Treatment of metastatic sialoblastoma with chemotherapy and surgery

Abstract

Tumors of the salivary gland are very uncommon in children. Sialoblastoma is a rare, aggressive, blastomatous, and potentially malignant congenital tumor. Distant metastases are rare. We present a case of sialoblastoma with lung metastases that developed in a 4‐year‐old girl adjacent to a congenital nevus in the left cheek. The tumor was inoperable at diagnosis but the largest of the pulmonary metastases was removed surgically. The patient responded well to chemotherapy and underwent surgical excision of the primary tumor, followed by three more courses of chemotherapy. Pediatr Blood Cancer 2008;50:134–137. © 2006 Wiley‐Liss, Inc.

Sialoblastoma of salivary glands in children: Chemotherapy should be discussed as an alternative to mutilating surgery

“…Sialoblastoma is a very rare congenital salivary gland tumor, only 26 cases are reported [1][2][3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19]. This tumor may exist before birth [7,11,13,18], or may be discovered after delivery [6,8,19].…”

“…The treatment of metastatic disease is also controversial. Some cases of successful chemotherapy have been reported [3,6,9,13,15,21]. Due to the young age of these patients, external beam radiotherapy does not appear to be an appropriate adjuvant treatment option [19].…”

“…This tumor may exist before birth [7,11,13,18], or may be discovered after delivery [6,8,19]. It mainly arises in the parotid gland (16 of the 21 published cases) [3,6,[8][9][10][11][14][15][16][18][19][20], but may also involve the submandibular gland (6 cases) [6,11,12] or even ectopic salivary gland tissue [2]. Due to the rarity of this tumor, no consensus has been reached concerning the treatment of sialoblastoma.…”

What’s New in the AFIP Fascicle on Salivary Gland Tumors: A Few Highlights from the 4th Series Atlas

“…Among the patients with metastases, three had pulmonary metastases [4,8,9] and one had lymph node metastases [10]. The patient that died experienced multiple recurrences and died of respiratory insufficiency and deterioration of her general health [11].…”

“…Chemotherapy has had a role in some cases, but limited number of cases makes it difficult to meaningfully assess the efficacy of both chemotherapy and radiotherapy [4,[8][9][10].…”