treatment.

Analysis of 498 patients with colorectal carcinoma was retrospectively reviewed to evaluate the incidence, risk factors and therapy of local recurrent carcinoma following curative resection. Complete follow-up information was obtained in all but four patients (99.2%). After a median follow up of 42 months, 64 out of 469 (13.6%) patients developed local recurrence (LR). The incidence of LR was higher in rectal than in colon cancer patients (18.3% vs 8.9%) (P less than 0.005). Separate univariate and Cox analyses for rectal patients showed tumor site (P less than 0.02). Dukes stage (P less than 0.002), and adjuvant radiotherapy (P = 0.05) determined risk of LR. For colon cancer patients risk of LR was determined by histological tumor grade (P less than 0.01). Out of 64 patients, 5 (7.8%) underwent radical excision of LR. Forty percent of these survived at 5-year (P less than 0.08). Palliative treatment (radio-chemotherapy) obtained a 5-year survival of 15.3%, with no survivors in no-treatment group. These results suggest that local recurrent colorectal carcinoma remain a difficult treatment problem. More effective combinations of surgery and adjuvant therapy are therefore mandatory to reduce the incidence of local failure in high risk colorectal patients.

Flow cytometric analysis in colorectal carcinoma: prognostic significance of cellular DNA content.

The prognostic value of DNA ploidy status was evaluated prospectively in 70 patients with colorectal carcinoma. Cellular DNA content was measured by flow cytometry from fresh specimens with multiple site sampling. Seventy-five percent of cases exhibited a DNA aneuploid pattern. In a univariate analysis, DNA ploidy status showed a statistically significant correlation with survival (p less than 0.05), weaker than Dukes' stage (p less than 0.001). No correlation was observed between survival and presence of multiple DNA stemlines. In a multivariate analysis, Dukes' stage was the strongest prognostic indicator (p = 0.01) while DNA ploidy status did not show an independent prognostic value. It is concluded that DNA ploidy status is associated with pathological features of aggressive malignancy, but it does not have a determinant role in predicting survival.

[Prognostic factors in stomach cancer: invasion of gastric serosa and lymph node involvement]. / Fattori di prognosi del cancro dello stomaco: invasione della sierosa gastrica e coinvolgimento dei linfonodi.

Concerning prognostic factors in gastric cancer, the Authors analyse the main two influencing long-term prognosis: the depth of penetration of the tumor in the gastric wall and the presence of lymph node metastasis. 194 operated gastric neoplasms were staged, according to TNM classification (U.I.C.C., 1987) and divided into 4 groups: in the first group both the serosa and the lymph nodes were tumor-free; in the second both were involved; in the third only lymph nodes were involved; in the fourth only the serosa was involved. Crossed statistic analysis between groups showed that the only factor that influenced the long-term survival is the node involvement in the patients with tumor-free gastric serosa. These results correspond to most of the others in literature, and point to the connection between these two factors in defining the stage of the disease.

Early gastric cancer.

A clear difference in survival among the patients affected by gastric cancer has been observed worldwide between Early Gastric Cancer (EGC) and Advanced Gastric Cancer (AGC). Optical fiber endoscopy has allowed a sharp increase in the number of diagnoses of EGC since the sixties. Among 182 our patients operated on, 19 [10.4%] had an EGC. A difference in incidence was found between males and females, while no difference was found in the age distribution. The main symptom was epigastric pain; EGCS common findings were ulceration or a flat tumor. Surgical procedures were carried out with the same criteria used for AGC. No difference was found in the tumor location. Local spread was studied according to the Japanese Research Society for Gastric Cancer, while histological type was classified according to Lauren classification. Crude 5-year survival was 80% (8 out of 10), with a mean of 76.8 +/- 13 (SEM) months.

The importance of symptom duration in relation to prognosis of carcinoma of the large intestine.

Between 1971 and 1982, 162 patients underwent surgical treatment for carcinoma of the large intestine; follow-up data was available for 94 per cent of the patients. Tumor stage distribution was homogeneous and did not differ significantly with increasing duration of symptoms. The duration of symptoms was not determinant of survival, whereas stage influenced survival. These results show that patients with carcinoma of the large intestine with a short symptomatic period do not have either less advanced tumors or better survival prospects.