Primary Angiosarcoma of Parotid Gland in a Child: A Rare Case with Diagnostic Challenge
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CASE REPORTS
Primary Angiosarcoma of Parotid Gland in a Child: A Rare Case with Diagnostic Challenge Sunil Pasricha1 · Ankush Jajodia2 · Meenakshi Kamboj1 · Gurudutt Gupta1 · Anila Sharma1 · Garima Durga1 · Divya Bansal1 · Anurag Mehta1 Received: 29 July 2019 / Accepted: 29 October 2019 © Springer Science+Business Media, LLC, part of Springer Nature 2019
Abstract Primary angiosarcoma of salivary glands is a rare entity that most commonly arises in the parotid gland and affects adults and the elderly. Angiosarcoma of the parotid gland in a child is exceedingly rare and thus can be a diagnostic challenge. We present a case of angiosarcoma of the parotid gland in a 6-year-old female child with bilateral lung metastases. The patient underwent chemotherapy and showed a partial response. Awareness of this entity in salivary glands is important for accurate and early diagnosis due to its distinct therapeutic and prognostic implications. Keywords Angiosarcoma · Lung · Parotid gland
Introduction
Case Report
Malignant mesenchymal tumors of the major salivary glands are rare, and the majority originate in parotid glands. Primary angiosarcoma involving the parotid gland is uncommon and predominantly affects the adults and elderly [1–4]. Angiosarcoma of the parotid gland in a child is exceedingly rare and a diagnostic challenge in a small biopsy. Awareness of this presentation is important for timely diagnosis due to its distinct management. The prognostic significance of angiosarcoma in a major salivary gland is contentious due to the paucity of reported cases. We describe a case of an angiosarcoma of the parotid gland in a 6-year-old female child.
A 6-year-old female was initially seen at an outside center for a large upper neck mass extending to the cheek. It had been present for a duration of 22 months. An ultrasonograph of the neck revealed a lobulated, well-defined, and hypoechoic mass lesion in the superficial parotid gland region. The mass measured 3.9 × 3.7 × 3.2 cm and showed multiple tiny cystic spaces. Color doppler ultrasound showed prominent internal vascularisation within the mass and low resistance in both arterial and venous blood flow. The biopsy report rendered a diagnosis of haemangioma. The patient subsequently received propranolol (50 mg for 18 months) which initially reduced the tumor size. After 2 months, however, the swelling increased considerably along with symptoms of facial nerve paresis. She was subsequently referred to our institution. On local examination, a large mass in right parotid region was identified. It measured 10 × 6 cm and was tender and firm. The overlying skin was tense, shiny, and red without ulceration. Magnetic resonance imaging (MRI) with contrast revealed a large soft tissue mass measuring 8.4 × 7.2 × 8.6 cm. Multiple vascular flow voids involved the entire parotid gland (Fig. 1a). The mass was compressing the right masseter and pterygoid muscles and displaced the right submandibular gland. Medially and posteriorly the mass indented the parapharyngeal fat and right para
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