Splenic Lymphangiomatosis Research Articles

Massive splenic lymphangiomatosis first diagnosed in pregnancy – a case report

Lymphangiomatosis is a rare disease characterised by multifocal or diffusely infiltrating lymphangioma. Lymphangioma is a benign tumour composed of a collection of endothelial-lined lymphatic spaces filled with serous or chylous material. Lymphangiomatosis can involve the spleen, liver, lungs, bone and skeletal muscle. This condition commonly presents in childhood, although a wide age range is seen.1

Multiple, life-compatible, congenital physical deformities in association with splenic lymphangiomatosis in Zoogoneticus tequila (Webb & Miller, 1998)

Multiple, life-compatible, congenital physical deformities in association with splenic lymphangiomatosis in Zoogoneticus tequila (Webb & Miller, 1998)

A Severe Bleeding Diathesis in a 6-Year-Old Girl Secondary to a Composite Diagnosis of Splenic Hemangiomatosis and Small Bowel Lymphangiomatosis

A 6-year-old girl presented with presumed relapse of childhood immune thrombocytopenia. Investigations revealed deranged coagulation parameters, abnormal small bowel thickening, and splenomegaly. A clinically significant bleeding diathesis emerged which was refractory to most hemostatic interventions. Laparatomy revealed a composite diagnosis of splenic hemangiomatosis and small bowel lymphangiomatosis. Splenectomy resulted in complete resolution of the coagulopathy. The diagnosis and management of these conditions is inherently complex and without clear guidance. We discuss our perioperative management of the bleeding diathesis. There is a need for long-term follow-up of the underlying pathologies particularly as potentially useful therapeutic agents have emerged.

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

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 lymphangiomatosis showing rapid growth during lactation: A case report

Splenic lymphangiomatosis is a very rare condition that, from 1990 to date, has been described only nine times. In the present report, we describe the first case of splenic lymphangiomatosis with rapid growth during lactation in a 35-year-old woman. We also underline the difficultly in making an accurate preoperative diagnosis, despite more modern imaging techniques. Total splenectomy was considered to be the treatment needed, both to make a definitive diagnosis and to exclude the presence of malignancy.

Splenic Lymphangiomatosis with Inflammatory Signs and Elevated Serum Interleukin-6: A Case Report

Lymphangiomatosis is a rare disorder believed to result from a developmental malformation of the lymphatic system. Lymphangiomatosis involves bone, soft tissues, and viscera in a diffuse fashion. We describe an unusual case of splenic lymphangiomatosis that was accompanied by remarkable inflammatory signs. A 38-year-old Japanese woman was admitted to our hospital because of gross splenomegaly. She presented with fatigue, anemia, elevated serum C-reactive protein and polyclonal hypergammaglobulinemia. Her serum concentration of interleukin-6 (IL-6) was also elevated. She was born with lymphangiomatosis in the right leg, which was partially resected during infancy. Because of the risk of traumatic rupture, a total splenectomy was performed two weeks after admission. During postoperative follow-up examinations the patient's inflammatory signs and her serum IL-6 level returned quickly to normal. This could have been caused by a large release of IL-6 from the spleen. We confirmed by immunohistochemistry that IL-6 was produced within the spleen. Our findings indicate a strong association between splenic lymphangiomatosis and IL-6 production.

Splenic lymphangiomatosis in children.

To study the clinical and imaging features of splenic lymphangiomatosis. The clinical and abdominal imaging data of 10 children with splenic lymphangiomatosis were retrospectively reviewed. The modalities used in the study included computed tomography (CT) (10 patients), sonography (five patients), and magnetic resonance (MR) imaging (two patients). Pathologic confirmation of lymphangiomatosis was obtained in nine patients. Splenic lymphangiomatosis was discovered incidentally in all cases and was a key finding in enabling the correct diagnosis in six children with extrasplenic disease as well. Only two children had clinical splenomegaly. Sonograms and MR images showed multiple, wEll-defined cysts. Multiple, low-attenuation lesions that did not enhance with intravenous administration of contrast material (n = 8) or a mottled spleen (n = 2) were seen at CT. One of the mottled spleens had target lesions on an early (arterial) image obtained after administration of a bolus of contrast material. One child underwent a splenectomy; one child underwent therapeutic embolization. Eight patients remained asymptomatic with respect to the spleen 1-20 years later. Splenic lymphangiomatosis is often an incidental imaging finding that frequently has a characteristic imaging appearance. The recognition of this appearance helps in diagnosis of this disease and may prevent the need for further invasive procedures. Splenic changes can be isolated or can coexist with bone or soft-tissue lymphangiomas.

Chylothorax, splenic lymphangiomatosis, and consumptive coagulopathy after surgical treatment of primary chylopericardium

Chylothorax, splenic lymphangiomatosis, and consumptive coagulopathy after surgical treatment of primary chylopericardium

Hepatic and splenic lymphangiomatosis with skeletal involvement: Report of a case and review of the literature: Morris J. Asch, Arthur H. Cohen, and Thomas C. Moore. Surgery 76:334–339 (August) 1974

Hepatic and splenic lymphangiomatosis with skeletal involvement: Report of a case and review of the literature: Morris J. Asch, Arthur H. Cohen, and Thomas C. Moore. Surgery 76:334–339 (August) 1974