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

Abstract

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.

Keywords: Pulmonary Langerhans' cell histiocytosis (PLCH); Spontaneous pneumothorax (SP); Thoracic endometriosis syndrome (TES); Video-assisted thoracoscopic surgery (VATS).

Fig. 1

Intraoperative findings. (A) Suspected nodules on the right parietal pleura (arrow) and (B) inter-pleural adhesions and suspected pulmonary lesion (arrow), where a wedge resection was performed. (2-column fitting image).

Fig. 2

H&E and immunohistochemical staining of the resected pulmonary tissue depicting a case of PLCH. All scale bars are represented in the images. (2-column fitting image).

Fig. 3

Perioperative findings. (A) CT thorax images showing suspected hyperdense pleural lesions. All scale bars are represented in the images. (B) Intraoperative finding of the later resected nodular blueish diaphragmatic pleura lesion (arrow). (1.5-column fitting image).

Fig. 4

H&E section of resected specimen of the right diaphragm. Overview showing predominantly scarred tissue with striated muscle remnants in the lower left quadrant (Left). There is an endometrial gland and large gaps visible. There is also evidence of bleeding and endometrial epithelium as well as diffused inflammation. An endometrial gland lined with typical linear endometriotic epithelium and adjacent endometrial stroma along with evidence of bleeding is characteristically visible for endometriosis (Right). In addition, there is extensive scarring and mixed lymphocytic and macrophage-rich inflammatory reaction in the area. All scale bars are represented in the images. (2-column fitting image).