2 Cases of a Benign Pulmonary Metastasizing Leiomyoma

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1 DOI: /trd ISSN: (Print)/ (Online) Tuberc Respir Dis 2009;67: CopyrightC2009. The Korean Academy of Tuberculosis and Respiratory Diseases. All rights reserved. 2 Cases of a Benign Pulmonary Metastasizing Leiomyoma 1 Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Departments of 2 Obstetrics and Gynecology, 3 Pathology, 4 Radiology, Korea University Hospital, Seoul, Korea Case Report Eun Joo Lee, M.D. 1, Hye Cheol Jeong, M.D. 1, Sung Yong Lee, M.D. 1, Je Hyeong Kim, M.D. 1, Sang Yeub Lee, M.D. 1, Chol Shin, M.D. 1, Jae Jeong Shim, M.D. 1, Kwang Ho In, M.D. 1, Kyung Ho Kang, M.D. 1, Se Hwa Yoo, M.D. 1, Sang Hoon Lee, M.D. 2, Han-Kyeom Kim, M.D. 3, Yu Whan Oh, M.D. 4 A benign pulmonary metastasizing leiomyoma is a recognized clinical entity that has been infrequently reported in the medical literature. We report two cases of a benign pulmonary metastasizing leiomyoma. A 35-year-old woman who underwent myomectomy and a cesarean section approximately 6 years earlier visited our hospital for further evaluation of incidentally revealed multiple lung nodules. A diagnostic percutaneuous biopsy was performed. Finally she was diagnosed with a benign metastasizing leiomyoma. The patient then received LH-RH and has been followed up since. The other 44-year-old woman presented after an initial radiology evaluation revealed the presence of multiple, small-sized lung nodules. She underwent a right middle lung wedge resection to confirm the diagnosis. Finally she diagnosed with a benign metastasizing leiomyoma. The multiple lung nodules have been followed up closely. Key Words: Benign Metastasizing Leiomyoma; Lung Nodules; Myomectomy; Hysterectomy Introduction A benign metastasizing leiomyoma (BML) is a rare condition that is characterized by the benign histological appearance and presence of well-differentiated smooth muscle cells in the lesions. This condition may present in women of any age with uterine leiomyoma. The patients are usually asymptomatic with the pulmonary lesions generally being discovered incidentally. The lung is the most common site of involvement 1,2. We report two cases of a benign metastasizing leiomyoma in women with a history of uterine leiomyoma. Address for correspondence: Kwang Ho In, M.D., Ph.D. Division of Respiratory and Critical Care Medicine, Department of Internal Medicine, Korea University College of Medicine, 126-1, 5-ga, Anam-dong, Sungbook-gu, Seoul , Korea Phone: , Fax: khin@kumc.or.kr Received: Jun. 28, 2005 Accepted: Aug. 22, 2005 This work was published in the Journal of Lung Diseases Volume 2, Number 1, Page Case Report Case 1 A 35-year-old woman presented after the initial radiology evaluation incidentally revealed the presence of multiple lung nodules. She complained of cough and whitish sputum but she denied dyspnea, hemoptysis or fever. There was no history of malignancy. Her medical history included only myomectomy and a cesarean section performed approximately 6 years earlier. No abnormal sounds were present upon auscultation of the chest. A chest X-ray revealed two lung nodules (Figure 1). A chest CT was subsequently performed, which demonstrated two nodules, one 0.8 cm diameter nodule in the right middle lobe and the another 1.0 cm diameter nodule in the left upper lobe (Figure 2). There was no mediastinal lymphadenopathy. A diagnostic transthoracic needle biopsy was performed and she was finally diagnosed with a benign metastasizing leiomyoma (Figure 3). The patient then received LH-RH and has been followed up since. 551

2 EJ Lee et al: 2 cases of a benign pulmonary metastasizing leiomyoma Figure 1. Chest radiograph shows multiple lung nodules. Figure 2. Chest CT shows two round, well-defined and poorly enhanced nodules. Figure 3. (A) Microscopic examination of a percutaneous needle biopsy specimen of the lung nodule shows intersecting bundles of spindle-shaped cells, which are strongly-positive for (B) smooth muscle actin immunohistochemical stain, and they highly express both (D) estrogen and (E) progesterone. However, the tumor cells are negative for (C) C-kit (CD 117) immunohistochemical stain (A, H&E stain, 400). 552

