Skip to main content
Top
Published in: BMC Cancer 1/2016

Open Access 01-12-2016 | Case report

A case of lymphangioleiomyomatosis associated with endometrial cancer and severe systemic lupus erythematosus

Authors: Kensuke Suzuki, Kazunori Nagasaka, Katsutoshi Oda, Hiroyuki Abe, Daichi Maeda, Yoko Matsumoto, Takahide Arimoto, Kei Kawana, Masashi Fukayama, Yutaka Osuga, Tomoyuki Fujii

Published in: BMC Cancer | Issue 1/2016

Login to get access

Abstract

Background

Lymphangioleiomyomatosis (LAM) is a rare idiopathic disorder that occurs in women of childbearing age, and consists of a diffuse proliferation of abnormal smooth muscle cells along the thoracic and abdominal lymphogenous route.

Case presentation

We experienced a case of a 47-yo woman with recent history of systemic lupus erythematosus (SLE) diagnosed with endometrial cancer, initially suspected to have metastasized to pelvic and para-aortic lymph nodes based on preoperative diagnostic imaging. Subsequent pathological diagnosis revealed stage IB endometrial cancer without evidence of lymph node involvement. Instead, enlarged pelvic and para-aortic lymph nodes were found to be due to extrapulmonary LAM, from a primary lesion found inside the uterine myometrium. SLE improved after surgery.

