[Lung transplantation : Histomorphological diagnosis and clinical aspects]. / Lungentransplantation : Histomorphologische Diagnostik und klinische Aspekte.

The function of pulmonary allografts is regularly impaired by alloimmune reactions with quite variable clinical outcomes, different involved effector cells and molecules, as well as affected anatomical compartments. Acute rejection of grafts after lung transplantation (LuTx) is not only associated with the subsequent development of acute graft dysfunction, but can also contribute - among other immunological and nonimmunological factors - to the development of chronic lung allograft dysfunction (CLAD), which is the main reason for the limited long-term survival after LuTx. In addition to ACR and analogous to other solid organ transplants, the importance of antibody-mediated (humoral) rejection (AMR) in LuTx has also been recognized. There are currently no specific laboratory, radiological, or clinical tests available for either ACR or AMR. Only by the synoptic examination of histopathological changes and interpretation against the background of microbiological, virological, serological, and functional findings, can adequate sensitivity and specificity be achieved in the diagnostics of rejection. In this article, the current criteria for histopathological diagnostics of rejection following LuTx are summarized and the most important differential diagnoses are discussed.

Molecular and clinicopathological analysis of Epstein-Barr virus-associated posttransplant smooth muscle tumors.

Epstein-Barr virus (EBV)-associated posttransplant smooth muscle tumors (PTSMT) are very rare complications. We aimed to provide a clinicopathological characterization which is based on our own case series (n = 5) as well as previously reported PTSMT cases (n = 63). Meta-analysis of PTSMT and molecular analysis of tumor cells from our cohort was performed. Most PTSMT developed in kidney-transplanted patients (n = 41/68, 60%). Liver/transplant liver was the main site of manifestation (n = 38/68, 56%). Tumors occurred after a median interval of 48 months (range 5-348) and developed earlier in children than in adults. Most tumors showed no marked cellular atypia, low mitosis rate and no tumor necrosis. Gene expression analysis of 20 EBV-related genes, including two microRNAs, revealed overexpression of MYC (p = 0.0357). Therapy was mainly based on surgical resection or reduced immunosuppression but no significant differences in overall survival were evident. Lower overall survival was associated with multiorgan involvement (n = 33/68, 48.5%) and particularly with intracranial PTSMT manifestation (n = 7/68, 10%; p < 0.02), but not transplant involvement (n = 11/68, 16%). In summary, PTSMT differ from conventional leiomyosarcomas by their lack of marked atypia, unusual sites of involvement and defining EBV association. Surgery and reduced immunosuppression show comparable clinical results and prognosis is associated with intracranial manifestation.

Multiple inflammatory myofibroblastic tumors involving lung and mediastinum: a rare clinical entity.

OBJECTIVE: Inflammatory myofibroblastic tumors (IMT) are a rare clinical entity. We retrospectively reviewed the clinicopathological characteristics and prognosis for all patients with surgically resected IMTs of the lung at our institution. MATERIAL AND METHOD: From January 1995 through February 2007, 16 patients, 9 men and 7 women ranging in age from 18 to 64 years with a median age of 46 years, were admitted to our hospital for IMT of the lung, mediastinum and thoracic outlet. Nine of them (56.3 %) had a history of pneumonia, while in the rest it was documented as an incidental finding on chest X-ray. Five of our patients (31.3 %) were under immunosuppressive therapy. CT scan was the diagnostic tool routinely used and PET performed turned out to be positive in 5 cases. Wedge resection was performed in the majority of cases along with 2 lobectomies and 2 segmentectomies. The resected lesions were studied histologically and immunohistochemically. There were no operative deaths. Follow-up was complete in all patients and ranged from 9 months to 135 months. No recurrence was observed. RESULTS: Overall 5-year survival was 93.8 %. Fifteen patients are still alive and the cause of death in one case was not related to the pseudotumor. Cox regression analysis was performed for different factors such as age, sex, previous pneumonia and immunosuppression. None of them was found to play a role in the development of an IMT. The type of intervention also did not seem to affect the prognosis in our series. CONCLUSION: IMTs are a rare clinical entity. An accurate preoperative diagnosis is difficult and complete resection remains the treatment of choice and leads to an excellent survival.

Indicators for steroid response in biopsy proven acute graft rejection after lung transplantation.

OBJECTIVE: Acute rejection (AR) after lung transplantation (LTx) requires prompt intervention. Most episodes respond to steroid pulse therapy. The aim of this study was to evaluate clinical indicators for non-response to steroid treatment in biopsy proven AR after the early postoperative period. METHODS: We prospectively analysed 85 patients more than 6 months after LTx with symptomatic biopsy proven AR (>or=grade A1) from Jan. 2005 until Nov. 2007 in a single centre. In 47 patients, AR was steroid-sensitive (group 1), 38 patients did not respond to steroid pulse therapy (group 2). All AR episodes were associated with clinical symptoms. Fifty-seven (67%) were low-grade rejections (ISHLT A1). RESULTS: Independent clinical predictors for steroid response vs. non-steroid response in biopsy proven AR were "days after transplantation" (p=0.01, adjusted hazard ratio (HR) 1.2), "decline in home spirometry slope" (p=0.03, HR 0.97), "adherence to home spirometry" (p=0.05, HR 0.98) and "serum CRP" (p=0.02, HR 0.87). Eight patients (21%) of group 2 developed BOS during the following 6 months. CONCLUSIONS: Early detection of deterioration in graft function seems to be crucial for effective treatment of AR. Home spirometry seems to be useful in detecting early changes in graft function and surveillance protocols could be potentially helpful in predicting patients likely to demonstrate a steroid response.

[Lichen planus esophagitis without skin, genital or oral mucosa involvement]. / Lichenoide Osophagitis ohne Haut-, Genital- oder Mundschleimhautbeteiligung.

Lichen ruber planus is a common skin and mucosal disease, with very rare involvement of the esophagus. We report on a 68-year-old patient suffering from dysphagia, with a reduced general condition and weight loss of 12 kg in the past 6 months due to lichen planus of the esophagus. Treatment by bougienage was very successful. This case report describes a lichen ruber planus of the esophagus without involvement of skin, genital or oral mucosa.

[Post-traumatic osteoid osteoma. Case report and review of the literature]. / Posttraumatisches Osteoidosteom. Fallbericht und Literaturübersicht.

Osteoid osteoma is a painful benign bone neoplasm that is rarely described after trauma but should be suspected. A case of osteoid osteoma 19 years after a tibial fracture is presented. The patient had pain in the tibia for 6 years before the osteoid osteoma was confirmed. He had been operated on twice for suspected osteomyelitis although the clinical symptoms suggested an osteoid osteoma. The radiographic appearance as well as a bone scan confirmed the diagnosis. Removal of the nidus resulted in immediate pain relief. A precise preoperative diagnosis of the lesion based on clinical findings, standard radiographs, high-resolution CT, and bone scan is mandatory. It is important to recognize this uncommon entity to avoid morbidity associated with a prolonged delay in diagnosis.