Ovarian steroid cell tumor and a contralateral ovarian thecoma in a postmenopausal woman with severe hyperandrogenism.

A 49-year-old woman presented with rapidly progressing hirsutism, receding hairline, male-pattern baldness and deepening of voice, which had developed over the past 2 years. Hormonal evaluation showed a markedly elevated serum testosterone level (418 ng/dl) and no evidence of increased production of cortisol, dehydroepiandrosterone, dehydroepiadrosterone-sulfate, androstenedione, or 17-hydroxyprogesterone. Transvaginal ultrasound examination suggested the presence of a small mass within the left ovary, but all other radiological studies, including adrenal and ovarian computed tomography, magnetic resonance imaging, radio-labelled cholesterol scintigraphy and positron emission tomography, were negative. Subsequently, bilateral selective venous sampling showed a marked testosterone gradient in the right ovarian vein. Bilateral salpingo-oophorectomy was performed (the patient had had a previous vaginal hysterectomy), and histopathological examination revealed a 10-mm steroid cell tumor within the right ovary and a 15-mm thecal cell tumor within the left ovary. The postoperative serum testosterone level returned to normal and the patient showed a slow regression of clinical symptoms. The simultaneous occurrence of a virilizing ovarian steroid cell tumor and an apparently non-functioning thecoma within the contralateral ovary emphasizes the potential pitfalls that may exist in the preoperative evaluation of patients with markedly increased testosterone production.

[Esophageal anastomosis--based on the experience with 1460 operations]. / Nyelócsó anasztomózisokról: 1460 mútét tapasztalata alapján.

The authors reviewed the clinical records of 1460 patients with esophageal anastomoses, the operations performed in their institute between 1973 and 2000. Surgical outcome was assessed with incidence of anastomotic leaks and overall mortality. Hand-sewn anastomoses were performed by inserting single layer interrupted monofil steel wire (507), PDS (232) or Vicryl (65) sutures and circular stapler (EEA, Ethicon) was used in 656 patients. 453 (65 cervical, 293 thoracic, and 95 abdominal) anastomoses performed between 1995 and 2000 were analyzed separately. During this 6-year period, the majority of operations (88%) were performed because of esophageal malignancy (squamous cell carcinoma of the esophagus or adenocarcinoma of the cardia). Surgery was performed for benign disease (e.g. stricture, perforation, and stage IV achalasia) in 12%. The incidence of anastomotic leak was the highest (20%) after cervical anastomosis, compared to a 4.4% or 1% associated with thoracic or abdominal anastomoses. These differences are statistically significant (p < 0.001). The rate of anastomotic leakage was strongly connected to surgical technique: it was 15% in hand-sewn anastomoses and only 4.4% with stapled anastomosis. During the 6-year period overall mortality was 6.6%t.

[Siewert-Stein classification of adenocarcinoma of the esophagogastric junction]. / Az oesophagogastricus junctio adenocarcinomáinak Siewert-Stein klasszifikációjáról.

Nowadays the terminology used for the definition of adenocarcinomas at the oesophagogastric junction is "cardiac carcinoma", which can be easily misunderstood. This definition of adenocarcinomas of the oesophagogastric junction does not allow correct comparison of diagnosis (endoscopic, radiological and pathologic), epidemiology and surgical therapy in national and international aspects, because different tumours can develope in the same area, and all called cardia tumors. Siewert and Stein recommended a classification to solve this problem. The classification of the tumours is morphological/topographical. Type I is adenocarcinoma of the distal part of the oesophagus. Type II is adenocarcinoma of the real cardia and type III is subcardial gastric adenocarcinoma. At classification, we always consider results of endoscopy (ortograde and retroflexed view of the oesophago-gastric junction), the x-rays of the oesophagus and stomach, findings at the operation and pathohistologic results. Between 1/1/1974 and 31/12/2000, a total number of 50,878 upper panendoscopic examinations were performed at the Endoscopic Laboratory of the Surgical Department. Adenocarcinoma of the cardia was diagnosed in 488 patients. According to the Siewert-Stein classification, type I tumour was found in 123 (25.2%), type II in 240 (49.18%), and type III was present in 125 (25.61%) patients. The importance of this classification is it enables unified pre-operative assessment and it can also help to decide the type of the surgical intervention. In our patients with type I cancer--depending of the size of the tumour--distal 2/3 oesophagectomy with the resection of the proximal lesser curve of the stomach or total gastrectomy were performed. In the first group oesophago-jejuno-gastrostomy, in case of total gastrectomy Roux-en-Y loop anastomosis was created. In patients with types II and III cancers total gastrectomy was performed. In every patient lymphadenectomy was performed. We suggest the use of this new classification in clinical, gastroenterology--with special regard to the endoscopy--and pathology.

