Rare lung cancers—Primary pulmonary leiomyosarcoma: A case report

Abstract

SummaryPrimary pulmonary sarcomas (PPS) are rare mesenchymal lung cancers, which do not present clinically or radiological different to lung carcinomas. Definite PPS diagnosis can only be made by histological analysis and detailed staging examinations in order to exclude a secondary pulmonary malignancy such as metastatic soft tissue sarcoma or another solid tumour. Here we present the case of a 66-year-old woman with a pulmonary mass infiltrating the diaphragm and the mediastinal adipose tissue, which was identified as leiomyosarcoma. The patient received curative surgery with complete tumour R0 resection. The prognosis of PPS is defined by tumour size, lymph node status and histological grading. Surgery is the mainstay of therapy and there is no definitive indication for adjuvant therapy for R0-resected and lymph-node-negative patients like in our case. However, multimodal therapy approaches such as (neo)adjuvant chemo- and radiotherapy can contribute to improving locoregional tumour control, which is the most important prognostic factor. With our case report we want to raise awareness for pulmonary sarcomas as a relevant proportion of rare lung cancers which have to be kept in mind during the differential diagnosis. Moreover, we aim to discuss the complex and individual interdisciplinary management.

Primary Pulmonary Leiomyosarcoma : an Extremely Rare, Difficult-to-Manage Case of Lung Cancer

“…The risk factors associated with PPL are similar to those of other sarcomas, such as history of radiation, chemical, occupational and environmental exposure. 5,8 The patient in our case had a history of smoking for 15 years with the Brinkman index of 150.…”

“…Pulmonary sarcomas are extremely rare, accounting for less than 10% of all soft tissue sarcomas. 1,2,5 Most of PPS are leiomyosarcomas, followed by fibrosarcomas and hemangiopericytomas. 4 Until 2006, there were only 300 cases of PPS that had been reported in the literature.…”

“…3 Around 25% of PPS patients show manifestation of lymph node metastasis. 5 Smoking is a risk factor for lung cancer, where 1 of 9 heavy smokers are reported to develop lung cancer. 7 In contrast, smoking is rarely found as a risk factor in PPL patients.…”

“…Primary pulmonary leiomyosarcoma is a very aggressive tumor that has the capability to grow and to cause symptoms very quickly. [1][2][3][4][5] We report a case of pulmonary leiomyosarcoma in a 51-year-old male with the chief complaint of cough in the past 2 weeks before admission. This report will discuss how pulmonary leiomyosarcoma is being treated using Carboplatin/Etoposide chemotherapy based on the existing literatures.…”

Rare lung cancers—2021 update on challenges, advances, and opportunities

“…Another rare primary lung tumor is PPS. Dr. Nagl presents the complex and individual interdisciplinary management of a patient with PPS and tries to increase the awareness for PPS [4].…”