Multiple Haemangiomas of The Spleen

We report multiple cavernous haemangiomas of the spleen in a 42 year old female diagnosed on ultrasound and confirmed on contrast enhanced CT scan, which was part of her investigative work up for chronic persistent upper abdominal pain. She underwent an elective splenectomy and an unremarkable recovery. Histopathology confirmed the diagnosis.

Introduction

Splenic haemangiomas though rare, still remain the most common benign neoplasm of the spleen. Although majority of patients are asymptomatic and are diagnosed incidentally, a 25% incidence of spontaneous rupture has been reported, justifying operative intervention in the apparently asymptomatic patient.

Case Report

A 42 year old female presented with dragging pain in the left hypochondrium of 3 months duration. The pain was constant, non-radiating and had no precipitating or relieving factors. There were no other associated complaints. On physical examination, the spleen was just palpable with tenderness in the left hypochrondrium on deep palpation. There was however no evidence of ascites, hepatomegaly, haemorrhagic skin lesions or lymphadenopathy. A complete haemogram showed a normal haemoglobin (11.7 g%), total count (8500/cu mm; P73 E3 L24) and platelet count (2 Lac/ cu mm). The prothrombin time and index were normal and biochemical indicators of hepatic, renal and pancreatic function were also within the normal range. An abdominal B mode, real time gray scale ultrasound showed a mild splenomegaly (10 x 6 x 4 inches) with multiple hyperechoic subcapsular lesions, the largest of which measured 4.3 x 3.2 cm. suggestive of multiple haemangiomas. A colour doppler showed a normal configuration of splenic vessels. A spiral CT of the abdomen with 8 mm axial cuts using 2.5 mm collimation with plain and contrast enhanced scans showed multiple hypodense lesions in the spleen with no enhancement in the arterial and venous phase but showed centripetal filling in delayed venous phase, suggestive of multiple haemangiomas. There were a few areas of fine calcification but no evidence of any similar lesion in the liver or the kidneys.

We subjected the patient to an elective splenectomy. The patient withstood the procedure well and recovered uneventfully.


Fig. 1 : Contrast CT scan of the abdomen showing multiple hypodense lesions in the spleen

Fig. 2 : Spleen, paraffin section (Haematoxyline and Eosin, 10x) showing multiple cystic spaces lined by flattened endothelium with abundant erythrocytes in the lumen

Pathological findings

The spleen measured 250 mm x 150 mm x 100 mm and weighed 840 g. The surface showed multiple lesions ranging

from 5 mm to 50 mm which were soft and compressible. On the cut surface, multiple discreet angiomatous lesions, randomly distributed throughout the entire spleen. Each consisted of large cystic spaces, lined by flattened endothelium with large number of erythrocytes in the lumen. The red pulp in between the angiomatous lesions showed markedly stretched sinuses. No mitoses or dysplastic changes were noticed. The white pulp showed apparently normal follicular patterns. A diagnosis of multiple cavernous haemangiomas of the spleen was made based on the above findings.

Discussion

Virchow is credited with the first ever description of the disease, classifying it into : Haemangiomas Simplex, Cavernous Haemangiomas, Haemangioma telangiectoides and angioblastoma.¹ Hodge reported the first successful surgery for splenic haemangioma in 1895.¹ Female are more commonly affected with a ratio of 1.1-1.4:1 (female : males)^1,2 with an overall incidence of 0.03-14.5%, based on autopsy findings, most cases being discovered between 21 and 50 yr. of age.¹ Various theories as to the origin of this neoplasm have been postulated with a general consensus being that they are a congenital malformation that later differentiates into capillary, cavernous or other pathological types. The slow rate of growth of the tumour accounts for the late manifestation of the disease. Though splenic haemangiomas and hamartomas are difficult to differentiate on radiological or pathological examination, they can be differentiated on histochemistry. Current research reveals the presence of both CD8 and Factor VIII related antigens on the surface of the endothelial cells of a hamartoma vis-a-vis slpenic haemangiomas which are factor VIII positive but CD8 negative.³ Diffuse and localised haemangiomas of the spleen can be further differentiated by the fact that diffuse haemangiomas frequently express CD68.⁴ Ramani, Reinhold and Semelka have characterised the Magnetic resonance features of visceral haemangiomas, distinguishing them from hamartomas on features of magnetic resonance alone. Majority of splenic haemangiomas (86.36%) are hyperintense (relative to the spleen) on T2 weighted images, show centripetal filling on dynamic Godalinium-enhanced imaging and demonstrate uniform delayed enhancement, while majority of hamartomas (75%) are hyperintense but show heterogeneous enhancement on early images which become more uniformly enhanced on delayed images. The author concludes that it may be reasonable to consider, without histological verification that splenic lesions with these characteristics represent haemangiomas.5 Although a wide variety of pathological types of splenic haemangiomas have been described in standard textbooks of pathology, suffice it to say that cavernous haemangiomas are the most frequently reported variety in the spleen. Like any vascular tumour a splenic haemangioma can undergo infarction, thrombosis, haemorrhage and fibrosis, inducing an inflammatory reaction, leading to perisplenic adhesions that may be the basis behind the clinical symptomatology.

