Spontaneous Hemothorax of Tumoral Origin Complicating a Von Recklinghausen's Disease: Report of Two Cases in Cameroon and Litterature Review

Author(s): LM Endale Mangamba, AD Balkissou, V Tongue Essoh, AD Batchom, B Barche, JP Engbang Ndamba, BH Mbatchou Ngahane

Von Recklinghausen's disease also known as neurofibromatosis type 1 (NF1), is one of the most common genetic diseases. It is responsible of the development of neurofibromas from nerve sheaths and very typical skin lesions. Chest damage according to the literature is rare. Spontaneous hemothorax is a subcategory of hemothorax that involves the accumulation of blood in the pleural space in the absence of trauma or other causes. This clinical entity secondary to vascular involvement is rarely described in the literature.

We present the cases of 2 young patients, with neurofibromatosis type 1 (NF1), without dermatological follow-up, or any contributive past medical history, with both massive spontaneous hemothorax. The first is a 26-year-old female nurse student, who consult for rapid worsening dyspnea over a week, associated with a dry and painful cough. She had no fever, no weight loss. Physical examination objectified NF1 lesions with coffee-milk stain type of skin neurofibromas, massive left fluid pleural effusion confirmed by imaging with a tumor of the posterior mediastinum. The second patient was 37 years old man, unemployed stable at the admission, also have a voluminous plexiform neurofibroma. Their evolution after pleural drainage was favorable. Patient with neurofibromatosis type1should be follow by dermatologist and refer in case of thoracic involvement. Spontaneous hemothorax are not exceptional, and can be associated with a tumor.

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