A 32-year-old male patient presented with enlargement of his upper lip since childhood, which was compromising his facial aesthetics, as well as causing inability to close the mouth completely. History revealed the presence of a red spot on his lower lip when he was 2 months old, and it had increased considerably ever since. The patient was systemically healthy, with no palpable lymph nodes. The swelling extended over the right half of the upper lip, crossing the midline. Clinically, the swelling was reddish pink in color, soft, fluctuant, lobulated, and painless on palpation, with well-defined margins (Figure, black arrow). It did not show either thrill or bruit on auscultation. On diascopy test, the swelling changed its color, which indicated its vascular nature. Panoramic radiograph showed no loss of alveolar bone. Magnetic resonance imaging discovered several high-intensity lobules. The differential diagnosis arrived at was infantile hemangioma and arteriovenous malformation. As a rule, the arteriovenous malformation is present at birth and it reveals thrill or bruit on auscultation. In this case, swelling was not present at birth, nor was there thrill or bruit on auscultation; the diagnosis arrived at was infantile hemangioma. Surgical treatment of infantile hemangioma was undertaken and the excised tissues were sent for further analysis. Histopathological examination revealed large dilated blood sinuses filled with red blood cells and thin walls lined by endothelium; immunostaining was positive for glucose transporter-1 protein, which confirmed our diagnosis of infantile hemangioma. One-year follow-up revealed a completely healed upper lip with no recurrence.
Infantile hemangioma or “strawberry marks” are benign tumors that arise from the proliferation of endothelial cells, which exist around blood-filled cavities, with an incidence rate of 4.5%.1 Infantile hemangioma progresses through 3stages: proliferation phase (first year of life), involuting phase (1 to 7 years) and involuted phase (regresses entirely by 8 to 12 years). The pathogenesis of infantile hemangioma is not very clear, but one mechanism that offers promise is hypoxia theory. The facilitative glucose transporter, glucose transporter-1 protein, is a key sensor of hypoxia that is used as a marker in infantile hemagioma.2 Its levels are increased in human mesenchymal stem cells of the umbilical cord and in mesenchymal tumors in hypoxic situations.3, 4 The endothelial cells also produce factors when induced by hypoxia; they seem to play a dominant role in transferring progenitor cells toward ischemic tissue. These factors, like Vascular endothelial growth factor-A (VEGF-A), Matrix metallopeptidase-9 (MMP-9), hypoxia-inducible factor 1α, and stromal cell-derived factor 1α have been shown to be upregulated in individuals with infantile hemagioma.5 Diagnosis of infantile hemangiomas is generally based on clinical history, examination, magnetic resonance imaging scans, histopathology, and immunostaining. Infantile hemangiomas regress spontaneously, barring a few that may require surgical intervention, cryotherapy, radioisotope therapy, systemic corticosteroids, and propranolol.
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-Sowmya Sham Kanneppady, MDa, Sham Kishor Kanneppady, MDS, PhDb,, Tanay Chaubal, MDSc, Ranjeet Bapat, MDSc, Rohit Pandurangappa, MDSc, Aung Myo Oo, MMedScid, Ohn Mar Lwin, MBBSe
This article originally appeared in the April 2019 issue of The American Journal of Medicine.
