Pediatric sialoblastoma: Evaluation and management.

OBJECTIVES: Sialoblastoma is a rare congenital salivary gland tumor of epithelial origin. The objectives of this study are to review the literature regarding clinical presentation of sialoblastoma, evaluate the effectiveness of various treatment methods, and present guidelines for evaluation and management in the pediatric population. DATA SOURCES: Case presentation and literature review. REVIEW METHODS: A comprehensive search was conducted to identify cases of pediatric sialoblastoma in the English-language literature. The presentation, evaluation, and management of reported cases were analyzed. We also report an invasive and recurrent case in a pediatric patient to highlight the aggressive nature of these lesions. RESULTS: Sixty-two cases of pediatric sialoblastoma were reviewed. The age at initial presentation ranged from before birth to 15 years. The parotid gland was the most common location (n = 47). Surgical excision was the primary treatment in all patients. Nine patients developed metastatic disease of the lung, lymph nodes, or bone. Almost a third of patients had recurrence and over two thirds of patients were tumor-free for at least 1 year following their last treatment intervention. CONCLUSION: Prompt and complete surgical excision should be recommended to prevent local and systemic recurrence of pediatric sialoblastoma. Chemotherapy has also shown promise in several cases, and clinical genomics may shed light on more therapy options. Patients should be closely followed for at least 12 months following diagnosis, or longer depending on the histopathological staging of the tumor.

Congenital Sialolipoma in an Infant.

Sialolipoma is a newly recognized tumor of the major and minor salivary glands and represents only 0.3% of all salivary gland tumors. Only 3 cases of congenital sialolipoma are available in the literature. In the current case, we performed a total parotidectomy with facial nerve preservation on a 12-week-old infant exhibiting huge mass in the parotid region. Histopathology results showed sialolipoma. There was no recurrence at the 18-month follow-up. Although it is a very rare disease in infants, congenital sialolipoma should be kept in mind in patients with parotid mass. The primary treatment is parotidectomy with facial nerve preservation.

Congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region.

We report a rare case of congenital tri-cavernous hemangiomas of the right buccal region, right accessory parotid gland, and masseter muscle region in an adult. The patient, a 25-year-old woman, complained of 3 masses in her right midcheek. Ultrasonographic and computed tomographic findings showed an irregular-shaped mass (multiple calcifications) with a well-defined margin in the masseter muscle region, an ellipse-shaped mass (multiple calcifications) with a well-defined margin in the right buccal region, and a comma-shaped mass (no calcifications) with a well-defined margin separate from the parotid gland in the right accessory parotid gland region. These iconographic findings suggested that the masses were all hemangiomas separately originating from the parotid gland, accessory parotid gland, and masseter muscle. The masses were completely removed through a standard parotid incision without postoperative facial palsy, skin deformity, and difficulty in secreting saliva. Findings from histologic examination of the tumor revealed multiple, thin-walled, and dilated blood vessels, confirming the diagnosis of cavernous hemangiomas. Ultrasonographic and computed tomographic findings were extremely useful in diagnosing the mass/masses as hemangioma before surgery, clarifying relationships between the mass and adjacent structures, and determining the surgical approach to the mass/masses.

Auriculotemporal nerve syndrome in association with congenital haemangiopericytoma: a case report.

BACKGROUND: Auriculotemporal nerve syndrome is characterised by recurrent episodes of facial gustatory flushing and/or sweating along the cutaneous distribution of the auriculotemporal nerve. The condition is rare in children and is normally a sequel of perinatal birth trauma. We report a case of a sixteen-month-old boy referred by paediatric oncology with recurrent, unilateral facial flushing of the left cheek which had been present for 2 months. The flushing only occurred during mastication. The patient had also received treatment for a rare vascular tumour, congenital haemangiopericytoma, of the left cheek and parotid region. The possible association between auriculotemporal nerve syndrome and congenital haemangiopericytoma is discussed. Knowledge of the presentation, aetiology and management of Auriculotemporal Nerve Syndrome can provide much needed reassurance to those suffering with this condition.

Sialoblastoma: a clinicopathologic and immunohistochemical study of 7 cases.

