Introduction
Hemangiolymphangioma is an exceptionally rare lymphatic and vascular malformation characterized by cystic, dilated veins and lymphatic vessels.1 Extensive involvement of the retroperitoneum and gastrointestinal tract is uncommon. These lesions are primarily congenital, accounting for 40-60% of cases, with an incidence of 1-2 per 10,000 newborns, and are more prevalent in females. They often present clinically in adulthood. Identified risk factors include prematurity, a family history of hemangiolymphangioma, and erythropoietin use.
Hemangiolymphangiomas are predominantly found in the head, neck, and axilla (95%), with only a small percentage (5%) appearing in the mediastinum, retroperitoneum, and mesentery. 2 This article reports a case of hemangiolymphangioma located in the mesentery of an 8-year-old girl, highlighting the pathological features of this case.
Case Report
8 year old girl presented with complaints of abdominal distension since 1.5 years which was insidious in onset, not associated with pain abdomen, puffiness of face, lower limb swelling. Abdominal examination revealed distended abdomen with a soft mass felt in the umbilical region. There was no tenderness and no guarding or rigidity. Laboratory tests did not show any significant abnormalities. CECT abdomen and pelvis were done and showed large mesenteric cyst noted measuring 16 cm x 8.4 cm x 4 cm occupying most of the mid and lower abdomen and part of the pelvis with displacement of the bowel loops as well as the mesenteric vessels.
Figure 2
Low power view- Multiple dilated blood vessels and cystic spaces surrounded by lymphocytes. These are surrounded by smooth muscle bundles and fibrous tissue.
Patient underwent exploratory laparotomy with mesenteric cyst excision under GA. Clear fluid of 2 litre was suctioned out during surgical procedure. Post surgical histopathological examination of the specimen was done. Grossly, received a cut open grey white to grey brown cystic structure measuring 14 x 8 x 2 cm. Thin and thick walls made out (Figure 1). Histopathological examination from multiple thin and thick cyst wall bits showed multiple dilated thick walled blood vessels which are filled with RBCs and also other cystic spaces which are thin walled lined by single layer of low cuboidal epithelium filled with proteinaceous fluid and surrounded by lymphocytes. These vessels are surrounded by smooth muscle bundles and fibrocollagenous tissue (Figure 2, Figure 3). Histopathological features were suggestive of Hemangiolymphangioma. On immunohistochemistry, vascular endothelial cells were positive for CD-31 (Figure 4) and lymphatic endothelial cells showed D2-40 positivity (Figure 5).
Discussion
Hemangiolymphangioma is a rare benign tumor believed to result from congenital vascular system malformations. It is thought to develop from mesenchymal tissue and contains both venous and lymphatic elements in varying amounts. 3 The tumor's formation may be due to a blockage in the communication between the venolymphatic system and the systemic circulation, which affects the dysembryoplastic vascular tissue.4
In the literature, there are various case reports documenting hemangiolymphangiomas occurring in unusual locations such as the spermatic cord, vertebral bodies, oral mucosa, gastrointestinal tract, pancreas, bladder, extremities, paravertebral area, and even the knee. When located in the abdomen, these tumors typically present as space-occupying lesions, causing symptoms such as compression, abdominal pain, or blood in thestool. These symptoms may persist for weeks or months, with the mass growing slowly and progressively. Potential complications include bleeding, perforation, torsion, or rupture of the tumor itself. Differential diagnosis should consider gastrointestinal stromal tumors,pseudomyxoma peritonei, with histopathological analysis being essentialfor accurate diagnosis. 5, 6, 7
The diagnosis begins with a comprehensive clinical history. Due to the rarity of these lesions, preoperative imaging can be difficult; nevertheless, computed tomography (CT) and magnetic resonance imaging (MRI) are useful for evaluating the extent and invasiveness of the lesion, aiding in preoperative surgical planning. Confirmation of the diagnosis is achieved through histopathological examination, which identifies a vascular lesion with both dilated venous and lymphatic vessels. The endothelial cells of these vessels are positive for immunohistochemical markers such as CD31, CD34, and D2-40. 8, 9
The differential diagnosis of mesenteric hemangiolymphangiomas includes several conditions, such as mesenteric cysts, lymphangiomas, mesenteric fibromas, gastrointestinal stromal tumors (GISTs), and vascular malformations. Mesenteric cysts typically present as fluid-filled lesions, whereas hemangiolymphangiomas display a combination of solid and cystic components with distinct vascular structures. Lymphangiomas are characterized by the absence of blood vessels, in contrast to mesenteric hemangiolymphangiomas, which contain both blood and lymphatic vessels. Mesenteric fibromas are solid, fibrous masses, unlike the vascular composition of hemangiolymphangiomas. GISTs appear as solid and homogeneous on imaging, while hemangiolymphangiomas show heterogeneous enhancement. Histopathological analysis demonstrating both blood and lymphatic vessels is essential for distinguishing mesenteric hemangiolymphangiomas from these other conditions. 9
The main approach to treating these lesions is total surgical resection, which typically yields low recurrence rates. There are limited documented cases of mesenteric hemangiolymphangioma with widespread involvement of the gastrointestinal tract. It is advisable to consider hemangiolymphangioma as a potential diagnosis in patients presenting with compressive abdominal symptoms, especially after excluding other benign and malignant neoplasms. 10, 11
Conclusion
Hemangiolymphangioma of the mesentery is a rare vascular malformation characterized by an abnormal proliferation of blood vessels and lymphatics. Understanding the pathophysiology of hemangiolymphangioma is crucial for effective management, as this condition can present similarly to other mesenteric masses. 1, 2 Ultimately, a multidisciplinary approach, involving surgeons, radiologists, and pathologists, is essential for addressing this complex vascular anomaly effectively, ensuring timely intervention and reducing the risk of severe complications. Regular follow-up is also important to monitor for recurrence or new symptoms, fostering improved quality of life for affected individuals. 3, 4