Conclusion

This is the first reported case of comorbid endometrial cancer, SLE, and aggressive LAM metastasizing to regional lymph nodes, and strengthens the clinical evidence for a common role of mTOR pathway hyperactivity and estrogen responsiveness in the pathophysiology of metastasizing lesions of the genital tract.
Literature
1.
go back to reference Moss J, Avila NA, Barnes PM, Litzenberger RA, Bechtle J, Brooks PG, Hedin CJ, Hunsberger S, Kristof AS. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med. 2001;164:669.CrossRefPubMed Moss J, Avila NA, Barnes PM, Litzenberger RA, Bechtle J, Brooks PG, Hedin CJ, Hunsberger S, Kristof AS. Prevalence and clinical characteristics of lymphangioleiomyomatosis (LAM) in patients with tuberous sclerosis complex. Am J Respir Crit Care Med. 2001;164:669.CrossRefPubMed
2.
go back to reference Gu X, Yu JJ, Ilter D, Blenis N, Henske EP, Blenis J. Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis. Proc Natl Acad Sci U S A. 2013;110:14960–5.CrossRefPubMedPubMedCentral Gu X, Yu JJ, Ilter D, Blenis N, Henske EP, Blenis J. Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis. Proc Natl Acad Sci U S A. 2013;110:14960–5.CrossRefPubMedPubMedCentral
4.
go back to reference Sandrini A, Silverstone E, Yates DH. Menstrual cycle variation of retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis. Intern Med J. 2011;41:832–5.CrossRefPubMed Sandrini A, Silverstone E, Yates DH. Menstrual cycle variation of retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis. Intern Med J. 2011;41:832–5.CrossRefPubMed
5.
go back to reference Matsui K, Tatsuguchi A, Valencia J, Yu ZX, Bechtle J, Beasley MB, Avila N, Travis WD, Moss J, Ferrans VJ Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol. 2000;31:1242–8.CrossRefPubMed Matsui K, Tatsuguchi A, Valencia J, Yu ZX, Bechtle J, Beasley MB, Avila N, Travis WD, Moss J, Ferrans VJ Extrapulmonary lymphangioleiomyomatosis (LAM): clinicopathologic features in 22 cases. Hum Pathol. 2000;31:1242–8.CrossRefPubMed
6.
go back to reference Iwasa Y, Tachibana M, Ito H, Iwami S, Yagi H, Yamada S, Okagaki A, Ban C, Mano M, Kodama Y, Ueda M. Extrapulmonary lymphangioleiomyomatosis in pelvic and paraaortic lymph nodes associated with uterine cancer: a report of 3 cases. Int J Gynecol Pathol. 2011;30:470–5.CrossRefPubMed Iwasa Y, Tachibana M, Ito H, Iwami S, Yagi H, Yamada S, Okagaki A, Ban C, Mano M, Kodama Y, Ueda M. Extrapulmonary lymphangioleiomyomatosis in pelvic and paraaortic lymph nodes associated with uterine cancer: a report of 3 cases. Int J Gynecol Pathol. 2011;30:470–5.CrossRefPubMed
7.
go back to reference Longacre TA, Hendrickson MR, Kapp DS, Teng NN. Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. Gynecol Oncol. 1996;63:404–10.CrossRefPubMed Longacre TA, Hendrickson MR, Kapp DS, Teng NN. Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. Gynecol Oncol. 1996;63:404–10.CrossRefPubMed
8.
go back to reference Yamashita S, Nakamura K, Shinozaki H, Minegishi T. Lymphangioleiomyomatosis suspected to be a gynecologic disease. J Obstet Gynaecol Res. 2011;37:267–9.CrossRefPubMed Yamashita S, Nakamura K, Shinozaki H, Minegishi T. Lymphangioleiomyomatosis suspected to be a gynecologic disease. J Obstet Gynaecol Res. 2011;37:267–9.CrossRefPubMed
9.
go back to reference Gopinath D, Attarbashi S, Reid F, Seif M. Extrapulmonary lymphangioleiomyomatosis complicated by vesicovaginal fistula. J Obstet Gynaecol. 2013;33:910–1.CrossRefPubMed Gopinath D, Attarbashi S, Reid F, Seif M. Extrapulmonary lymphangioleiomyomatosis complicated by vesicovaginal fistula. J Obstet Gynaecol. 2013;33:910–1.CrossRefPubMed
10.
go back to reference Vatansever D, Sozen H, Iyibozkurt AC, Comba C, Yavuz E, Topuz S. Extrapulmonary lymphangioleiomyomatosis mimicking lymphoma metastatic to uterus. J Obstet Gynaecol Res. 2015;41:823–7.CrossRefPubMed Vatansever D, Sozen H, Iyibozkurt AC, Comba C, Yavuz E, Topuz S. Extrapulmonary lymphangioleiomyomatosis mimicking lymphoma metastatic to uterus. J Obstet Gynaecol Res. 2015;41:823–7.CrossRefPubMed
11.
go back to reference Hayashi T, Koike K, Kumasaka T, Saito T, Mitani K, Terao Y, Ogishima D, Yao T, Takeda S, Takahashi K, Seyama K. Uterine angiosarcoma associated with lymphangioleiomyomatosis in a patient with tuberous sclerosis complex: an autopsy case report with immunohistochemical and genetic analysis. Hum Pathol. 2012;43:1777–84.CrossRefPubMed Hayashi T, Koike K, Kumasaka T, Saito T, Mitani K, Terao Y, Ogishima D, Yao T, Takeda S, Takahashi K, Seyama K. Uterine angiosarcoma associated with lymphangioleiomyomatosis in a patient with tuberous sclerosis complex: an autopsy case report with immunohistochemical and genetic analysis. Hum Pathol. 2012;43:1777–84.CrossRefPubMed
12.
