Spontaneous massive hemothorax related to a neurofibroma: A case report | |
DOI: 10.5606/e-cvsi.2018.683 | |
Slim Charfi1, Abdessalem Hentati2, Walid Abid2, Imed Frikha2 | |
1Department of Pathology, CHU Habib Bourguiba, Sfax, Tunisia 2Department of Cardiovascular and Thoracic Surgery, CHU Habib Bourguiba, Sfax, Tunisia |
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Keywords: Hemothorax; neurofibroma; neurofibromatosis | |
A 33-year-old woman was admitted due to sudden-onset dyspnea and right-sided chest pain. Imaging studies revealed a right-sided
hemothorax and an hyperdense mass of the posterior mediastinum. A monobloc and complete tumor resection was done by thoracotomy.
The diagnosis of a neurofibroma was confirmed by the pathological exam. A careful examination of the patient did not suggest any signs
of von Recklinghausen's disease. To the best of our knowledge, this is the first case of spontaneous massive hemothorax secondary to a
neurofibroma in non-von Recklinghausen's disease. |
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Spontaneous massive intra-thoracic bleeding is rare
and life-threatening complication. It usually occurs due
to vasculopathy. A concomitant mediastinal neoplasm
related to spontaneous hemothorax is a rare finding.[1]
Herein, we report a case of spontaneous hemothorax
secondary to a neurofibroma of the mediastinum in
non-von Recklinghausen?s disease. |
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CASE PRESANTATION
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A 33-year-old woman with a non-specific medical
history was admitted to our hospital due to suddenonset
dyspnea and right-sided chest pain. Physical
examination revealed tachypnea (respiratory rate:
44/min) and decreased breath sounds in the right lung
base. No other abnormalities were noted. Laboratory
tests showed a low hemoglobin level of 8.6 g/dL.
Coagulation tests were normal. A chest radiograph
revealed a large amount of right-sided pleural effusion
(Figure 1). Thoracic computed tomography (CT)
demonstrated a right-sided hemothorax and an
hyperdense mass of the posterior mediastinum which
was measured 146×126×114 mm in size with an
intense contrast enhancement. A collapse of the right
lung and a mediastinal shift to the left were noted
(Figure 2). Figure 1: A chest radiograph showing large amount of right-sided pleural effusion. A written informed consent was obtained from the patient and a transfusion of three units of blood was done, followed by a thoracotomy. During operation, an abundant hemothorax was noted with a bleeding mass of the posterior mediastinum which extended through an intervertebral foramen. Therefore, a monobloc and complete tumor resection with removal of the pleural hematoma was performed. Pathological examination showed a bilobate and encapsulated mass measuring 14×11×11 cm in size. The cut surface was white-to-yellowish with myxoid and hemorrhagic areas. Histological examination revealed a low cellular spindle cells proliferation with no cellular pleomorphism and mitosis. The stroma was fibrous with myxoid areas. Immunohistochemical study showed that tumor cells were positive for PS100 and vimentin and negative for smooth actin muscle, desmin, and CD34. The diagnosis of a neurofibroma was considered. A careful examination of the patient did not suggest any signs of von Recklinghausen?s disease. In addition, there was no café-au-lait spots, hyperpigmented macules, and axillary or inguinal freckles. Also, there was no history of first-degree relative with neurofibromatosis. The patient was discharged on Day 5. The follow-up was unremarkable. |
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Spontaneous massive hemothorax secondary
to a neurofibroma is a rare and often lethal
complication.[1] Spontaneous massive hemothorax
usually occurs secondary to pulmonary infarction,
arteriovenous fistula, ruptured aneurysm, and
tumors. Numerous benign and malignant tumors
have been blamed for the development of spontaneous massive hemothorax including angiosarcoma,
chondrosarcoma, fibrosarcoma, peripheral
neuroectodermal tumor, teratoma, and neural
tumors.[2-6] Neural tumors includes neurofibromas,
schwannomas, malignant peripheral nerve sheath
tumors, and ganglioneuromas.[6,7] In the literature, all
published cases of spontaneous massive hemothorax
secondary to a neurofibroma have been shown to
be related to von Recklinghausen?s disease.[1] In
this context, this is the first case of spontaneous
massive hemothorax secondary to a neurofibroma in
non-von Recklinghausen?s disease. In conclusion, neurofibroma should be considered among neural tumors causing hemothorax even for patients without von Recklinghausen?s disease.
Declaration of conflicting interests
Funding |
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