Granulomatosis with polyangiitis in a patient with polydipsia, facial nerve paralysis, and severe otologic complaints: a case report and review of the literature.

BACKGROUND: Granulomatosis with polyangiitis, formerly known as Wegener granulomatosis, is a necrotizing vasculitis with granulomatous inflammation that belongs to the class of antineutrophil cytoplasmic antibodies-positive diseases. It occurs in a localized and a systemic form and may present with a variety of symptoms. Involvement of the upper respiratory tract is very common, while neurologic, endocrinological, and nephrological dysfunction may occur. CASE PRESENTATION: We describe the case of a 29-year-old Central European male patient presenting with severe bilateral sensorineural hearing loss, otorrhea, and one-sided facial nerve paralysis. The patient was unsuccessfully treated with i.v. antibiotics at another hospital in Berlin, and tympanic tubes were inserted. After presentation to our emergency room, he was hospitalized and further diagnostics started. Increased fluid intake and 12 kg weight gain over the last months were reported. The patient was diagnosed with granulomatosis with polyangiitis and diabetes insipidus. The patient's condition improved after treatment with rituximab. DISCUSSION: A comprehensive PubMed search of all articles with granulomatosis with polyangiitis and diabetes insipidus was conducted to assess which combination of symptoms occurs simultaneously and whether other parts of the pituitary are commonly involved. The 39 selected articles, describing 61 patients, showed that ear-nose-throat involvement occurred most commonly, in 71% of cases. Of patients, 59% had involvement of the anterior pituitary gland, while true panhypopituitarism occurred in 13% of cases. Only one case report featured the same set of symptoms as described herein. CONCLUSION: Granulomatosis with polyangiitis is a highly variable disease, commonly involving the upper airways, but that may present with symptoms solely related to the pituitary gland. Clinicians should have a low threshold to investigate for granulomatosis with polyangiitis in patients with therapy-resistant otorrhea. Patients may present with a complex set of symptoms, and integrating different specialists when additional symptoms occur may lead to faster diagnosis.

Clinically detected gastroenteropancreatic neuroendocrine tumors are on the rise: epidemiological changes in Germany.

AIM: To study the epidemiologic changes of gastroenteropancreatic neuroendocrine tumors (GEP-NET) in Germany, we analyzed two time periods 1976-1988 and 1998-2006. METHODS: We evaluated epidemiological data of GEP-NET from the former East German National Cancer Registry (DDR Krebsregister, 1976-1988) and its successor, the Joint Cancer Registry (GKR, 1998-2006), which was founded after German reunification. Due to a particularly substantial database the epidemiological data from the federal states of Mecklenburg-Western Pomerania, Saxony, Brandenburg and Thuringia, covering a population of more than 10.8 million people, were analyzed. Survival probabilities were calculated using life table analysis. In addition, GEP-NET patients were evaluated for one or more second (non-GEP-NET) primary malignancies. RESULTS: A total of 2821 GEP neuroendocrine neoplasms were identified in the two registries. The overall incidence increased significantly between 1976 and 2006 from 0.31 (per 100.000 inhabitants per year) to 2.27 for men and from 0.57 to 2.38 for women. In the later period studied (2004-2006), the small intestine was the most common site. Neuroendocrine (NE) neoplasms of the small intestine showed the largest absolute increase in incidence, while rectal NE neoplasms exhibited the greatest relative increase. Only the incidence of appendiceal NET in women showed little change between 1976 and 2006. Overall survival of patients varied for sex, tumor site and the two periods studied but improved significantly over time. Interestingly, about 20% of the GEP-NET patients developed one or more second malignancies. Their most common location was the gastrointestinal tract. GEP-NET patients without second malignancies fared better than those with one or more of them. CONCLUSION: The number of detected GEP-NET increased about 5-fold in Germany between 1976 and 2006. At the same time, their anatomic distribution changed, and the survival of GEP-NET patients improved significantly. Second malignancies are common and influence the overall survival of GEP-NET patients. Thus, GEP-NET warrant our attention as well as intensive research on their tumorigenesis.