Lung Cancer |

Malignant PEComa of Lung Presenting as an Endobronchial Lesion FREE TO VIEW

Tracy Sambo, MD
Author and Funding Information

Presence St. Joseph, Chicago, IL

Chest. 2015;148(4_MeetingAbstracts):522A. doi:10.1378/chest.2265505
Text Size: A A A
Published online


SESSION TITLE: Lung Cancer Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

PURPOSE: The patient is a 40-year-old otherwise healthy man who developed a cough productive of whitish sputum and intermittent fevers over the four weeks prior to presentation. On evaluation he was noted to have a WBC = 14.3K and mild anemia; a chest radiograph suggested a left lower lobe pneumonia, with question of a mass. A CT scan revealed a 7 cm centrally located mass in the left lower lobe of the lung with an endobronchial lesion. Flexible bronchoscopy with biopsy was performed along with several rounds of laser ablation in an effort to clear the bronchial obstruction. Given the location of the tumor and to achieve a negative margin of surgical resection, a left pneumonectomy was performed. In addition to showing the treatment of the PEComa, this case report demonstrates the importance of considering a PEComa in the differential diagnosis of a pulmonary infiltrate or nodule.

METHODS: Case report - the patient was followed from diagnosis through treatment and in follow up.

RESULTS: Microscopic evaluation of the endobronchial tissue revealed a malignant, cellular tumor composed of spindle and epithelioid cells showing severe nuclear atypia, cytological pleomorphism, high mitotic rate, atypical mitosis, and foci of coagulative tumor necrosis. The cells were negative for S100, HMB 45, CD31, smooth muscle actin, calponin, and desminimmunostains (block B14). Final pathology yielded a poorly differentiated malignant epithelioid and spindle cell neoplasm, consistent with a malignant PEComa.

CONCLUSIONS: Although there are several reports in the literature of PEComa of the female gynecological tract and retroperitoneum, there is a paucity of literature describing malignant PEComa presenting as a solitary lung tumor. Even fewer reports describe the diagnosis of PEComa in the liver, adrenal gland, and other intra-abdominal locations. While generally associated with a gynecologic or intra-abdominal location, it is important to consider this disease process in the differential diagnosis of a pulmonary infiltrate or nodule. The unfortunate location of this tumor at the junction of the left upper and lower lobe bronchi necessitated apneumonectomy for surgical cure.

CLINICAL IMPLICATIONS: This case study illustrates an unusual tumor, the PEComa in an unusual location, the lung. While it is usually associated with a gynecologic or intra-abdominal location, this case shows that it is important to consider this disease process in the differential diagnosis of a pulmonary infiltrate or nodule as well.

DISCLOSURE: The following authors have nothing to disclose: Tracy Sambo

No Product/Research Disclosure Information




Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543