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Cervical Hibernoma: a Very Rare Tumor of Childhood – Case Report with Review of Literature

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Cervical Hibernoma: a Very Rare Tumor of Childhood – Case Report with Review of Literature Published online: 2021-05-23 Case Reports and Review of Literature Cervical Hibernoma: A Very Rare Tumor of Childhood – Case Report with Review of Literature Abstract Advait Prakash1, Hibernomas are uncommon benign soft‑tissue tumors which are composed of brown fat. They are Sangram Singh1, usually seen in adults in the fourth and fifth decades and the common locations are thigh, shoulder, Kshiteej Dhull2, and back. Only few cases in the posterior neck region have been reported in literature. Hibernoma 2 is very uncommon in the pediatric age group and those in the neck region are extremely rare. Sankalp Sharma , 1 We present a hibernoma presenting as a swelling on the posterior aspect of base of the neck in a Tanmay Vyas , 5‑month‑old male infant. Considering the rare occurrence in the pediatric age group and difficulty Anvesh Jain1 in diagnosis, the clinical features, radiological features, pathological characteristics, differential 1Department of Pediatric diagnosis, and management of this rare tumor of childhood are discussed herewith. Surgery, Sri Aurobindo Institute of Medical Sciences Keywords: Brown fat tumor, cervical mass, hibernoma, pediatric neck mas and Post Graduate Institute, Indore, Madhya Pradesh, India, 2Department of General Introduction or bleeding. The swelling caused some Surgery, Sri Aurobindo Institute discomfort with movement, but there were of Medical Sciences and Post Hibernomas are uncommon benign Graduate Institute, Indore, no neurological deficits. The patient did not soft‑tissue tumors, developed from fetal Madhya Pradesh, India have difficulty in swallowing or breathing; brown fat tissue, vestigial remnants of the his medical history was unremarkable. On evolution of the species.[1] They are seen examination, it was a nontender, soft, freely in the rare areas where brown fat remnants mobile swelling measuring 5 cm × 4 cm may persist, such as interscapular area, with skin over the central part showing mediastinum, axilla, retroperitoneum, and mild excoriation. Cough impulse and neck.[2,3] Cervical hibernoma is very rare transillumination tests were negative. benign tumor that should be considered Various differential diagnoses that were a differential diagnosis for posterior neck considered were lipoma, lipoblastoma, masses. It occurs mainly in adults and very hemangioma, encephalocele, and few cases have been reported in children.[4,5] lymphangioma. The symptoms are compression of cervical structures, respiratory compromise, Laboratory evaluation showed hemoglobin Horner’s syndrome, and hemiparesis.[4] We of 11.2 g% with normal total and Submitted: 19‑Mar‑2018 differential leukocyte counts, erythrocyte Accepted in Revised Form: hereby present a case of neck hibernoma in 21‑Jun‑2018 a 5‑month‑old male infant along with the sedimentation rate, and peripheral smear. Published: 24‑Apr‑2020 review of clinical features and management Serum C‑reactive protein was negative. of this extremely rare tumor in the pediatric Ultrasound of the swelling revealed a Address for correspondence: age group. heterogeneous mass at the base of posterior Dr. Advait Prakash, aspect of neck in midline with increased Department of Pediatric Case Report Surgery, Sri Aurobindo Institute vascularity. On magnetic resonance imaging of Medical Sciences and Post A 5‑month‑old male child presented with (MRI), a subcutaneous lesion was found Graduate Institute, Indore, a history of a small swelling at the base superficial to the paravertebral muscles Madhya Pradesh, India. of the nape of neck of 3‑month duration. without any communication with the spinal E‑mail: drprakashadvait@ rediffmail.com It gradually increased in size in the last cord. T1‑ and T2‑weighted images showed 4 weeks before presentation. The infant increased peripheral vascularity. On T2 had an uneventful birth history with images, it was observed as a hypointense Access this article online normal developmental milestones. There lesion [Figure 1]. was no history of trauma, discharge, Website: www.ijmpo.org The mass was excised completely, DOI: 10.4103/ijmpo.ijmpo_63_18 and the defect was repaired in layers. Quick Response Code: This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution‑NonCommercial‑ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non‑commercially, How to cite this article: Prakash A, Singh S, as long as appropriate credit is given and the new creations are Dhull K, Sharma S, Vyas T, Jain A. Cervical licensed under the identical terms. hibernoma: A very rare tumor of childhood – Case report with review of literature. Indian J Med Paediatr Oncol 2020;41:72-4. For reprints contact: [email protected] 72 © 2020 Indian Journal of Medical and Paediatric Oncology | Published by Wolters Kluwer ‑ Medknow Prakash, et al.: Cervical hibernoma: A very rare tumor of childhood a b Figure 2: (a) Gross specimen after complete excision, (b) microscopic histopathological evaluation of the excised specimen Figure 1: Magnetic resonance imaging showing posterior cervical lesion Diagnostic imaging studies are invaluable in the preliminary Histopathological evaluation (HPE) confirmed the diagnosis diagnosis of fatty tumors.