3 Tuberculosis and Respiratory Diseases Vol. 67. No. 6, Dec Figure 4. Chest radiograph shows multiple, round lung nodules. Figure 5. Chest CT reveals multiple nodules ranging in diameter from a few millimeters to 2 cm. Case 2 A 44-year-old woman presented after an initial radiology evaluation incidentally revealed the presence of multiple, small-sized lung nodules. The patient denied dyspnea, cough, sputum production, hemoptysis or fever and there was no history of a malignancy. Her significant medical history included only a total hysterectomy performed approximately 10 years earlier. No crackles nor rales were present upon auscultation of the chest. A chest X-ray showed multiple lung nodules (Figure 4). CT of the chest was subsequently performed, which demonstrated multiple lung nodules ranging in size from a few millimeters to 2 cm in diameter (Figure 5). However, there was no mediastinal lymphadenopathy, pleural effusion or calcification. A diagnostic percutaneous biopsy was performed. The lesions were mainly suspected of being a benign spindle cell tumor. She underwent a right middle lung wedge resection to confirm the diagnosis and was finally diagnosed with a benign metastasizing leiomyoma (Figure 6). The patient did not receive any further treatment, and the multiple lung nodules have been followed up closely. Discussion Leiomyomas are the most common benign uterine neoplasms of the myometrium. However, a BML is a rare condition characterized by the occurrence of benign smooth muscle tumors. These lesions are most often located in the lung in association with uterine leiomyomatosis. Other sites of involvement include the pelvic lymph nodes, skull base, spine, brain, heart, ret- 553

4 EJ Lee et al: 2 cases of a benign pulmonary metastasizing leiomyoma Figure 6. (A) The resected nodule is clearly demarcated from thesurrounding lung tissue. (B) It shows thetypical features of a leiomyoma with no findings suggestive of malignant potential such as coagulative necrosis, increased mitotic activity or significant atypia. (C) The low cuboidal metaplastic bronchiolar epithelia are invaginated and entrapped between the fascicles of the smooth muscle (H&E stain, A, 40, B, 200, C, 200). roperitoneum etc 1,2. This condition may present in women of any age with uterine leiomyoma. Abramson et al 3 reported that nodules can be observed 3 months to 20 years after a hysterectomy or myomectomy. The clinical course is typically indolent, with the patient mortality commonly occurring from an unrelated disease process. There is little correlation between the extent of the disease and the respiratory symptoms 3. The etiology of metastasizing leiomyomas includes the presence of metastatic disease from an existing leiomyoma and the presence of multicentric leiomyomatous growths 4-6. Estrogen and progesterone receptors have been identified on the lung lesions, which has led to treatment options based on hormonal therapy through surgery or medical oophorectomy. However, surgery or hormonal therapy may not always be indicated. The effects of natural hormonal changes such as pregnancy and menopause have also been associated with the regression of these lesions 7. Almost all patients reported thus far were Caucasians. We report two Oriental patients. One woman had myomectomy performed before the discovery of her pulmonary lesions, and the other underwent a total hysterectomy and myomectomy. Both experienced surgical trauma such as a hysterectomy or myomectomy. These cases further support the higher risk of a benign metastasizing leiomyoma after a surgical procedure on the uterus. In our cases, one woman was closely observed without any management for 5 years. Despite the numerous lung nodules, she has rarely complained of respiratory symptoms such as dyspnea, cough, sputum production and hemoptysis. She has remained stable without any evidence of aggravation. To our knowledge, there are only 2 other cases reported in Korea. Both underwent a hysterectomy due to a benign uterine leiomyoma. After being diagnosed with abenign metastasizing leiomyoma, one was observed and the other was treated with a wedge resection of all the lung nodules 8,9. In conclusion, benign metastasizing leiomyoma is a rare entity that usually affects women after a hysterectomy or myomectomy for leiomyoma. The lesions are usually discovered incidentally and have an indolent clinical course. The lung is the most common site of metastatic involvement. Therefore, benign metastasizing leiomyoma should be considered for any asymptomatic patient presenting with multiple pulmonary nodules and a history of uterine leiomyoma. References 1. Giove S, Scappaticci E, Baldi S, Ricci C, Minetto E. Benign metastasizing leiomyoma of the uterus: case report. Minerva Med 1984;75: Abramson S, Gilkeson RC. Multiple pulmonary nodules in an asymptomatic patient. Chest 1999;116: Abramson S, Gilkeson RC, Goldstein JD, Woodard PK, 554

5 Tuberculosis and Respiratory Diseases Vol. 67. No. 6, Dec Eisenberg R, Abramson N. Benign metastasizing leiomyoma: clinical, imaging, and pathologic correlation. AJR Am J Roentogenol 2001;176: Esteban JM, Allen WM, Schaerf RH. Benign metastasizing leiomyoma of the uterus: histologic and immunohistochemical characterization of primary and metastatic lesions. Arch Pathol Lab Med 1999;123: Houck WV, Broderick TJ, Cohen SA. Cohen NM. Benign metastasizing leiomyoma. Surg Endosc 2002;16: Tietze L, Gunther K, Horbe A, Pawlik C, Klosterhalfen B, Handt S, et al. Benign metastasizing leiomyoma: a cytogenetically balanced but clonal disease. Hum Pathol 2000;31: Arai T, Yasuda Y, Takaya T, Shibayama M. Natural decrease of benign metastasizing leiomyoma. Chest 2000; 117: Kang SA, Choi SI, Kim YA, Kim CJ, Yang DG, Kang JH, et al. A case of benign metastasizing pulmonary leimyoma. Tuberc Respir Dis 2005;58: Hwang JK, Park KY, Park JW, Park JK, Jeong SH, Jeong JB, et al. A case of benign metastasizing leiomyoma in the lung. Tuberc Respir Dis 2000;49: