ADENOCARCINOMA OF MINOR SALIVARY GLAND ORIGIN WITH SKELETAL METASTASIS IN A CHILD

Primary epithelial neoplasms of the salivary gland in children are uncommon but are well recognized and occur principally in the major salivary glands. The purpose of this report is to document our experience with an adenocarcinoma of the buccal submucosa (one of several sites of minor salivary gland tissue) that metastasized to multiple bones as the initial sites of distant disease after a local recurrence. The clinical history, imaging studies, and microscopic sections including immunoperoxidase studies were evaluated from the primary tumor, local recurrence, and a metastatic lesion from the femur. The histopathologic features and immunohistochemical phenotype of the adenocarcinoma in the buccal submucosa supported its salivary gland origin. This case of adenocarcinoma of the intraoral buccal tissues independent of the parotid gland in a 12-year-old female is an unusual clinical presentation of a salivary gland neoplasm in childhood, and its ability to metastasize to distant skeletal sites is also remarkable in terms of a primary salivary gland carcinoma regardless of age at diagnosis.     

Surgery in disabled children: General gastroenterological aspects

Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70–75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation.

Conclusion : This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.     

Surgery in disabled children: general gastroenterological aspects

Cerebral palsy (CP) is a non-progressive but not unchanging disorder of movement and/or posture, due to an insult to or anomaly of the developing brain. Gastrointestinal surgery can play a role in the treatment of pathologies frequently associated with a condition of neurological impairment such as gastro-oesophageal reflux disease (antireflux procedure), feeding difficulties (percutaneous endoscopic gastrostomy/jejunostomy) and swallowing difficulties (ligation of salivary gland ducts). Gastro-oesophageal reflux occurs in up to 70-75% of children with cerebral palsy. Children with gastro-oesophageal reflux disease (GERD) may present with feeding difficulties, recurrent vomiting and recurrent chest infection associated with poor growth and nutrition, reactive airway disease particularly nocturnal asthma, choking attacks, anaemia, and wheezing. Nutritional deprivation in children with cerebral palsy is the summation of several factors which result in reduced intake. Percutaneous endoscopic gastrostomy (PEG) has radically changed the handling of children with nutritional problems who, before the introduction of this procedure, were force fed parenterally or enterally, by nasogastric tube, conventional surgical gastrostomy or central venous access. In children with CP, PEG is the preferred technique for long-term enteral feeding. Swallowing dysfunction is the main cause of drooling in cerebral palsy, and medical treatment is often inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct, salivary gland resection or salivary duct (parotid and submandibular) ligation. CONCLUSION: This review focuses on the role of surgery in managing gastrointestinal aspects in children with CP and, in particular, surgical experience at our department with fundoplication, PEG placement and ligation of salivary ducts.     

An atypical case of parotid gland swelling and arthritis in a child

Background

Early onset sarcoidosis is a rarely reported disease in children.

Case characteristics

2½-year-old girl with chronic enlargement of bilateral parotid glands and polyarthritis.

Observation

Biopsy of salivary gland revealed non-caseating granuloma.

Outcome

Polyarthritis and salivary gland swelling resolved completely after starting oral corticosteroids.

Message

Sarcoidosis is an important differential diagnosis in young children with joint and salivary gland involvement.     

