Splenic cord capillary hemangioma with non-islet cell tumor hypoglycemia: a case report

Abstract

Background: Splenic cord capillary hemangioma is a rare benign vascular lesion classified as a splenic hamartoma. On the other hand, non-islet cell tumor hypoglycemia (NICTH) is one of the rare causes of spontaneous hypoglycemia and is considered to be one of the paraneoplastic syndromes. To the best of our knowledge, this is the first reported case of a splenic cord capillary hemangioma with NICTH.

Case presentation: A 25-year-old male was referred to our hospital with hypoglycemia. Except for his low blood sugar, there were no abnormal findings from laboratory tests, which included an endocrinological examination. Enhanced computed tomography confirmed the presence of a solid mass measuring about 6 cm in the retroperitoneum, and a tumorectomy was performed. During this operation, it became clear that the tumor turned out to be a splenic parenchyma, and as a result, a total splenectomy was performed. Microscopically, we diagnosed this as a cord capillary hemangioma, and through immunohistochemistry, we found that some tumor cells were positive for insulin-like growth factor -II. Fortunately, the hypoglycemia-related symptoms disappeared after surgical resection was performed. The patient is still alive and well without evidence of local tumor recurrence 15 years after the operation.

Conclusions: Splenic cord capillary hemangioma, one of the types of splenic hamartomas, is a very rare benign vascular lesion and might be associated with hypoglycemia thought to be NICTH.

Keywords: Insulin-like growth factor-II; Non-islet cell tumor hypoglycemia; Splenic cord capillary hemangioma; Splenic hamartoma; Splenic tumor.

Fig. 1

Contrast-enhanced computerized tomography (CT) image. Enhanced computed tomography confirmed the presence of a solid mass measuring about 6 cm with well-defined and regular borders in the retroperitoneum. Peak contrast enhancement in a part of the tumor was seen in the delayed phase

Fig. 2

Macroscopic and microscopic findings. (a) Macroscopic findings. The solid tumor (3.5 × 2.7 × 2.5 cm) was encapsulated by spleen parenchyma, and the cut surface was a yellowish white. (b, c) Microscopic findings on hematoxylin and eosin (HE) staining. Each lobular lesion was surrounded by fibrous tissue and filled with blood cells (B HE × 40, C HE× 100). (d) The lesions consist of many vascular channels (HE × 100). Immunohistochemical findings of the lesion. (e) Negativity of capillaries and small veins for CD8 (× 100) and (f) positivity for CD31 (× 100). Only lymphocytes were positive for CD8 (e). (g) Positivity of capillaries for CD34 (× 100). (h) Some tumor cells were positive for IGF-II (× 400). Bar scale, 100 μm. (b-h)

Fig. 3

Immunohistochemical findings: synaptophysin(a), chromogranin A(b), insulin(c) and Ki67(d). Immunohistochemically, the tumor is negative for synaptophysin, chromogranin A and insulin and less than 1% positive for Ki67. Bar scale, 20 μm. (all)