[Esophageal implantation metastasis from adenocarcinoma of the cardia]. / Kardia adenokarcinóma implantációs metasztázisa a nyelócsóben.

A 74 years old male patient was admitted to our department suffering from dysphagia for five months. Gastric Barium studies showed a cardia tumour with polypoid lesions in the wall of the esophagus, and gastro-esophageal reflux disease was also diagnosed. Endoscopy verified a cardia tumour with esophageal metastasis, and biopsy was obtained from the two lesions. Histology showed that both tumours were adenocarcinomas. Endoscopic ultrasonography classified the cardia tumour as grade T2. As observed during endoscopy, the wall of the esophagus at the level of the polypoid lesion was hypo-echogenic and thick which was result of thickened mucosa. Total gastrectomy and oesophagectomy was performed. Pathology showed that the cardia tumour was pT2N2 and type Siewert-Stein II. The esophageal polypoid lesion was also proved an adenocarcinoma, which was localized only to the mucosa. No tumour cells were found in the blood- or in lymph vessels between the tumour and the esophageal adenocarcinoma. We think that the polypoid adenocarcinoma in the esophagus is an esophageal metastasis implantation from the cardia adenocarcinoma. It is based on the exclusion of other possible tumour dissemination routes. Probably the gastro-esophageal reflux is responsible for the implantation of tumour cells.

[Ret-protooncogene mutation, verified by molecular genetic methods, in a Hungarian MEN Type 2a family]. / Molekuláris genetikai módszerekkel igazolt ret-protoonkogén mutáció magyar MEN2A család esetében.

Multiple endocrine neoplasia Type 2 (MEN2) is a hereditary tumour syndrome characterized by the association of medullary thyroid cancer, phaeochromocytoma and hyperparathyroidism. It is inherited as an autosomal dominant trait. During the past few years the cloning of the gene responsible for the syndrome, the ret protooncogene, made the molecular genetic diagnosis of the disease possible. In this study we demonstrate the results of the MEN2 mutation analysis performed in three members of a Hungarian MEN2A family. The mutation analysis was carried out according to the method of Dr. W. Hoppner's Laboratory (Hamburg) that is the main centre for MEN2 genetic diagnosis in Germany. Two Members of the family are affected, one suffered from both medullary thyroid cancer and phaeochromocytoma, the other (the first patient's daughter) had only medullary thyroid cancer. We found a ret exon 11 codon 634 mutation, that resulted in the change of TGC to TAC, a cysteine-tyrosine amino acid exchange. We found no mutation in the youngest member of the family. This result is of great clinical significance, because the carrier status of this individual can thus be excluded and, therefore, there is no need for prophylactic thyroidectomy and further clinical screening tests. As molecular genetic diagnosis of MEN2 becomes possible, the uncertain clinical examinations used for MEN2 diagnosis seems to be less important.

[Importance of endocrine imaging methods in multiple endocrine neoplasia type 2A proved by mutation analysis]. / Az endokrin szervek képalkotó vizsgálatának jelentösége mutációvizsgálattal igazolt multiplex endokrin neoplasia 2A típusának esete kapcsán.

Multiple endocrine neoplasias are rare, inherited disorders. The authors describe a case history of a patient with multiple endocrine neoplasia type 2A, who presented with unusual clinical manifestations. The diagnosis of phaeochromocytoma, which was the first manifestation of the disorder, was greatly facilitated with radiologic imaging methods. The authors review, on the basis of recent data from the literature, the importance of radiologic methods, which improved due to methodological advance. Finally, the authors emphasize the importance of follow-up for early diagnosis.