Clinical features

Majority of cases being asymptomatic (75-80%)² several patients may however present with a dragging pain in the left hypochondrium with or without gastro-intestinal disturbances. A mass, or a palpable spleen is present only in 12.5% of patients with a majority of cases being discovered on investigative procedures for other disorders.² Thrombocytopenia with manifestations of coagulopathy represents a rare presentation of the disease. Although initially described for a cavernous haemangioma on the extremity in an infant. Kassabach - Merritt syndrome can complicate a splenic haemangiomas.^6,7 The primary mechanism of thrombocytopenia is thought to be related to the trapping of platelets by abnormal endothelium of the tumour with selective intratumoural consumption. However, 25% of patients may present with spontaneous rupture, necessitating urgent surgical intervention.¹

Conventional diagnostic modalities of ultrasound and computerised tomography are effective in diagnosing cases with discreet visceral haemangiomas but fail to achieve appreciable sensitivity and specificity in obtaining a diagnosis when the angiomatous changes are diffuse. Velkova and Neveda report a combined accuracy of only 61.3% of both ultrasound and CT scan for suspected focal lesions of the liver and spleen8 leaving a substantial number of patients requiring further investigative procedures to establish a diagnosis. Digital subtraction angiography and fine needle biopsy under ultrasonographic guidance offer effective means to establish a diagnosis in these cases but these remain invasive procedures. Various studies by Wijaya, Kapoor and Roach;⁹ Phillpott, Ali and Briscoe;¹⁰ and Licht, Goffner and Yung11 confirm the usefulness of Tc-99m labeled erythrocyte scintigraphy / SPECT scan as a diagnostic modality for splenic haemangiomas. Cases reported by Hoeger and Helmke;¹² Schulz and Urban;¹³ and Platokouki and Aronis¹⁴ all describe patients with multiple visceral angiomatosis which improved after splenectomy inspite of the other haemangiomas remaining in situ. In such patients presenting with coagulopathy indium-111-labelled platelets can be utilised to delineate intratumoural platelet consumption.^15,16

The treatment most often consists of splenectomy, however Willcox and Speer in their evaluation of splenic haemangiomas over 8 year period at the Mayo Clinic propose that small splenic lesions that meet the radiologic criteria of haemangiomas can be safely observed.² Keeping in mind the fact that there exists a 25% incidence of spontaneous rupture and the poor follow up of patients in the Indian setup it may be justifiable to subject patients with a single large or multiple small haemangiomas (especially those in a subcapsular location) to elective splenectomy to avoid such disastrous consequences occurring in places where qualified and timely medical aid may be inaccessible.

CONCLUSION

Splenic haemangiomas are the commonest benign tumours of the spleen, the majority of which are asymptomatic, being diagnosed as an incidental finding on investigative procedures for other disorders. They however have to be ruled out as a cause of consumptive coagulopathy in patients presenting with Kassabach-Merritt syndrome. Though a majority of haemangiomas can be effectively demonstrated with conventional radiological imaging techniques of ultrasonography and CT, other diagnostic modalities can be relied on to localise missed lesions. Treatment usually consists of splenectomy. Though it has been suggested that small haemangiomas can be safely followed up, in view of a 25% incidence of spontaneous rupture and the poor follow up in the Indian setting, elective splenectomy may be justified.

References

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3. Zukergerg LR, Kaynor BL, Silverman ML, et al. Splenic hamartoma and capillary haemangioma are distinct entities : immunohistochemical analysis of CD8 expression by endothelial cells. Hum Pathol 1991; 22 : 1258-61.

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10. Phillpott J, Ali SA, Briscoe EG, Cesani F. Three Phase Tc99m Labeled RBC Scintigraphy of a Splenic Haemangioma. Cli Nucl Med 1997; 22 (3) : 158-60.

11. Light M, Goffner L, Yung E. Giant Splenic Hemangioma : Confirmation of Diagnosis with Labeled Erythrocyte Scintigraphy. Cli Nucl Med 1999; 24 (10) : 781-3.

12. Hoeger PH, Helmke K, Winkler K. Chronic Consumptive Coagulopathy due to an Occult Splenic Hemangioma : Kasabach Merritt Syndrome. Eur J Pediatr 1995; 154 (5) : 365-8.

13. Schulz AS, Urban J, Gresler P. Anemia, Thrombocytopenia and Coagulopathy due to Occult Diffuse Infantile Hemangiomatosis of the Spleen and Pancreas. Eur J Pediatr 1999; 158 (5) : 379-83.

14. Platokoui H, Aronis S, Mitsika. A Diffuse Splenic and Visceral Hemangiomas Complicated by Consumptive Coagulopathy. Acta Paediatr Jpn 1998; 40 (14) : 381-4.

15. Pampin C, Devillers A, Treguiner C. Intratumoral Consumption of Indium 111-Labeled Platelets in a Child with Splenic Hemangioma and Thrombocytopenia. J Pediatr Hematol Oncol 2000; 22 (3) : 256-8.

16. Wareell RP Jr, Kempin SJ, Benuu RS. Intratumoral Consumption of Indium Labeled Platelets in a Patient with Hemangiomatosis and Intravascular Coagulation Kasabach-Merritt Syndrome. Cancer 1983; 52 (12) : 2256-60.

Letter to the Editor

Sir,

The Dangerous side-effects of the drug LERKA (Lercanidipine)

Before taking this drug in April 2003, my husband was walking about without stick or support with exceptionally well developed muscles of legs and arms. He had sat down for 2 hours at a stretch to correct 38 page draft of a senior lawyer.

After taking 2¾ tablet of this drug, he continued to look dull like a child and not able to do any mental or physical activity.

But after 13 exposures of hyperbaric oxygenation, he was mentally alert to correctly recognize a person after 20 years and narrate incidences of nineteen fifties of hospital, like naming the hospital and observe minor defect in the chamber which the senior attendant was surprised about but admitted to correct it by next day which never came, because with poor inhalation capacity of paralyzed chest muscles, he developed left basal pneumonia and succumbed to the treatment for this in ICU of Baccha Nursing Home. I wish to pass on this information to my medical fraternity to save as many patients as possible. I can give detailed day-to-day hour-to-hour report with dates and timings if required.

Dr. (Mrs) Usha Omprakash
Consultant Obstetrician and Gynaecologist