Visceral lymphangiomatosis of the spleen and adrenal gland: a rare presentation

Introduction

Lymphangiomas are rare, benign, transcompartmental lesions of the organ systems involved, which have to be distinguished from other lesions having similar appearances such as abscesses and cystic metastases. The diagnosis of lymphangiomatosis is based on factors such as multifocality, condition of the underlying parenchyma, attenuation and enhancement pattern. ¹ However, contrast-enhanced computed tomography (CECT) findings of visceral lymphangiomatosis are quite characteristic. The role of imaging is essential for the early and accurate diagnosis of the entity, monitoring lesion progression and potential complications, and to prevent unnecessary surgical interventions.

Case report

A 31-year-old man presented to the surgery department with complaints of insidious onset pain for 4 weeks in the left upper abdomen. Clinical examination revealed tenderness in the left upper quadrant on deep palpation. Ultrasonography and CECT of the abdomen were requested as part of the diagnostic work-up. On ultrasonography, a few multiloculated lesions, the largest measuring 4 × 5 cm, were visualised with septations and internal echoes involving the upper pole, lower pole and hilum of the spleen and showing extension to the left suprarenal region. CECT revealed mild splenomegaly and a bulky left adrenal gland with evidence of multiple cystic lesions demonstrating numerous septations (Figure 1). Based on the imaging findings, a diagnosis of visceral lymphangiomatosis of the spleen and adrenal gland was made. Differential diagnoses that were being considered were abscess, hemangioma and cystic pheochromocytoma. The patient was referred to the department of general surgery for further management where he underwent laparoscopic splenectomy in view of mild splenomegaly. Laparoscopically assisted surgical resection of the left adrenal gland with ligation of the central adrenal vein was performed. Histopathological examination and immunohistochemistry studies of the resected specimen confirmed the diagnosis of visceral lymphangiomatosis. The postoperative recovery period was uneventful, and the patient was discharged after one week without complications. Follow-up CECT at 6 months revealed no findings of recurrence.

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Figure 1.

Axial contrast-enhanced computed tomography image in portal venous phase demonstrating multiple non-enhancing cystic lesions involving the splenic parenchyma and left adrenal gland consistent with features of visceral lymphangiomatosis.

Discussion

Systemic or multifocal lymphangiomatosis is a very uncommon entity. ² Lymphangiomatosis limited to the spleen is rare and involvement of the adrenal gland is even rarer. ³ Most common sites for lymphangiomatosis are the neck, perirenal and pararenal spaces. ⁴ Lymphangiomas are classified as capillary, cavernous and cystic. On unenhanced computed tomography (CT), cystic lymphangiomas are visualised as well-defined hypodense collections with septations and on CECT they demonstrate no vascularity. The prognosis of lymphangiomas is dependent on the organ system involved and the extent of involvement. Lymphangiomas are transcompartmental lesions that can cross adjacent spaces of the organ systems involved. Portal venous hypertension and omental varices are some known associations with systemic lymphangiomatosis. ⁵

Management options for visceral lymphangiomatosis involving the spleen include therapeutic embolisation for focal involvement. While laparoscopic splenectomy is considered as the procedure of choice for diffuse involvement with normal or moderately enlarged spleens, open splenectomy is preferred in cases with gross splenomegaly. ⁶ During surgery, whether laparoscopic or open, the search for accessory spleens is an important step as they might be involved in the pathological process. Pazopanib, a tyrosine kinase inhibitor, may cause stabilisation of lymphangiomatosis through vascular endothelial growth factor (VEGF). ⁷ However, for visceral lymphangiomatosis involving the adrenal gland, surgical resection is currently the only treatment. Although open transabdominal adrenalectomy has been applied for the surgical removal of comparatively large adrenal cystic lymphangioma, laparoscopic resection with minimal invasiveness is becoming the standard management option for all benign adrenal tumors. ⁸ Nevertheless, cystic pheochromocytoma needs to be ruled out before considering surgical options.

Conclusion

Radiologists must be aware of the imaging appearances of this rare entity, and visceral lymphangiomatosis must be considered in the differential diagnosis of non-enhancing lesions involving the adrenal gland and spleen.