Sialoblastoma is a rare congenital or perinatal salivary tumor that varies in histologic features and biologic potential. Seven cases from the files of the Armed Forces Institute of Pathology are presented. These tumors occurred in 4 males and 3 females with ages ranging from prenatal to 6 months at the time of discovery. Five lesions originated from the parotid gland; 2 lesions were from the submandibular gland. All lesions presented as nodular to multinodular swellings and ranged in size from 2.0 to 7.0 cm. The principal sign or symptom was rapid growth. Two histologic patterns with differing behavior predominated: (1) a favorable pattern had semiencapsulation of cytologically benign basaloid tumor cells with intervening stroma; and (2) an unfavorable histology of anaplastic basaloid tumor cells, minimal stroma, and broad pushing to infiltrative periphery. Four and three tumors had favorable and unfavorable growth patterns, respectively. One unfavorable lesion had vascular invasion, and another demonstrated perineural invasion. All 3 tumors with unfavorable histology recurred. Tumor cells in 3 cases were immunohistochemically reactive for keratin, S-100, smooth muscle actin, and calponin to varying degrees. All 3 tumors were reactive for p63. alpha-Fetoprotein was expressed in 2 unfavorable tumors. Ki67 was expressed at 3% in a favorable tumor and 40% and 80% in the 2 unfavorable lesions. Treatment involved surgical excision. One patient received adjuvant chemotherapy. Two sialoblastomas resulted in recurrences within a year and another developed a recurrence after 4 years. One sialoblastoma developed lung metastasis within 1 month of the original biopsy. Although a clinical correlation is suggested by a favorable/unfavorable histologic grading system the biologic behavior is nonetheless considered unpredictable.

Lesions of the accessory parotid gland in children.

The accessory parotid gland, a little-known and seldom-mentioned anatomical variant, is a nodule of normal salivary tissue separate from the main parotid gland, located on the masseter muscle and connected to the Stensen duct at that level. It can be the site of both congenital and acquired lesions.

Infantile congenital parotid lipomatosis: a rare case report.

A case of congenital parotid lipomatosis in an infant is reported and literature is reviewed. This rare condition in children presents as gradually increasing parotid swelling, which is difficult to diagnose preoperatively as this condition is not considered in the differential diagnosis of a parotid mass. Complete excision with superficial or total parotidectomy with preservation of facial nerve is the treatment of choice.

Congenital sialolipoma of the parotid gland first reported case and review of the literature.

Tumours of the parotid gland in children are uncommon, and represent only 1.3% of all benign salivary tumours. Lipomas of the parotid are also rare, and account for 0.5% of all parotid gland tumours. Sialolipoma is a new variant of salivary gland lipoma, consisting of adipose and glandular tissue that was first proposed by Nagao et al. in 2001. Ten cases of parotid gland lipoma associated with glandular elements have been previously reported in the literature. All have been in adults and none in children. We present the first reported case of congenital sialolipoma that had developed in a female infant. It was managed successfully by superficial parotidectomy undertaken at ten weeks of age.

Management of parotid hemangioma in 100 children.

Most problematic infantile hemangiomas are successfully treated with pharmacological therapy. However, there are reports that hemangioma of the parotid gland responds poorly to corticosteroid and interferon. To better clarify the management of parotid hemangioma, the authors retrospectively studied the records of 100 consecutive patients, seen between 1975 and 2002. The characteristics of the tumor, including sex ratio, presence at birth, size, side, complications, and involvement of adjacent structures, were recorded. The indications for and response to treatment and the need for surgical procedures were documented and statistically analyzed. The female-to-male ratio was 4.5:1. Forty percent of parotid hemangiomas were on the right side, 36 percent were on the left, and 24 percent were bilateral. Forty-five percent of patients had a premonitory cutaneous lesion at birth. Fifty-nine percent of parotid hemangiomas ulcerated during the early proliferative phase. Eighty-eight percent involved nearby structures (ear, 70 percent; lip, 34 percent; subglottic region, 21 percent; eye, 18 percent; and nose, 3 percent). Seven percent of patients required tracheostomy, and 3 percent had signs of congestive heart failure. Seventy infants received pharmacological treatment. Sixty-seven patients were initially managed with corticosteroids; regression or stabilization was noted in 83 percent of tumors (56 of 67 tumors). Twenty-one patients received interferon: 11 in whom corticosteroid therapy had failed, seven in whom the tumor stabilized with corticosteroid therapy but further regression was needed, and three who had interferon as primary therapy. Ninety-five percent of the lesions that were resistant to corticosteroid subsequently responded to interferon alfa-2a or -2b. The overall response rate to pharmacological therapy was 98 percent. A reconstructive procedure was necessary during the involuting or involuted phase in 66 percent of patients: 92 percent had preauricular excision of redundant skin and/or fibrofatty tissue and 37 percent of patients had auricular revision. In summary, drug therapy was effective in the majority of infants with parotid hemangioma, whether given because the tumor was large, deforming, ulcerated, or involved nearby structures with functional consequences. Infantile hemangioma in the parotid gland responded to pharmacological treatment in a similar manner as hemangioma in other locations.

