Early Surgical Intervention in an Infant with Angiomyxoma: Ensuring Complete Excision and Prevention of Recurrence

Abstract

Angiomyxoma is a rare, benign mesenchymal tumor rarely encountered in the infantile hard palate. This case report describes the presentation, diagnostic workup, and successful management of an angiomyxoma in an 11-month-old male infant. An 11-month-old male was presented to the ENT department with a slowly enlarging, asymptomatic palatal swelling first noticed at 9 months of age. Intraoral examination revealed a 2 cm × 2 cm, soft, nontender mass on the anterior hard palate. A contrast-enhanced computed tomography scan identified a well-defined, oval, heterogeneously enhancing cystic lesion (8 mm × 15 mm × 18 mm) with no bony erosion. The patient underwent complete surgical excision under general anesthesia. The lesion was excised in toto via an intraoral approach, revealing a well-encapsulated, gelatinous mass. Histopathological examination confirmed the diagnosis of angiomyxoma, showing spindle cells in a myxoid stroma with numerous thin-walled vessels. The postoperative course was uneventful, and the child resumed feeding normally. At the 6-month follow-up, there was no evidence of recurrence, and both functional and cosmetic outcomes were excellent. This case underscores that angiomyxoma, though rare, should be considered in the differential diagnosis of pediatric palatal masses. A combination of cross-sectional imaging and complete surgical excision is the cornerstone of management, leading to a favorable prognosis with low recurrence risk.

INTRODUCTION

Superficial angiomyxoma (SAM), also referred to as cutaneous myxoma, represents a rare benign mesenchymal neoplasm that was first comprehensively characterized by Allen et al. in 1988.[1] It may arise sporadically or in association with Carney complex.[2,3] The lesion most commonly manifests between the fourth and fifth decades of life, with several studies suggesting a mild male predominance.[1,3,4] Clinically, SAM typically appears as a slowly enlarging, painless, polypoid, or papulonodular cutaneous growth that may have been present for months to years. In patients with Carney complex, multiple lesions are frequently observed. Although SAM can occur at virtually any cutaneous site, the trunk, lower extremities, and head-and-neck region are the most frequently affected areas.[3,4] Definitive diagnosis requires complete excision and histopathologic examination. Microscopically, SAM is characterized by a paucicellular proliferation of bland spindle- to stellate-shaped cells embedded in an abundant myxoid matrix, accompanied by a delicate arborizing capillary network. Significant nuclear atypia and mitotic figures are typically absent.[4] Immunohistochemically, the neoplastic cells consistently express vimentin and CD34.[4] In the present report, we describe in detail an unusual case of palatal angiomyxoma occurring in an infant.

CASE REPORT

The clinical history for this 11-month-old, otherwise healthy male infant began at 9 months of age when his mother first noted a small, pea-sized swelling on his hard palate. Over the ensuing 2 months, the parents observed a gradual but persistent increase in the size of this nonpainful mass, which prompted their visit to the ENT outpatient department.

The patient’s prenatal, natal, and postnatal history was unremarkable. He was meeting all developmental milestones. There was no history of trauma, recurrent infections, or similar swellings. Family history was negative for genetic disorders, neurofibromatosis, or any familial tumor syndromes. The parents were appropriately concerned but compliant with medical advice.

On intraoral examination, a solitary, well-defined, 2 cm × 2 cm soft, mobile, and nontender mass was observed in the anterior hard palate, slightly to the left of the midline. The overlying mucosa was smooth, intact, and of normal color [Figure 1]. The mass was nonfluctuant and nonpulsatile with no palpable thrill. No facial asymmetry was present. Examination of the nose, oropharynx, and neck revealed no cervical lymphadenopathy or other abnormalities.

Figure 1.

Clinical presentation of palatal angiomyxoma in an 11-month-old child showing (a) preoperative appearance of the palatal swelling, (b) postoperative appearance after complete surgical excision, and (c) the excised angiomyxoma specimen

The computed tomography (CT) scan revealed a well-defined, oval, heterogeneously enhancing cystic lesion measuring 8 mm × 15 mm × 18 mm in the midline of the anterior hard palate, with its epicenter overlying the mucosa. Crucially, there was no evidence of bony erosion or deep tissue invasion into the palatal bones. The primary challenges included the patient’s young age, which necessitated general anesthesia for a compliant CT scan, and the broad differential diagnosis for a pediatric palatal mass. The differential diagnosis included congenital lesions such as dermoid cyst, benign mesenchymal tumors (e.g., lipoma and neurofibroma), and a minor salivary gland tumor. The primary diagnostic challenge was the patient’s young age and the need for a general anesthetic for both definitive imaging and biopsy.

