Unicentric mesenteric Castleman's disease with littoral cell angioma, anemia, growth retardation and amenorrhea: A case report.

Ling Yang,Rong-Bo Liu,Shuang Zhao

doi:10.3892/ol.2015.2933

Ling Yang, Rong-Bo Liu + Show 1 more

Open Access

https://doi.org/10.3892/ol.2015.2933

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Abstract

Castleman’s disease (CD) is a rare lymphoproliferative disorder of unknown origin, and littoral cell angioma (LCA) is a rare vascular tumor of the spleen with an unknown etiology. The current study reports the case of a 28-year-old female who presented with anemia, growth retardation and amenorrhea. Physical examination revealed a mass in the mesentery, splenomegaly with multiple small nodules, hepatomegaly and an infantile uterus. Histopathological analysis of the resected mass and spleen confirmed the diagnosis of hyaline-vascular CD and LCA. The patient’s anemia resolved, and menstruation and breast development also commenced following surgery. To the best of our knowledge, this is the first report of CD accompanied by littoral cell angioma, anemia, growth retardation and amenorrhea.

Highlights

Castleman's disease (CD) is a rare lymphoproliferative disorder with unknown origin
CD was initially first described in 1954 and is known as angiofollicular hyperplasia or giant lymph node hyperplasia; it is a rare disorder of lymphoid tissue (7)
CD may occur in any area in which lymphoid tissue is normally found, it most commonly occurs in the mediastinum (70%)

Summary

Introduction

Castleman's disease (CD) is a rare lymphoproliferative disorder with unknown origin It is not frequently identified in the abdomen, it can occur in any region containing lymph nodes (1,2). The current study describes the clinical, laboratory, radiological and histological findings, and the successful treatment of a patient with concurrent CD and LCA. Moderate hepatomegaly, splenomegaly and a nontender mass in the hypogastrium were observed. A moderately enhanced, well‐defined mass of 4.8x5.6 cm in size was detected at the mesentery on computed tomography (CT) imaging; centrally located ring‐shaped calcification could be observed. As the underlying nature of the mass, nodules of the spleen and hepatomegaly could not be determined in this patient, exploratory laparotomy was performed to remove the mass and spleen, with additional liver biopsy. The patient's symptoms resolved and laboratory tests normalized. At the time of writing, no recurrence had been identified

Discussion

Findings

Castleman B and Towne VW