A case of lymphangioleiomyomatosis originated in the pelvic cavity

Jung-mi, Han; Lee, Kyung‐Hee; Kim, Jong Man; Rhim, Chae Chun; Park, Young-Han; Kang, Jung-Bae; Jeon, Sunyoung

doi:10.3802/jgo.2008.19.3.195

Cited by 17 publications

(17 citation statements)

References 18 publications

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“…Furthermore, 3/6 cases described by Song et al (5) also exhibited concurrent pulmonary LAM and extrapulmonary LAM. However, the present case showed a localized liver mass without a pulmonary manifestation prior to or following the surgical procedure, which is similar to the cases described by Han et al (7) and Singh et al (12).…”

Section: A B C D E Fsupporting

confidence: 91%

“…The primary sites for LAM include the pancreas, retroperitoneum, pelvis, mediastinum, kidney hilus, uterus and mesentery (6). The clinical features of extrapulmonary lymphangioleiomyomatosis are a palpable abdominal mass, abdominal pain, and chylous ascites (7). Differential diagnoses should include lymphoma, schwannoma, paraganglioma at the para-aortic area, and metastatic tumors in the lymph nodes (2).…”

Section: Introductionmentioning

confidence: 99%

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Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review

Fu¹,

Li²,

Li³

et al. 2016

Experimental and Therapeutic Medicine

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Abstract. Lymphangioleiomyomatosis (LAM) is a progressive disorder of unknown etiology that predominantly affects the lungs, and the resulting respiratory failure can be fatal. Extrapulmonary LAM is a rare disease that often occurs concurrently with pulmonary LAM. Usually, extrapulmonary LAM presents as a localized, well-circumscribed mass. Due to the rare occurrence of extrapulmonary LAM and its atypical location, extrapulmonary LAM is often difficult to diagnose prior to surgery. The present study describes the clinicopathological features of primary liver LAM in a 26-year-old woman. To the best of our knowledge, the present study presents the first case of a primary solitary extrapulmonary form of LAM in the liver. IntroductionLymphangioleiomyomatosis (LAM) is a progressive disorder that predominantly affects the lungs, and the resulting respiratory failure can be fatal (1). The typical histology of LAM shows a proliferation of smooth muscle arranged in fascicular, trabecular, and papillary patterns associated with slit-like vascular channels (2). Pulmonary LAM can occur sporadically or in association with the inherited hamartoma syndrome tuberous sclerosis (TSC) (3,4).Extrapulmonary LAM is a rare disease that is usually presented as a localized, well-circumscribed mass (5). The primary sites for LAM include the pancreas, retroperitoneum, pelvis, mediastinum, kidney hilus, uterus and mesentery (6). The clinical features of extrapulmonary lymphangioleiomyomatosis are a palpable abdominal mass, abdominal pain, and chylous ascites (7). Differential diagnoses should include lymphoma, schwannoma, paraganglioma at the para-aortic area, and metastatic tumors in the lymph nodes (2). Due to the rare occurrence of extrapulmonary LAM and its atypical location, extrapulmonary LAM is often difficult to diagnose, especially prior to surgery. To the best of our knowledge, the present study reports the first case of primary LAM presenting as a liver mass. Case reportCase summary. The present study was approved by the Ethics Committee of the Qingdao University Medical College (Qingdao, China). Written informed consent was obtained from the patient's family. In 2009, a 26-year-old woman presented a liver lesion of ~6 cm diameter during a routine examination at Jiaozhou People's Hospital of Qingdao (Qingdao, China). The liver lesion was diagnosed as a liver cyst following a computed tomography (CT) scan of the abdomen. The patient exhibited no clinical symptoms until April 2012. Enhanced abdominal CT scans showed that the mass was located in the left lateral hepatic lobe with 5.7x7 cm enhancement and uneven density (Fig. 1). The radiological differential diagnosis included left lateral hepatic adenoma or focal nodular hyperplasia. Subsequently, a hepatic lobectomy was performed in May, and the patient was discharged after one week.Pathologic findings. Gross examination demonstrated that the liver resection specimen measured 15x10x4 cm. After the specimen was cut longitudinally into 1-cm lobe sections, the liver lobe section exh...

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Section: A B C D E Fsupporting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review

Fu¹,

Li²,

Li³

et al. 2016

Experimental and Therapeutic Medicine

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“…The three primary locations of the E-LAM lesions were the posterior mediastinum, the upper retroperitoneum adjacent to the abdominal aorta, and the pelvic cavity. To the best of our knowledge, only two previous reports have described E-LAM cases in Korea 7,8. The first case of E-LAM was reported by Kim et al7 in 2005, and a second case was reported by Han et al8 in 2008.…”

Section: Discussionmentioning

confidence: 96%

Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases

Song

Choi

et al. 2014

Korean J Pathol

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

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“…The current treatment modality for LAM is primarily based on the antagonism of estrogen action. The fact that LAM usually develops in females of reproductive age, that the cells of LAM have receptors for estrogen and progesterone, and that the disease is exacerbated by pregnancy or administration of estrogen, supports that the etiology of this tumor is closely related to hormones 2 . The most commonly used treatment is intramuscular progesterone injection, which became the standard of care following a case report by Sieker and McCarty 16 in 1988.…”

Section: Discussionmentioning

confidence: 99%

“…This tumor mainly develops in females in the reproductive age. Most cases of this tumor primarily occur in the lungs 2,3 . Extrapulmonary LAM is quite rare and mainly located in the pelvis, mediastinum and retroperitoneum 4 .…”

Section: Introductionmentioning

confidence: 99%

Extrapulmonary lymphangioleiomyoma presenting as a mesenteric cystic mass

Jun

Jang

Han

et al. 2011

Basic and Applied Pathology

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Lymphangioleiomyoma (LAM) is a rare benign tumor characterized by a disorganized smooth muscle cell proliferation within the walls of pulmonary, mediastinal, and retroperitoneal lymphatic vessels. The mesenteric LAM is a quite unusual presentation. A 47-year-old woman was admitted to our hospital with a 7-year history of abdominal mass. A computed tomography showed a well marginated mass with soft tissue attenuation. Laparoscopic excision was performed. The specimen revealed a well circumscribed dark cystic mass. The cut surface showed a thin walled cystic tumor with haphazardly distributed trabeculated septa. Microscopically, the tumor showed variable sized ectatic spaces lined by flattened endothelial cells and surrounded by epithelioid or spindle-shaped cells. The lining cells were positive for D2-40, CD31, and CD34. The surrounding cells were positive for alpha-smooth muscle actin and human melanoma black-45. The final diagnosis was a solitary LAM arising in the mesentery. The patient showed an uneventful postoperative course for 10 months.

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A case of lymphangioleiomyomatosis originated in the pelvic cavity

Cited by 17 publications

References 18 publications

Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review

Solitary extrapulmonary lymphangioleiomyomatosis of the liver: A case report and literature review

Extrapulmonary Lymphangioleiomyoma: Clinicopathological Analysis of 4 Cases

Extrapulmonary lymphangioleiomyoma presenting as a mesenteric cystic mass

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