The therapeutic plan was complete surgical excision. Under general anesthesia with oral endotracheal intubation, a vertical elliptical incision was made over the mass. The lesion was meticulously dissected and found to be a soft, gelatinous, well-encapsulated mass that separated easily from the underlying palatal bone and surrounding mucosa [Figure 1]. The lesion was excised in toto with a clear margin of healthy tissue. The surgical defect was closed primarily with absorbable sutures. Blood loss was minimal.

The postoperative course was uneventful. The child resumed feeding within a few hours and was discharged the following day. Histopathological examination (HPE) of the specimen confirmed the diagnosis. It showed spindle and stellate cells within an abundant, loose myxoid stroma, interspersed with numerous thin-walled, dilated blood vessels, consistent with angiomyxoma [Figure 2]. Surgical margins were free of tumor. The intervention was well-tolerated, and adherence to postoperative care was excellent. At the 6-month follow-up, the palatal wound had healed completely with no functional deficits in feeding or phonation and no clinical signs of recurrence. The parents reported high satisfaction with the outcome.

Figure 2.

100× showing spindle-shaped cells (highlighted within the circled area) in the histopathology of the excised palatal angiomyxoma

DISCUSSION

The extreme rarity of intraoral (SA) Solitary Angioma is highlighted by the fact that, to the authors’ knowledge, only a few cases, including the present one, have been documented in the literature.[5,6,7,8] Our review of these cases reveals a slight male predominance, with patients ranging in age from 0 to 70 years (mean: 39.3 years), aligning with the demographic profile of extraoral SA which typically affects middle-aged adults.[3,5,6,7,8] The subsites involved are varied, including the buccal mucosa, floor of the mouth, hard palate, gingiva, tongue, lip, and, as in our case, the soft palate. This appears to be the first reported instance of SA arising specifically in the soft palate without an association with Carney complex, expanding the known anatomical spectrum of this tumor.

Clinically, intraoral SAs typically present as slow-growing, painless, soft, and mobile masses.[5,7] However, the case reported by Morimoto et al.[7] and the present infant case demonstrate that a rapid growth pattern can also occur, potentially due to inflammatory or edematous changes secondary to local trauma or habit. This variability can lead to misdiagnosis, as the clinical appearance is often nonspecific. Common preoperative diagnoses include common benign entities such as mucocele, fibroepithelial polyp, lipoma, salivary gland tumor, or cystic lesions.[5,7] This underscores the necessity for an HPE to achieve a definitive diagnosis.

The histopathological hallmark of SA is a poorly circumscribed, multilobulated tumor composed of bland spindle-shaped to stellate cells dispersed in an abundant myxoid stroma, rich in hyaluronic acid.[1,5] A key diagnostic feature is the presence of numerous small, thin-walled blood vessels and a scattered inflammatory infiltrate, often including neutrophils, which are rarely seen in other myxoid lesions such as oral focal mucinosis or odontogenic myxoma.[3,5] Immunohistochemically, tumor cells are typically diffusely positive for vimentin and CD34 and negative for desmin, S-100, and smooth muscle actin, which helps to rule out other differentials such as myxoid neurofibroma or aggressive angiomyxoma.[5,7,9] A significant recent advancement is the discovery that a substantial subset (approximately 55%) of SAs demonstrate a loss of protein kinase A regulatory subunit 1 alpha (PRKAR1A) expression, a finding that can be a useful diagnostic adjunct, particularly in distinguishing it from histologic mimics such as superficial acral fibromyxoma and myxofibrosarcoma, which typically retain PRKAR1A expression.[4]

The standard and definitive treatment for SA is complete surgical excision. Although early studies of cutaneous SA reported high local recurrence rates of up to 30%–40%, likely due to incomplete excision of these poorly circumscribed lesions, the data for intraoral cases appear more favorable.[3,5] In the reviewed cohort of 12 intraoral cases, only one exhibited recurrence, which occurred twice in a gingival lesion that was initially excised with positive margins.[7] This suggests that intraoral SA may have a lower recurrence potential than its cutaneous counterpart. As advocated by Morimoto et al.[7] and demonstrated in the robotic-assisted excision of a large alveolar lesion by Nair et al.,[8] resection with a clear safety margin is crucial to prevent recurrence. In the present infant case, meticulous excision was performed with preservation of vital structures, resulting in a favorable outcome with no recurrence.

CONCLUSION

This case highlights a rare presentation of a benign mesenchymal tumor in an unusual location. A high index of suspicion, appropriate imaging, and complete surgical excision are key to the successful management of pediatric palatal masses, ensuring excellent outcomes and low recurrence rates.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient’s mother has given her consent for images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.