Ultrasonography in the study of salivary gland lesions in children

Salivary gland lesions are uncommon in children and may be related to the parotid, submandibular or sublingual glands. Inflammatory lesions are the most common cause of salivary gland abnormalities in children and can be due to acute viral, acute suppurative, or recurrent acute or chronic inflammation. Intraparotid lymphadenitis may also occur, as in cat-scratch disease or in other causes of cervical lymphadenitis. Salivary gland neoplasms are rare in children, and most of them are benign including mainly hemangioma, pleomorphic adenoma, or lymphangioma. Other lesions, such as sialolithiasis, mucocele, or ranula, may also be seeen. Ultrasonography should be the initial imaging study used for the examination of salivary gland lesions in children, given the fact that most of such lesions are benign and are shown up clearly by sonography. In most cases, this technique permits the differentiation of intraglandular and extraglandular lesions, and may suggest the correct diagnosis. The entire lesion could not be totally depicted by US however, and other imaging techniques such as CT or MRI may be necessary. Vascular lesions can be demonstrated more clearly through the use of color Doppler imaging. Some of the lesions may appear similar, and clinical correlation is important for the differential diagnosis. This article discusses the sonographic appearance and clinical manifestations of the spectrum of salivary gland abnormalities that may occur in children. Received: 3 September 1997 Accepted: 31 December 1997     

Pediatric salivary gland lesions

The salivary glands comprise three main pairs of glands (ie, the parotid, the submandibular, and the sublingual) and a number of minor glands found in the mucosa of the upper aerodigestive tract. Lesions may be inflammatory or obstructive in nature or may stem from granulomatous or neoplastic disease. As such, establishing a definitive diagnosis is often quite challenging. This article reviews widely used diagnostic approaches and briefly describes various salivary gland lesions within an etiologic framework.     

Generalized Cytomegalic Inclusion Disease in a Newborn Infant

A case of generalized inclusion body disease in a newborn premature male infant is reported. The diagnosis is based on the clinical picture, the demonstration of typical cells in a biopsy from the parotid gland, and a positive antibody titer against salivary gland virus. This is the first patient from Scandinavia in whom the diagnosis has been made intra vitam and who has survived, although with cerebral damage. Several diagnostic features are discussed. Early treatment with corticosteroids is recommended.     

Salivary gland neoplasms in children: the experience of the Istituto Nazionale Tumori of Milan

BACKGROUND: Epithelial salivary gland tumors are very uncommon in pediatric age. We report a series of 52 cases treated at the Istituto Nazionale Tumori of Milan, Italy, over a 30-year period. These results are presented in conjunction with a literature review of salivary tumors with a view to providing an up-to-date overview of the clinical course, prognosis, and treatment options for this rare tumor. PROCEDURE: Fifty-two cases of epithelial salivary tumors were reviewed and the clinical-pathological information concerning tumor characteristics, therapy, and follow-up were collected. Patients' age ranged between 4 and 18 years. RESULTS: The major salivary glands were the main site of tumor occurrence (79% of cases arose in parotid glands); 37 patients had benign tumors (pleomorphic adenoma), 15 had malignant tumors (12 mucoepidermoid carcinoma, 9 low grade). All the patients were treated by surgery; local relapses after parotidectomy were 4% and 25%, in benign and malignant tumors, respectively. When tumor enucleation was performed, recurrences occurred in 50% of benign neoplasms. At the time of the report, all patients with benign tumors were alive, 35(95%) without evidence of disease; only one patient with malignant tumor died of disease. CONCLUSIONS: Epithelial salivary glands tumor in children had different characteristics compared with their adult counterpart with respect to the frequency of histotypes and site of occurrence, but their prognosis seems to be similar. Parotidectomy (total or superficial) is the best choice for achieving good cure rates in both benign and malignant tumors.     

CASE REPORT: CONGENITAL SALIVARY GLAND ANALAGE TUMOR PRESENTING WITH NEONATAL RESPIRATORY DISTRESS

Stridor in the newborn is not an unusual entity. This article describes a rare presentation of neonatal respiratory distress caused by a benign nasopharyngeal salivary gland analage tumor.     

Congenital minor salivary gland sialoblastoma: a case report and review of the literature

Sialoblastoma is the most common epithelial tumor of the salivary gland. We report a case of congenital sialoblastoma arising in a minor salivary gland of the buccal mucosa of a male infant. After radiologic evaluation, an incisional biopsy was performed and then the mass was excised en bloc. Histologic features were both favorable and unfavorable. However, there was no recurrence for 5 months. In spite of a reported histologic grading system, the clinical course of isolated sialoblastoma is considered unpredictable. More published case reports of this rare tumor may enable histologic and clinical correlation in order to accurately predict prognosis.     

