Superficial Angiomyxoma of the Umbilicus in an Infant

A 6-month-old female infant presented with a history of a progressively enlarging umbilical swelling which was noticed by parents soon after birth. Baby was born in a hospital by vaginal delivery, at term, out of a nonconsanguineous marriage, cried immediately after birth. The umbilical cord had been clamped at birth, and the stump shed by day 7 of life. Soon after the shedding of the stump, parents noticed a swelling in the umbilical region. It was nonreducible, persisting as such, without any change in size. A local physician had diagnosed it as omental hernia through umbilicus. An ultrasound was done, which showed heterogeneous, hypoechoic subcutaneous mass, with no definite communication from underlying organs. There was no history of trauma, discharge, bleeding, or tenderness in the lesion. Furthermore, the infant had no associated fever, poor feeding, vomiting, lethargy or constipation. The child had been feeding and developing appropriately till 6 months of age. Prenatal period was uneventful, with no abnormality detected in antenatal scans. On examination, the infant was active and playful with stable vitals and no pallor, icterus, or cyanosis. There was a solitary soft, umbilical pedunculated mass of size about 9.5 cm ×5.5 cm, with intact overlying skin, without any tenderness, or signs of inflammation or discharge [Figure 1]. It showed partial trans-illumination, was freely mobile with a small underlying defect palpable at umbilicus, but without any gurgling sound on attempted taxis. Auscultation revealed no bowel sound or bruit. A provisional diagnosis of umbilical hernia with cicatrization, possibly an omentocele, was kept. Rest of the abdomen, cardiorespiratory, and central nervous system examinations was normal. The patient was planned for elective surgery on cosmetic grounds.Figure 1: Clinical picture of the umbilical mass, mobile, pedunculated with intact overlying skin without any signs of inflammationRoutine investigations revealed hemoglobin of 13.5 g/dL, total leukocyte count 6500/µL, C-and reactive protein 2 mg/dL, with normal biochemistry parameters. The mass was completely excised under general anesthesia. Intraoperatively, the mass was loosely adherent to surrounding tissue but easily resected. The three umbilical ligaments were ligated, hernia repaired, and umbilicus was reconstructed [Figure 2]. The umbilical mass was sent for histopathological examination (HPE), which revealed gelatinous, lobulated mass with a glistening cut surface on gross examination [Figure 3a]. Under the oil immersion field on H and E staining [Figure 3b], hypocellular myxoid stroma with scattered spindle cells and thin-walled blood vessels were seen. No cytologic atypia or mitotic activity was noted. The HPE was thus suggestive of superficial angiomyxoma of the umbilicus [Figure 3]. The postoperative period was uneventful. The infant was discharged and kept under regular follow-up. The operated site healed well by 10 days, and there was no re-appearance of swelling at last follow-up 26 months postexcision, with a healthy incision scar and no discharge.Figure 2: Postoperative view of reconstructed umbilicusFigure 3: (a) Gross specimen of partly skin covered soft-tissue masses covered by the skin and cut surface show gray-white mucoid areas. (b) (H and E, ×10) Epidermis lined myxoid tumor showing bland spindle and stellate-shaped tumor cells with interspersed thin walled dilated blood vessels. Hypocellular myxoid stroma with scattered spindle cells and thin-walled blood vessels were seen. No cytologic atypia or mitotic activity was notedSuperficial angiomyxoma is a rare entity in infants, with most cases reported in adults.[1] It usually presents as an asymptomatic, slow-growing mass.[1,2] Differential diagnoses for umbilical swelling in infancy include umbilical hernia, granuloma, lymphangioma, or sarcoma. Radiologic imaging aids in delineating lesion extent, but histopathology remains diagnostic.[3] The primary management is tumor excision. Incomplete excision is common because of the infiltrating nature of the neoplasm and absence of a definite capsule. Considering the low mitotic activity of angiomyxoma, adjuvant radiotherapy and chemotherapy are not preferred. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.