Splenic Haemangioendothelioma in an Infant

Primary Haemangioendothelioma (HE) of spleen is a rare vascular tumour. These tumours have intermediate biologic behaviour between haemangioma and angiosarcoma. So far, few cases have been reported in literature and most of them are in adults or adolescents. Here the present authors report a 6-month-old infant who presented with mass per abdomen. Contrast Enhanced Computerised Tomography (CECT) abdomen revealed a splenic mass which was treated by partial splenectomy. Histopathology confirmed it to be haemangioendothelioma of spleen.

A 6-month-old male infant was presented to the outpatient department of paediatric surgery with complaint of progressively increasing abdominal swelling since one month. The child was apparently normal a month back when his mother noticed an abdominal lump while bathing him. There was no history of pain, fever, any respiratory distress, jaundice or bleeding tendency. His perinatal history was uneventful.

Baby was alert and playful with stable vitals. His weight was 6 kg. On inspection, abdomen was flat, umbilicus was central and all quadrants were moving equally with respiration. On palpation, there was no local rise of temperature and there was no tenderness. There was a solitary lump occupying the left hypochondrium, epigastrium, left lumbar and umbilical regions. The lump was firm in consistency and the margins were smooth. All the borders were well defined except the upper border which merged with the left costal margin. Swelling moved with respiration as well as side to side. Renal angle was free and bilateral hernial orifices were intact. On percussion, liver dullness was felt in right 5^th intercostal space. Dull note was heard over the lump and rest of the abdomen is tympanitic. On auscultation, venous hum was heard over the lump.

Patient was admitted and investigated further. On routine blood investigation, Hb was 10.0 gm/dL, total leucocytes count was 14200/thousand/μL, platelet count was 75000 thousand/microL, total serum bilirubin was 0.73 (0.2-1.2 mg/dL), direct serum bilirubin was 0.32 (0.0-0.3 mg/dL). SGOT was 88.40 (05-40 IU/L) and SGPT was 65.42 (0-45 IU/L). Viral markers were negative. Coagulation profile was within normal limit.

Ultrasonography revealed an intraperitoneal hetroechoeic mass lesion measuring 5 cm × 4 cm originating from the liver. Lesion was well marginated with areas of necrosis and calcification. A Contrast Enhanced Computerised Tomography (CECT) was advised for further delineation. CECT abdomen suggested a large heterogeneous soft tissue mass measuring 13 cm × 13 cm × 9 cm in left hypochondriac, left lumbar and epigastric region. Heterogeneous enhancement was seen in post contrast study with few areas of non enhancement likely necrosis. Focus of calcification was also noted within. Mass was seen to be scalloping the upper pole of the spleen and it was displacing and abutting the adjacent bowel loops with maintained fat planes [Table/Fig-1]. The differential diagnoses were Small round cell tumour and Mesenchymal tumour.

[Table/Fig-1]:

CECT showing splenic mass (arrow).

A trucut biopsy was planned for histopathological correlation. Histopathological examination of the specimen showed irregular congested vascular channels by plump round to spindled indented nuclei and moderate amount of cytoplasm some of the cells had vacuolated cytoplasm with presence of intracytoplasmic erythrocytes in few. Areas of haemorrhage were also present. No mitotic activity and necrosis were found. Features were suggestive of vascular tumour and Immunohistochemistry (IHC) was advised to rule out Epithelioid haemangioma and Haemangioendothelioma. On IHC CD31 and CD34 showed strong immunoreactivity in all the vascular channels and some of the endothelial cells. Impression was high grade vascular neoplasm (possibility of haemangioendothelioma and angiosarcoma).

Consultation was done with radiation oncologist but chemotherapy was not possible due to vascular nature of the mass. So the patient was worked up and posted for excision of the tumour and biopsy. On exploration with left sub-costal transverse incision, there was a 6 cm × 6 cm × 5 cm globular mass arising from the lower pole of the spleen [Table/Fig-2,3]. The mass was fleshy and vascular abutting the tail of pancreas. Feeding vessels were ligated and partial splenectomy was done. Specimen was sent for histopathological examination. Histopathology confirmed it to be haemangioendothelioma. Post-operative course was uneventful and patient was discharged on post-operative day seven.

[Table/Fig-2]:

Intraoperative picture of splenic mass.

[Table/Fig-3]:

Excised specimen of splenic mass.

The term ‘Haemangioendothelioma’ includes a heterogeneous group of vascular neoplasms, which are intermediate between benign and malignant tumours [1]. Although haemangioendothelioma is a vascular tumour, it exhibits mild cellular atypia and low mitotic rate; an intermediate appearance between haemangioma and conventional angiosarcoma histopathologically [2]. To the best of knowledge and extensive literature search the present authors could not find splenic haemangioendothelioma in children in two reports only, one pediatric patient and another 9-year-old child with splenic haemangioendothelioma that was also treated with partial splenectomy [3,4].

Mallory (1908) defined haemangioendothelioma as all proliferations that originated from endothelial cells of blood vessels [5]. Enzinger and Weiss later categorised haemangioendothelioma into composite epithelioid, hobnail, Kaposiform and epithelioid sarcoma-like haemangioendothelioma [6].

The treatment options for splenic haemangioendothelioma is limited due to very less number of cases reported. In paediatric age group partial splenectomy is preferred in view of their partially developed immunity. In adults, the most successful therapy is considered to be complete splenectomy [7]. Chen CW et al., reported and elderly female of epithelioid haemangioendothelioma of spleen with metastasis. She was treated with oral thalidomide which showed decrease in disease progression [8].

As no specific clinical manifestations are evident in the early stages of development so the diagnosis of splenic HE remains challenging [9]. The common causes of mortality in these cases are infection, consumptive coagulopathy and metastasis. The index case presented with lump abdomen which was diagnosed with computerised tomography and trucut biopsy. Partial splenectomy was done in view of immature immune system of the infant. No infant has been reported in literature so far with this diagnosis. The infant is in close follow-up for last six months and so far doing well.

Pediatric haemangioendothelioma is a rarity and diagnosis is a challenge. Due to concerns of potential rupture, thrombocytopenia, anaemia, coagulopathy and intermediate malignant potential, these cases should be managed with splenectomy.

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