[Leiomyosarcoma of the spermatic cord with paraneoplastic beta-hCG production]. / Leiomyosarkom des Samenstranges mit paraneoplastischer beta-hCG Produktion.

Leiomyosarcomas of the spermatic cord are very rare tumours, which, in contrast to the rhabdomyosarcoms of childhood, occur almost exclusively in adults. We report the case of a 51-year-old male patient with a metastasizing, pleomorphic leiomyosarcoma of the spermatic cord. The tumour showed paraneoplastic secretion of beta-chain human chorionic gonadotropin (beta-hCG), as was demonstrated by the elevated levels of beta-hCG in serum samples and by immunohistochemistry. Histologically, the tumour was a high-grade leiomyosarcoma which showed an aggressive course with pulmonary metastases appearing 4 months after primary surgery. A concomitant rise in the serum levels of beta-hCG was also noted at this time. A wide spectrum of tumours with a choriocarcinomatous component or paraneoplastic production of beta-hCG has been described, the vast majority being carcinomas. Only two leiomyosarcomas producing beta-hCG have so far been reported. The paraneoplastic production of beta-hCG should prevent confusion with germ-cell tumours, especially teratomas.

[Intestinal hamartomatous venous hemangioma in childhood]. / Das intestinale hamartomatöse venöse Hämangiom im Kindesalter.

Hemangiomas of the gastrointestinal tract are very rare in both children and adults. The following case history describes a child with uncharacteristic abdominal pain and stenosis of the terminal ileum. Intraoperatively we found an enlarged hamartomatous alteration of the ileocecal region. The histological analysis showed a hamartomatous venous hemangioma. Problems concerning classification and diagnosis are discussed.

[Proliferating serous papillary cystadenoma of the borderline type in myometrium of the fundus uteri]. / Proliferierendes serös-papilläres Zystadenom vom Borderline-Typ im Myometrium des Fundus uteri.

The 68-year old patient with clinical signs of peritonitis was laparotomized, and a cystic, ruptured structure thought to be an endometriotic cyst was found intraoperatively in the fundus uteri. Histomorphological investigation, however, revealed a proliferating serous papillary cyst-adenoma (borderline tumour) of the myometrium with surrounding, partially atypical endosalpingiosis.

[Treatment of watermelon stomach (GAVE syndrome) with endoscopic argon plasma coagulation (APC). A new therapy approach]. / Behandlung des Wassermelonen-Magens (GAVE-Syndrom) mit der endoskopischen Argon-Plasma-Koagulation (APC). Ein neuer Therapieansatz.

The case of a 74-year-old male patient with a watermelon stomach (GAVE-syndrome: gastric antral vascular ectasia) is reported. The superficial annual mucosa showed the characteristic picture of ectatic capillaries, some of them plugged with fibrin thrombi. Anemia due to chronic blood loss had developed in our patient. The vascular lesions of the antral mucosa were treated endoscopically in three sessions with an argon-plasma-coagulation (APC). Three months after completion of therapy the vascular changes of the antral mucosa had resolved almost completely. In addition no further blood loss had occurred. Many treatment modalities of the watermelon stomach abnormality exist. Nowadays, vaporization of the vascular lesions with the Neodym-Yag laser has widely replaced surgical treatment. The argon-plasma-coagulation uses instead of laser energy conduction of electric energy by ionized argon gas (plasma), which produces coagulation necrosis of tissues. The potential advantages of the argon-plasma-coagulation lie in the limited deep penetration, which reduces the risk of perforation and the symmetric spread of the coagulation effects in the surrounding mucosa. These properties make the argon plasma-coagulation a promising tool for the endoscopic therapy of mucosal lesions of the GI-tract. Further attractive is the low cost of the argon-plasma-coagulation equipment compared with laser devices.

[Involvement of the large intestine in neurofibromatosis type 1]. / Dickdarmbeteiligung bei Neurofibromatose Typ 1.

This report presents the cases of two female patients aged 54 and 57 years, both with colon involvement of type 1 neurofibromatosis. The first woman had a polypoid neuronal hyperplasia containing small Wagner-Meissner corpuscles, which had allowed identification of the neuronal nature of the lesion in a small mucosal biopsy specimen taken previously. The second patient had an idiopathic megacolon of the sigmoid, which had to be extirpated because of acute obstruction and ileus. Morphological examination revealed a typical plexiform neurofibroma of the bowel wall and neuronal hyperplasia of the colonic mucosa and submucosa, which had obviously caused disordered gut mobility leading to functional stenosis and extreme dilatation of prestenotic bowel parts. Gastrointestinal neurofibromatosis is rare and is characterized morphologically by neuronal hyperplasia of the mucosa and submucosa, sometimes containing small aggregates of ganglion cells by which it can easily be identified. However, in the majority of cases the increase of proliferating mucosal nerve fibres can only be confirmed by S-100 protein immunostaining. Furthermore, solitary, multiple and plexiform neurofibromas are found, but only the last, which arises from mesenteric or subserosal nerves, is virtually pathognomonic for neurofibromatosis. Gastrointestinal neurofibromas are usually late manifestations of the disease, but in exceptional cases they can be the initial sign of neurofibromatosis in patients who have no external stigmata that arouse suspicion. The occurrence of gastrointestinal neurofibromas should therefore lead to a careful search for other features of NF-1 in the affected patients and in their families.

Endometrioid adenoma (polypoid endometriosis) of the omentum maius.

Endometrioid adenoma (polypoid endometriosis) of the omentum maius in an 89-year old female is reported. The tumor measured 1 cm in diameter and consisted of resting or weakly proliferating, partially macrocystic endometrial glands, encapsulated by delicate fibrous connective tissue. Immunohistochemically, glandular and stromal cells expressed estrogen receptor strongly, progesterone receptor weakly and CA 125 only weakly in a few glands. This is another, but rare example of the capacity of the omentum maius to develop tumors of the Müllerian variety.

[Juxtapapillary gangliocytic paraganglioma of the duodenum]. / Juxtapapilläres gangliozytisches Paragangliom des Duodenums.

A juxtapapillary duodenal polyp (measuring 1.5 x 1.0 x 1.2 cm) was removed endoscopically in a 57-year-old man who had had recurrent intestinal bleedings. Histological examination revealed a typical duodenal gangliocytic paraganglioma with carcinoid and paraganglioma-like components, as well as neuroid portions with ganglion cells. Various neuroendocrine markers (neuron-specific enolase, chromogranin, protein-gene product 9.5) were demonstrated immunohistochemically. Tests for pancreatic polypeptide, substance P, enkephalin, cholecystokinin, glucagon and S-100 proteins, were all positive.

Trichoblastic fibroma (fibromatoid trichoepithelioma).

Six examples of so-called trichoblastic fibroma occurred in five females and one male. The age of the patients ranged from 46 to 70 years (mean: 61 years). Three tumours were on the back, one each on the left gluteus, right skull and left thigh; they were present one to 20 years and varied in size from 1.8 to 6 cm. The tumour nodules were easily shelled out at operation, and the macroscopic aspect was that of fibroma. Although there seems to be a close relationship to trichoepitheliomas, this tumour deserves a separation from classical conventional trichoepitheliomas and differs from these by size, consistency, involvement of deeper tissues, nodular character, unusual location, age of patients and additional histopathologic findings. In our opinion, these rare tumours may also be classified as fibromatoid trichoepitheliomas.