Journal of Pathology and Translational Medicine

Original Article

Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases: Dae Hyun Song, In Ho Choi, Sang Yun Ha, Kang Min Han, Jae Jun Lee, Min Eui Hong, Yoon-La Choi, Kee-Taek Jang, Sang Yong Song, Chin A Yi, Joungho Han; Korean J Pathol. 2014;48(3):188-192. Published online June 26, 2014; DOI: https://doi.org/10.4132/KoreanJPathol.2014.48.3.188

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Background

Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease that predominantly affects females. Usually, LAM affects the lung; it can also affect extrapulmonary sites, such as the mediastinum, the retroperitoneum, or the lymph nodes, although these locations are rare. A localized form of LAM can manifest as extrapulmonary lesions; this form is referred to as extrapulmonary lymphangioleiomyoma (E-LAM). Due to the rare occurrence of E-LAM and its variable, atypical location, E-LAM is often difficult to diagnose. Herein, we report the clinicopathological information from four E-LAM cases, and also review previous articles investigating this disease.

Methods

Four patients with E-LAM were identified at the Samsung Medical Center (Seoul, Korea) from 1995 to 2012. All E-LAM lesions underwent surgical excision.

Results

All patients were females within the age range of 43 to 47 years. Two patients had para-aortic retroperitoneal masses, while the other two patients had pelvic lesions; two out of the four patients also had accompanying pulmonary LAM. In addition, no patient displayed any evidence of tuberous sclerosis. Histologically, two patients exhibited nuclear atypism with cytologic degeneration.

Conclusions

E-LAM should be considered in the differential diagnosis of patients presenting with pelvic or para-aortic masses. We also conclude that further clinical and pathological evaluation is needed in patients with E-LAM and nuclear atypism.

Citations

Citations to this article as recorded by

Surgical Management of Solitary Extrapulmonary Lymphangioleiomyomatosis in the Mesentery: A Case Report
Jack Menzie, Chih C Kuan, Travis Ackermann, Yeng Kwang Tay
Cureus.2024;[Epub] CrossRef
Lymphangioleiomyomatosis with Tuberous Sclerosis Complex—A Case Study
Aleksandra Marciniak, Jolanta Nawrocka-Rutkowska, Agnieszka Brodowska, Andrzej Starczewski, Iwona Szydłowska
Journal of Personalized Medicine.2023; 13(11): 1598. CrossRef
A case of lymphangioleiomyomatosis with endometrial cancer diagnosed by retroperitoneoscopic para-aortic lymph node dissection
Aiko Ogasawara, Shogo Yamaguchi, Hiroaki Inui, Mieko Hanaoka, Daisuke Shintani, Sho Sato, Masanori Yasuda, Akira Yabuno
JAPANESE JOURNAL OF GYNECOLOGIC AND OBSTETRIC ENDOSCOPY.2022; 38(1): 158. CrossRef
Primary retroperitoneal PEComa: an incidental finding
Bárbara Monteiro Marinho, António Gâmboa Canha, Donzília Sousa Silva, José Davide Pinto Silva
BMJ Case Reports.2022; 15(11): e250466. CrossRef
Imaging Findings of Thoracic Lymphatic Abnormalities
Jingshuo (Derek) Sun, Thomas Shum, Fardad Behzadi, Mark M. Hammer
RadioGraphics.2022; 42(5): 1265. CrossRef
Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient
Lucy Grant, Saliya Chipwete, San Soo Hoo, Anjali Bhatnagar
BMJ Case Reports.2019; 12(2): e226358. CrossRef
Summary of the Japanese Respiratory Society statement for the treatment of lung cancer with comorbid interstitial pneumonia
Takashi Ogura, Nagio Takigawa, Keisuke Tomii, Kazuma Kishi, Yoshikazu Inoue, Eiki Ichihara, Sakae Homma, Kazuhisa Takahashi, Hiroaki Akamatsu, Satoshi Ikeda, Naohiko Inase, Tae Iwasawa, Yuichiro Ohe, Hiromitsu Ohta, Hiroshi Onishi, Isamu Okamoto, Kazumasa
Respiratory Investigation.2019; 57(6): 512. CrossRef
Incidental lymphangioleiomyomatosis in the lymph nodes of gynecologic surgical specimens
Ikumi Kuno, Hiroshi Yoshida, Hanako Shimizu, Takashi Uehara, Masaya Uno, Mitsuya Ishikawa, Tomoyasu Kato
European Journal of Obstetrics & Gynecology and Reproductive Biology.2018; 231: 93. CrossRef
Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review
Weiwei Fu, Yujun Li, Hong Li, Ping Yang, Xiaoming Xing
Experimental and Therapeutic Medicine.2016; 12(3): 1499. CrossRef
Incidental Pelvic and Para-aortic Lymph Node Lymphangioleiomyomatosis Detected During Surgical Staging of Pelvic Cancer in Women Without Symptomatic Pulmonary Lymphangioleiomyomatosis or Tuberous Sclerosis Complex
Joseph T. Rabban, Brandie Firetag, Ankur R. Sangoi, Miriam D. Post, Charles J. Zaloudek
American Journal of Surgical Pathology.2015; 39(8): 1015. CrossRef

Case Reports

Diffuse Leiomyomatosis of the Esophagus: A case report.: Ok Jun Lee, Hwa Sook Jeong, Jong Myeon Hong, Ro Hyun Sung; Korean J Pathol. 1996;30(12):1159-1162.