go back to reference Hirasawa A, Sato T, Ueno M, Akahane T, Susumu N, Betsuyaku T, Aoki D. Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. J Clin Oncol. 2014;26:1341–5. Hirasawa A, Sato T, Ueno M, Akahane T, Susumu N, Betsuyaku T, Aoki D. Distinguishing between lymphangioleiomyomatosis and carcinomatous peritonitis in a patient with ovarian cancer. J Clin Oncol. 2014;26:1341–5.
13.
go back to reference Wan YL, Shih LY, Ko SF, Kuo MC, Ng SH. Imaging findings of retroperitoneal lymphangiomyomatosis in a patient with lymphoma. Clin Imaging. 2006;30:218–20.CrossRefPubMed Wan YL, Shih LY, Ko SF, Kuo MC, Ng SH. Imaging findings of retroperitoneal lymphangiomyomatosis in a patient with lymphoma. Clin Imaging. 2006;30:218–20.CrossRefPubMed
14.
go back to reference Inubashiri E, Hata K, Kanenishi K, Shiota A, Ohno M, Yamamoto Y, Nishiyama Y, Ohkawa M, Hata T. Positron emission tomography with the glucose analog [F]-fluoro-2-deoxy-D-glucose for evaluating pelvic lymph node metastasis in uterine corpus cancer: comparison with CT and MRI findings. J Obstet Gynaecol Res. 2009;35:26–34.CrossRefPubMed Inubashiri E, Hata K, Kanenishi K, Shiota A, Ohno M, Yamamoto Y, Nishiyama Y, Ohkawa M, Hata T. Positron emission tomography with the glucose analog [F]-fluoro-2-deoxy-D-glucose for evaluating pelvic lymph node metastasis in uterine corpus cancer: comparison with CT and MRI findings. J Obstet Gynaecol Res. 2009;35:26–34.CrossRefPubMed
15.
go back to reference Park JY, Kim EN, Kim DY, Suh DS, Kim JH, Kim YM, Kim YT, Nam JH. Comparison of the validity of magnetic resonance imaging and positron emission tomography/computed tomography in the preoperative evaluation of patients with uterine corpus cancer. Gynecol Oncol. 2008;108:486–92.CrossRefPubMed Park JY, Kim EN, Kim DY, Suh DS, Kim JH, Kim YM, Kim YT, Nam JH. Comparison of the validity of magnetic resonance imaging and positron emission tomography/computed tomography in the preoperative evaluation of patients with uterine corpus cancer. Gynecol Oncol. 2008;108:486–92.CrossRefPubMed
16.
go back to reference Hohman DW, Noghrehkar D, Ratnayake S. Lymphangioleiomyomatosis: a review. Eur J Intern Med. 2008;19:319–24.CrossRefPubMed Hohman DW, Noghrehkar D, Ratnayake S. Lymphangioleiomyomatosis: a review. Eur J Intern Med. 2008;19:319–24.CrossRefPubMed
17.
go back to reference Sabatini DM. mTOR and cancer: insights into a complex relationship. Nat Rev Cancer. 2006;6:729–34.CrossRefPubMed Sabatini DM. mTOR and cancer: insights into a complex relationship. Nat Rev Cancer. 2006;6:729–34.CrossRefPubMed
18.
go back to reference Oda K, Ikeda Y, Kawana K, Osuga Y, Fujii T. mTOR signaling in endometrial cancer: from a molecular and therapeutic point of view. Curr Obstet Gynecol Rep. 2015;4:1–10.CrossRef Oda K, Ikeda Y, Kawana K, Osuga Y, Fujii T. mTOR signaling in endometrial cancer: from a molecular and therapeutic point of view. Curr Obstet Gynecol Rep. 2015;4:1–10.CrossRef
19.
go back to reference Fernandez D, Perl A. mTOR signaling: a central pathway to pathogenesis in systemic lupus erythematosus? Discov Med. 2010;9:173–8.PubMedPubMedCentral Fernandez D, Perl A. mTOR signaling: a central pathway to pathogenesis in systemic lupus erythematosus? Discov Med. 2010;9:173–8.PubMedPubMedCentral
21.
go back to reference Kitajima K, Murakami K, Yamasaki E, Fukasawa I, Inaba N, Kaji Y, Sugimura K. Accuracy of 18F-FDG PET/CT in detecting pelvic and paraaortic lymph node metastasis in patients with endometrial cancer. AJR Am J Roentgenol. 2008;190:1652–8.CrossRefPubMed Kitajima K, Murakami K, Yamasaki E, Fukasawa I, Inaba N, Kaji Y, Sugimura K. Accuracy of 18F-FDG PET/CT in detecting pelvic and paraaortic lymph node metastasis in patients with endometrial cancer. AJR Am J Roentgenol. 2008;190:1652–8.CrossRefPubMed
Metadata
Title
A case of lymphangioleiomyomatosis associated with endometrial cancer and severe systemic lupus erythematosus
Authors
Kensuke Suzuki
Kazunori Nagasaka
Katsutoshi Oda
Hiroyuki Abe
Daichi Maeda
Yoko Matsumoto
Takahide Arimoto
Kei Kawana
Masashi Fukayama
Yutaka Osuga
Tomoyuki Fujii
Publication date
01-12-2016
Publisher
BioMed Central
Published in
BMC Cancer / Issue 1/2016
Electronic ISSN: 1471-2407
DOI
https://doi.org/10.1186/s12885-016-2413-z

Other articles of this Issue 1/2016

BMC Cancer 1/2016 Go to the issue
Webinar | 19-02-2024 | 17:30 (CET)

Keynote webinar | Spotlight on antibody–drug conjugates in cancer

Antibody–drug conjugates (ADCs) are novel agents that have shown promise across multiple tumor types. Explore the current landscape of ADCs in breast and lung cancer with our experts, and gain insights into the mechanism of action, key clinical trials data, existing challenges, and future directions.

Dr. Véronique Diéras
Prof. Fabrice Barlesi
Developed by: Springer Medicine