Selected Case From the Arkadi M. Rywlin International Pathology Slide Series

Abstract

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.

Splenic cystic lymphangiomatosis in association with omental varices and portal hypertension

“…Lymphangiomatosis affects somatic soft tissues in the neck, axilla, mediastinum, retroperitoneum, and extremities, [ 1 , 8 , 9 ] and it predominantly affects the neck (75%) and axilla (20%) in a case series involving children. [ 8 ] The lymphangiomatous process may diffusely involve multiple organ systems when the term “systemic” lymphangiomatosis is applied, [ 1 , 2 , 4 ] and it may be confined to a solitary organ, such as the liver, [ 9 ] spleen, [ 10 ] and kidney. [ 11 ] In the latter condition, lymphangiomatosis is called the “isolated” form.…”

“…[ 11 ] In the latter condition, lymphangiomatosis is called the “isolated” form. [ 4 ] It mainly occurs in infants and children and seldom affects patients older than 20 years. [ 12 ] The disease in children is often systemic, while it is mostly isolated in adults.…”

“…[ 12 ] The disease in children is often systemic, while it is mostly isolated in adults. [ 4 ] Visceral lymphangiomatosis involving the spleen is rare, and Bisceglia et al mentioned that <150 cases of both systemic and isolated lymphangiomatosis of the spleen have been reported until the end of 2013. [ 4 ]…”

“…[ 1 , 2 ] It has been suggested that lymphangiomatosis is a congenital dysplasia of lymphatic tissue and is due to the abnormal development of the lymphatic vessels during fetal life. [ 3 ] Since lymphangiomatosis may be asymptomatic or present as a sense of fullness, [ 4 ] splenic cystic lymphangiomatosis is a diseases of little concern. Herein, we present the case of a woman with splenic cystic lymphangiomatosis.…”

Post-hysterectomy intravenous leiomyomatosis: A case of successful multidisciplinary surgery under non-extracorporeal circulation

“…However, a previous study reported that GnRHa therapy following surgery in LPD may prevent the recurrence of new lesions (12). Evidence of long-term efficacy of postoperative treatment in intravenous leiomyomatosis is lacking, and further investigation is required.…”