Primary pleural leiomyosarcoma with rapid progression and fatal outcome: a case report

Abstract

IntroductionLeiomyosarcomas are neoplasms of smooth muscles that most commonly arise from the uterus, gastrointestinal tract, or soft tissue. Primary pleural leiomyosarcoma is extremely rare. To the best of our knowledge, only nine cases have been published to date. Because of the rarity of pleural leiomyosarcoma and its similarity (clinical and histological) to other pleural neoplasms, particularly sarcomatous mesothelioma, diagnosis is often difficult.Case presentationA 58-year-old North African man was admitted with complaints of dyspnea and chest pain to our hospital. Chest computed tomography revealed right pleural effusion and pleural thickening. A transthoracic needle biopsy yielded a diagnosis of leiomyosarcoma, and tumor cells were strongly and uniformly positive for vimentin, a smooth muscle actin at immunohistochemical analysis. A general examination did not show any metastatic lesions in other areas. One month after diagnosis, the tumor grew rapidly, with pulmonary invasion, and therefore he was treated only by palliative care. He died from respiratory failure one month later. Because no organ of origin of the leiomyosarcoma, other than the pleura, was detected, this case was diagnosed as a primary pleural leiomyosarcoma.ConclusionsAlthough leiomyosarcoma originating from the pleura is rare, this entity is increasingly described. The purpose of presenting this case report is to raise awareness among clinicians to consider this clinical entity as a differential diagnosis when a pleural mass is identified.

Primary pleural leiomyosarcoma presenting as multiple pleural masses

“…[4,5] The tumor very rarely encases the lung like mesothelioma, but mostly forms a mass with or without pleural effusion. [2][3][4][5] Our case had three separate masses in the parietal pleura, and to the best of our knowledge, this is the first reported case of PPL with multiple foci in the English literature. It is known that leiomyosarcomas mostly spread to the body via hematogenous metastasis.…”

“…[2] Microscopically, leiomyosarcomas are characterized by malignant spindle cells with scant fibrillary cytoplasm arranged in chords and sheets with a variable mitotic activity. Leiomyosarcomas are nearly uniformly positive for smooth muscle actin, desmin, and vimentin, and negative for calretinin, carcinoembryologic antigen, cytokeratin, leukocyte common antigen, neuroendocrine filament, and CD-117.…”

“…[3] It is named PPL if the pleura is the primary site. [2][3][4][5] The pathological examination of diaphragmatic masses in our case demonstrated a PL. The positron emission tomography demonstrated no other primary focus than the apical lesion, thus the final diagnosis was PPL.…”

“…[1] Pleural leiomyosarcomas are unusual, and fewer than 10 cases have been reported. [2] Most PLs are metastatic from uterus, gastrointestinal tract, and retroperitoneum. [3] It is named PPL if the pleura is the primary site.…”

Primitive pleural leiomyosarcoma: case report

“…[1] Only a biopsy of the tumor process can confirm the diagnosis, however, it must be done either by thoracoscopy or thoracotomy. The primitive pleural origin is confirmed after having eliminated all other locations by a thoraco-abdominal-pelvic CT. [2] In pathology, cells are arranged in long beams intersecting at right angles, with abundant eosinophilic cytoplasm and often fibrillar, optionally containing a vacuole notching the kernel. The nucleus is central, rounded in "cigar".…”