A CASE OF SPLENIC HAMARTOMA ASSOCIATED WITH THROMBOCYTOPENIA

Ratbi El. Amine, Sabbar Wadie, Bakali Younes, Alaoui Mhamdi Mouna, Raiss Mohamed, Hrora Abdelmalek and Sabbah Farid Surgical Clinic C, University Hospital IbnSina, Rabat, Morocco. ...................................................................................................................... Manuscript Info Abstract ......................... ........................................................................ Manuscript History Received: 05 June 2020 Final Accepted: 10 July 2020 Published: August 2020

A pathological examination of the specimen revealed the 25x 14 cm spleen, weighing 900 g, the presence of well limited cystic cavity has coagulated hemorrhagic content without endocystic vegetation measuring 10x7x6 cm.
At microscopic examination of specimen of the spleen and immunohistochemistry was consistent with the diagnosis of splenic hamartoma.The patient was seen at the ambulatory clinic six months after her surgery with an auspicious evolution, presenting normal hemoglobin and platelet levels.

Discussion:-
The concept of hamartoma was formulated in 1994 by Albrecht (3)a non-neoplastic tumor-like congenital malformation consisting of a variable mixture of tissues normally present in the organ affected, sometimes with an overgrowth of one or more of them (4).
Hamartoma are rare benign tumors of the spleen, composed of an aberrant mixture of normal tissue components. Forming single or multiple masses within the splenic pulp (1). It can occur in any age group (5 months to 86 years) with a mean of age of 47 years (5) and with no sex predilection (6), woman tend to have larger lesions, probably due to hormonal factors (7) no statements can be proven.More than 80% of patients with splenic hamartomas are asymptomatic and the lesions are usually detected during imaging studies, surgery or post mortem (5,8), sometimes few cases have symptoms such as pain, palpable mass or splenomegaly may also cause anemia, thrombocytopenia or pancytopenia or malignant hematological conditions, has also been reported (9). Moreover, our patient presented with low thrombocytopenia level, due to the important of splenomegaly she presented, returning to her normal level after surgery was performed. other less common symptoms include fever, night sweats, malaise and spontaneous rupture (5). They may also be associated with multiple hamartomatosis, tuberous sclerosis and with Wiskott-Aldrich-like syndromes and Kasbach-Merrit syndrome (10,11).
Imaging of splenic hamartomas may be not specific. At abdominal ultrasound they usually appear as solid, homogeneous hypoechoic mass (10), with positive vascularization at color Doppler (12), sometimes with multiple anechoic cystic changes or with an inhomogeneous appearance (10). They can also appear as an isodense mass on CT. after contrast medium administration, these masses show early and sustained enhancement during the delayed phase of contrast-enhanced CT (8). Clinical or imaging diagnosis of this type of tumor is difficult to make before the operation, nevertheless, two documented cases succeed in having a preoperative diagnosis (13,14) us our patient. At MRI hamartomas are isointense on T1 weighted images and hyperintense on T2 weighted images, with heterogeneous enhancement after gadolinium administration (15,16).
The vascular tumors of the spleen represent the main differential diagnosis of this disease including hemangioma, littoral cell angioma, lymphangioma, hemangioendothelioma, sclerosingangiomatoid nodular transformation of the spleen, and angiosarcoma. Solid lesions of the spleen, such as inflammatory myofibroblastic tumor, lymphoma, 214 metastatic disease, disseminated fungal or mycobacterial infections, and sarcoidosis, are also included in the radiologic differential diagnosis (7). Diagnosis is confirmed by histopathological examination.
There are threehistologically types of splenic hamartoma: the pulposal type, resembling the splenic red pulp, the lymphoid type or follicular, resembling the splenic white pulp, and the fibrous type (17). Immunohistochemistry may reveal CD8 positive cells lining the vascular channels (18). These cells are also positive for CD31, factor VIIIrelated antigen, and vimentin. Immunostaining for CD34 has led to inconsistent results, and the endothelial cells are negative for CD21. CD68 is positive in scattered stromal macrophages but negative in the cells lining the vascular channels (7).
Currently, laparoscopy splenectomy is become a standard for treatment of most of splenicdisease (19). But open splenectomy is considered better indicated in case of splenomegaly, when the diameter of spleen exceeds 20 cm (20). In the present case, the length of spleen was 19 cm, therefore we have adopted an open approach due to the presence of thrombocytopenia, the risk of spontaneous splenic rupture and its vascularization.The prognosis of splenic hamartomas is good, as local and distal recurrence rate is very low and splenectomy can therefore be considered curative (21).
Splenic hamartomas associated with thrombocytopenia have rarely been reported. Komo et al (22) report that only 19 cases have been reported in literature and only 6 cases have been reported in the Japanese literature. Watanabe et al (23) reported that the mean diameter of splenic hamartomas was 5.3 cl, and the diameter of those associated with thrombocytopenia was 9.9 cm. Furthermore, they reported that thrombocytopenia was caused by hypersplenism due to splenomegaly. In the present case, thrombocytopenia caused by hypersplenism due to splenomegaly of splenic hamartoma, the histological examination not showed any sign of myelodysplastic syndrome.

Conclusion:-
this observation reports the case of splenic hamartoma associated with hematological disorders in particular thrombocytopenia, the preoperative diagnosis can be suspected by scanner. Surgery can confirm the final diagnosis and resolves the symptoms related to hypersplenism.