Hamartomas of Body: A Revisited Entity - An Experience of a Tertiary Care Hospital

Abstract

Introduction/Background: Hamartoma is a mass of disorganized tissue indigenous to the particular site. Although traditionally considered as developmental malformations, many hamartomas have clonal chromosomal aberration that is acquired through somatic mutation, thus now considered to be neoplastic. They arise virtually in all organs, either sporadically or in association with autosomal dominant syndrome. Materials and Methods: A retrospective study was carried out in a tertiary care health institute for 3-year period (January 2013 to December 2016), which included all visceral hamartomas from head to toe. Detailed clinicoradiological and pathological examination was done along with ancillary tests for definite diagnosis. Observation: The study included nine cases, categorized site wise as head and neck (one case of cystic lymphangioma), cardiothoracic (one case of chondroid hamartoma), gastrointestinal tract (GIT) (one case of Brunner's gland hamartoma), hepatic (one case of cystic lymphangioma), and genitourinary tract (four cases of angiomyolipoma [AML] and one case of myelolipoma). The age ranged from 1 year to 70 years showing a male predilection. The tumors were variable sized ranging from 0.5 to 20 cm in dimension either solitary or multiple. Some large tumors clinically masqueraded as malignant tumors and presented with complications. The postsurgical course and prognosis of all patients were uneventful. Conclusion: Hamartomas are usually not associated with significant morbidity but for the size and location of the tumor. Despite slow-growing and self-limiting behavior, some predispose to malignancy especially in the epithelioid AML and familial GIT hamartoma or may be seen associated with malignancy in syndromic forms.