[2] Plain X‑rays and computed of benign hibernoma. HPE showed the tumor to have a tomography (CT) scan can suggest the fat density of the thick intact capsule consisting of collagenous connective tumor. Results of ultrasonography examinations can be tissue. On microscopy, abundant brown fat with a plenty confusing. The MRI is the most reliable method showing of fat lobules separated by connective tissue septa was location, size, extent, and mass characteristics of the present. This was depicted by cells with granular deeply tumor.[1] Hibernomas are usually depicted as heterogeneous eosinophilic cytoplasm along with clear multivacuolated masses with marked contrast enhancement. The CT and cells filled with lipid droplets [Figure 2a and b]. The nuclei MRI examinations show a well‑demarcated mass having were small, regular, and round with no atypia and rare signal intensity which is intermediate between subcutaneous mitoses. Postoperative recovery was uneventful, and the fat and muscle. The lesion enhances after contrast injection. patient was discharged on the 3rd postoperative day. There Despite presenting as brown fat, the T1‑ and T2‑weighted was no recurrence after 1 year of follow up. images demonstrate high‑signal intensity but slightly less than that of the subcutaneous fat. On fat‑suppression Discussion sequences, there may be incomplete fat suppression Hibernoma is a rare benign soft‑tissue tumor composed because of the nature and amount of lipids.[6,7,11] of multiloculated fat cells derived from brown fat. This The needle biopsy carries a risk of hemorrhage and often tumor was first described in 1906 by Merkl. The term leads to inconclusive results; hence, the definitive diagnosis hibernoma was coined in 1914 by Gery because of the is based on histopathological analysis after surgical excision morphologic similarity of the tumor to the brown fat biopsy.[6] The microscopic examination of the tumor is [6,7] cells of the hibernating mammals. Brown adipose characterized by cells of various degrees of differentiation. tissue exists in humans in intrauterine life and may be Usually, multivacuolar adipocytes and brown fat cells st recognized after the 21 week of gestation. Brown fat with granular eosinophilic cytoplasm are interspersed is found physiologically in newborns in the axilla and with univacuolar adipocytes.[1] The characteristic color the subpleural regions, but it usually disappears after of hibernoma is derived from the increased vascularity [7] 8 weeks of life. As maturation proceeds, it is slowly combined with abundant mitochondria.[1] Four histological replaced by white fat. It may persist in certain areas variants of hibernoma have been described by Furlong such as neck, axilla, periaortic area, mediastinum, and et al. [Table 1].[5] perirenal areas.[8] The function of this brown fat is to promote nonshivering thermogenesis.[1] On thorough Recent studies have suggested that formation of hibernoma literature search, we found that <20 cases of cervical may have an underlying genetic predisposition. It has hibernoma have been reported till date. Majority of been found that these tumors present with a balanced cases are among adult patients and cervical hibernoma translocation between 11q13 chromosome and other in children is extremely rare. chromosomes. These translocations have been associated with MEN1 and AIP genes.[12] Hibernomas are mostly seen in the fourth and fifth decades of life, and previously, a female predominance was reported The differential diagnosis of any lipomatous tumor should with a mean age of 38 years.[5‑7] Clinically, their consistency include lipoma, lipoblastoma, hibernoma, and liposarcoma, is multiple but usually harder than a regular lipoma. It is a all of which can be differentiated on detailed HPE.[13] The mobile, slow‑growing mass. It is usually asymptomatic but lipoma is the most common soft‑tissue tumor and may show rarely may present with pain weight loss.[9,10] Most common cytologic similarities to lipoblastoma. Both of them differ by anatomical locations were the thigh shoulder, back, neck, the fact that the extent of vacuolated adipocytes is greater in chest, arm, and abdominal cavity.[8] number and smaller in lipoblastoma in contrast to lipoma.[14] Indian Journal of Medical and Paediatric
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