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Abstract PDF: Diffuse leiomyomatosis of the esophagus is a rare condition and usually extends from the mid-esophagus to the proximal third of the stomach. Macroscopically, there is a marked diffuse thickening of the esophageal wall, with or without nodularity, predominantly affecting the circular muscle coat. Microscopically, the disorder is characterized by the loss of the normal orientation of the smooth muscle fibers of all three layers. We report a case in a 37-year-old woman which was incidentally discovered at exploratory thoracotomy.

Diffuse Leiomyomatosis of the Uterus: A Brief Case Report.: Su Jin Kim, Mee Sook Roh; Korean J Pathol. 2005;39(1):63-65.

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Abstract PDF: Diffuse leiomyomatosis of the uterus is a rare condition that is distinguished from the uterine leiomyoma due to the diffuse involvement of the myometrium by numerous, ill-defined, smooth muscle nodules. We present here a case of diffuse uterine leiomyomatosis in a 34-year-old woman. The hysterectomy revealed a symmetrically enlarged uterus containing numerous, small, ill-defined leiomyomatous nodules. Microscopically, the nodules were composed of compact fascicles and interweaving bundles of uniform benign smooth muscle cells. On the immunohistochemical staining, the progesterone receptor level was higher in the leiomyomatosis than in the adjacent normal myometrial tissue, but the estrogen receptor level and Ki-67 labeling index were equal in both areas. At the twelve months follow-up, this patient has been doing very well with no evidence of pelvic or intraabdominal recurrence of disease.

Original Article

Pulmonary Lymphangioleiomyomatosis: Pathologic Analysis of Eight Korean Cases.: Seung Sook Lee, Jeong Wook Seo, Eul Keun Ham, Yong Il Kim, Nam Hee Won, Jung Gi Im, Young Soo Shim; Korean J Pathol. 1994;28(4):358-367.

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Abstract PDF: Histopathology of pulmonary lymphangioleiomyomatosis(LAM) is studied using four new cases and six previously reported cases, which include two cases without definite evidence of LAM. The important diagnostic features of this lesion were nodular proliferation of immature smooth muscle and cleft or cyst formation within the nodules of smooth muscle cells. The nuclei of the smooth muscle cells were bigger than those of blood vessels or fibrotic lung, and the direction of nuclei was irregular. The lung parenchyma showed little inflammatory change but there were multiple air cysts with smooth muscle nodules at their margin. There were two cases with exuberant proliferation of smooth muscle nodules and two cases with papilliferous projections of the cells into lymphatic lumen. Whereas, three cases had only a few small slender nodules of smooth muscle cells at the margin of air cyst. The lymphatic lumen with smooth muscle nodules is dilated in four cases but other four cases show collapsed lumen. Pulmonary hemorrhage and hemosiderosis were prominent in three cases. There were variety of histology in terms of the cellularity of smooth muscle nodules, the size of the lymphatic lumen and the degree of pulmonary destruction, which may have significance on the clinical presentation and prognostication.

Case Report

Pulmonary Lymphangioleiomyomatosis: A case report.: Won Bo Jo, Nam Hee Won, Seung Yong Paik, Hae Kyung Ahn; Korean J Pathol. 1991;25(3):269-274.

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Abstract PDF: Lymphangioleiomyomatosis(LAM) is a rare disease of women of child-bearing age in which there is progressive hyperplasia of atypical smooth mucle along lymphatics in the lung, and/or axial lymphatics in the thorax and abdomen, resulting in honeycombing of lung. Interestingly there has been a speculation that it represents a forme furste or incomplete expression of tuberous sclerosis complex. This is based on the observation that patients with tuberous sclerosis can manifest pulmonary lesions indistinguishable from LAM. We report a case of LAM occuring in a 39-year-old female, who complained of recurrent pneumothorax, chest pain and shortness of breath. Three years ago, the patient had right nephrectomy under the diagnosis of ruptured angiomyolipoma. A X-ray film of the chest showed honeycombing with a diffusely reticulonodular pattern and cyst-like spaces. She had a characteristic facial appearance of adenoma sebaceum, which her father and uncle had. Microscopically, the lung showed a marked smooth muscle proliferation around the slit-like lymphatic spaces and also some respiratory bronchioles.

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