A unique case of thoracic endometriosis syndrome and pulmonary Langerhans' cell histiocytosis: Six recurrent pneumothoraces.

Spontaneous pneumothorax (SP) in women of reproductive age with causes such as thoracic endometriosis syndrome (TES) presents a diagnostic and therapeutic challenge. A 33-year-old women was treated conservatively with chest tube insertion for a first occurrence of a right-sided pneumothorax in September 2015. In January 2016, a right-sided video-assisted thoracoscopic surgery (VATS) wedge resection and partial parietal pleurectomy was performed due to a recurrence. A right-sided VATS was again performed in December 2016 with multiple wedge resections and a total pleurectomy revealing a pulmonary Langerhans' cell histiocytosis (PLCH) in the histological and immunohistochemical examinations. The patient was recommended an abstinence of smoking and further course was unremarkable until May 2019, when due to a recurrent pneumothorax, she received a talc pleurodesis via right-sided VATS. Due to yet another recurrence, she underwent a talc slurry pleurodesis over a right sided chest drain. In March 2020 due to recurrence, a right-sided VATS was performed and a blueish nodular lesion was resected from the diaphragm. The histological examination revealed an endometriosis with a diagnosis of TES. Since the patient did not exhibit a temporal relationship between her periods and the onset of pneumothorax symptoms, a final diagnosis of non-catamenial endometriosis-related pneumothorax was made. The patient is currently continuing smoking abstinence and is under hormone therapy. She has not presented with a recurrence. In clinical practice, it is important not to just relay on the information available to us, but to reevaluate the patient history to uncover new clues leading to a new diagnosis.

Difficult diagnosis and rare morphology of lymphangioleiomyomatosis with giant cysts.

We report on a 32-year-old woman with a second manifestation of a tension pneumothorax two weeks after drainage therapy. The chest CT-scan revealed multiple large bilateral pulmonary cysts. She underwent minimally invasive wedge resection and pleurectomy for treatment. The extensive histologic evaluation revealed the diagnosis of a lymphangioleiomyomatosis (LAM) with an uncommon pattern of lung cysts. Initial staining for HMB-45 was negative. Repeated evaluation of other sections and reference pathology examination detected minimal expression of HMB-45. This case illustrates that immunohistochemistry for HMB-45 may be negative, although LAM is present and repeated immunohistochemistry may be necessary to establish the correct diagnosis.

De novo expression of fetal ED-A(+) fibronectin and B (+) tenascin-C splicing variants in human cardiac allografts: potential impact for targeted therapy of rejection.

Management of acute and especially chronic rejection after human cardiac transplantation is still challenging. Chronic rejection, represented by allograft vasculopathy (CAV) and cardiac interstitial fibrosis (CIF) is known to cause severe long-term complications. Rejection associated tissue-remodelling entails the reoccurrence of fetal variants of Fibronectin (Fn) and Tenascin-C (Tn-C), which are virtually absent in adult human organs. In a rat model, an extensive re-expression could be demonstrated for ED-A(+) Fn with spatial association to CAV and CIF. Thus, it is of great interest to investigate the cardiac tissue expression and distribution in human samples. From 48 heart transplanted patients, 64 tissue specimens derived from right ventricular biopsies were available. Histopathological analysis was performed according to the International Society for Heart and Lung Transplantation (ISHLT) guidelines for the detection of acute rejection. By immunohistochemistry, protein expression of ED-A(+) Fn, B(+) Tn-C, alpha-smooth muscle actin, CD31 and CD45 was assessed and analysed semiquantitatively. Co-localisation studies were performed by means of immunofluorescence double labelling. Histopathological analysis of the 64 samples revealed different ISHLT grades (0R in 36 cases, 1R in 20 cases and 2R in 8 cases). There was a distinct and quantitatively relevant re-occurrence of ED-A(+) Fn and B(+) Tn-C in most samples. Semi-quantitative evaluation did not show any correlation to the acute rejection grade for all markers. Interestingly, significant correlations to the extent of inflammation could be shown for ED-A(+) Fn (r = 0.442, p = 0.000) and B(+) Tn-C (r = 0.408, p = 0.001) as well as between both proteins (r = 0.663, p = 0.000). A spatial association of ED-A(+) Fn and B(+) Tn-C to CAV and CIF could be demonstrated. A relevant re-occurrence of ED-A(+) Fn and B(+) Tn-C following human heart transplantation could be demonstrated with spatial association to signs of rejection and a significant correlation to tissue inflammation. These data might contribute to the identification of novel biomarkers reflecting the rejection process and to the development of promising strategies to image, prevent or treat cardiac rejection.