Sonographic appearance of a congenital parotid gland hemangiolymphangioma simulating malignancy in an infant.

Congenital hemangiolymphangioma, also called mixed angioma, is a benign tumor that very rarely occurs in the parotid gland. We present a rare case of hemangiolymphangioma of the parotid gland in a 4-month-old boy whose clinical and radiologic presentation simulated malignancy. Gray-scale and color Doppler sonography revealed an enlarged left parotid gland and inhomogeneous hypoechoic and hyperechoic areas scattered throughout the gland. Mild internal vascularity was noted on color Doppler sonographic examination. CT revealed a predominantly fatty mass involving the superficial lobe of the parotid gland. The results of fine-needle aspiration cytology were inconclusive, and total parotidectomy was performed. Histopathologic examination of surgical specimens confirmed a diagnosis of hemangio lymphangioma. The patient recovered well and was free of recurrence at the 6-month follow-up visit. To our knowledge, this case report is the first to describe the findings of congenital hemangiolymphangioma of the parotid gland on sonography and CT. This rare diagnosis should be considered in neonates and infants presenting with a rapidly growing parotid gland tumor suggesting malignancy. Histopathologic examination is necessary to confirm the diagnosis.

Primary angiosarcoma of the parotid gland arising from benign congenital hemangioma.

A case of malignant transformation of a benign congenital hemangioma of the parotid gland is presented. The malignant tumor occurred in a woman with a history of congenital hemangioma surgically removed 8 years previously. No radiotherapy had been administered at the time of primary excision. The recurrent tumor consisted of a large lesion occupying nearly all the parotid gland and infiltrating the surrounding soft tissues and overlying skin. Its histopathologic features were typical of epithelioid angiosarcoma. The vast majority of vascular lesions of major salivary glands are benign. However, pathologists should be aware of the remote possibility of malignant transformation in these lesions.

Sialoblastoma.

Tumours of the salivary gland are very uncommon in children. We present a case of sialoblastoma, a very rare congenital salivary-gland tumour, initially recognised on antenatal ultrasound. After histological diagnosis, a superficial parotidectomy was performed at 21 days of age with preservation of the facial nerve. There is no recurrence at2 years.

The congenital basal cell adenoma of salivary glands. Contribution to the differential diagnosis of congenital salivary gland tumours.

Congenital epithelial tumours of the salivary glands are very rare. The Salivary Gland Registry maintained in the Department of Pathology. University of Hamburg, contains only three cases among a total of 6,646 salivary gland tumours from the years 1965-1994. The three cases were classified as congenital basal cell adenoma, two of the parotid gland and one of the submandibular gland. Histologically, the three adenomas were similar in structure to the adult counterpart of basal cell adenoma with solid, trabecular or tubular (duct-like) patterns. In some cystic spaces of the duct-like structures PAS- and Astra blue-positive substances were secreted. On immunocytochemistry, the luminal duct-like cells showed membranous expression of cytokeratins 3, 5, 6, 7, 13 and 19. In the isomorphic basaloid cells of the solid and trabecular cell nests few cells expressed cytokeratin. On the outside of the solid cell nests there were smaller elongated myoepithelial-like cells, which expressed cytokeratin 14 and vimentin. Cytokeratins 1, 2, 4 and 18 were not expressed. The pattern of expression reflects the different stages of maturity of the tumour cells and is related to the development of the salivary glands until the end of the 3rd embryonal month with an arrest of further cell differentiation. No acinic cells, invasive growth, recurrence or metastases were observed. The differential diagnosis includes other congenital salivary gland tumours, such as hybrid basal cell adenoma-adenoid cystic carcinoma, sialoblastoma or embryoma, carcinoma, hamartoma and teratoma.