MRI and MR sialography of juvenile recurrent parotitis

Background  

Juvenile recurrent parotitis (JRP) is the second most common inflammatory salivary gland disease of childhood, after mumps. Diagnosis of JRP is usually based on clinical history of recurrent unilateral or bilateral parotid swelling and demonstration of sialectasis. Conventional sialography, digital sialography, US, MRI and sialoendoscopy have been used as investigative tools for the diagnosis of JRP. MR sialography is increasingly recognized as a useful supplement to sialography in salivary duct disorders.     

Pediatric chronic sclerosing sialadenitis: Küttner tumor

Chronic sclerosing sialadenitis is an uncommon cause of salivary gland enlargement mainly occurring in the fifth and seventh decade of life. In the Western population, chronic sclerosing sialadenitis has been characterized as an IgG4-related disease. Although rare, this lesion occurs in children. To increase awareness about this entity in the pediatric age group, we report the case of an 11-year-old boy with a hard, 4.0-cm circumscribed mass in the right submandibular gland. Histologically there was marked distortion of the gland architecture by a dense lymphocytic infiltrate and extensive fibrosis with septa that crossed and distorted the gland, leaving atrophic acini and dilated, irregular ducts. The lymphoid infiltrate formed multiple follicles with active germinal centers, numerous plasma cells, and areas with diffuse arrangement. Immunophenotyping showed abundant CD20- and CD3-positive lymphocytes; cytokeratin AE1/AE3 highlighted the distorted architectural pattern; IgG staining showed large numbers of positive cells infiltrating the interstitium and surrounding the atrophic acini and ducts. IgG4 staining revealed a large proportion of positive infiltrating elements. Küttner tumor belongs to the group of IgG4-related sclerosing diseases. The differential diagnosis includes pleomorphic adenoma and other salivary gland neoplasms. Its recognition in children is important clinically because this entity is amenable to steroid treatment, and additional work up and follow up is warranted to stave off other IgG4-related diseases/complications.     

Congenital absence of lacrimal puncta and of all major salivary glands: case report and literature review

A 7-year-old girl had dry mouth and recurrent infections of the lacrimal fistulae with decreased lacrimal secretion. All four puncta were absent, and a Schirmer test showed decreased lacrimal secretion. Salivary gland imaging with sodium pertechnetate 99mTcO4 showed absence of all major salivary glands. Lower lip biopsy disclosed normal structure of the salivary gland. No evidence of abnormal inheritance patterns could be demonstrated.     

Salivary Gland Tumors in Children: A Retrospective Clinical Review

Seven patients with salivary gland tumors who underwent between 1972 and 2012 were retrospectively evaluated. The age of the patients ranged from 6.3 to 13 years old; five were females and two were males. Five patients had stage IVa, one patient had stage I, and one patient had stage II disease. The surgical margin was found to be positive in five cases. There were three adenoid cystic carcinoma, two adenocarcinoma, one anaplastic carcinoma, and one mucoepidermoid carcinoma. There were five parotid, one lacrimal gland, and one palatal involvement. Three patients who had low stage tumors were treated with surgery alone. Four of the cases received adjuvant radiation and chemotherapy. One patient with parotid tumor died with progressive disease. One case with lacrimal gland neoplasm was alive 48 months after discontinuation of treatment. The other palate case was lost to follow-up 1 month after the beginning of the treatment. Surgery is the primary treatment, with radiotherapy and chemotherapy used as adjuvant treatments. The treatment options need to be selected and planned for each individual patient.     

Aseptic sialadenitis in preterm infants associated with long-term oro-gastric tube feeding

Sialadenitis is a rare disease in the newborn and the pathogenesis in this age group is not fully understood. We report five cases of neonatal sialadenitis in stable preterm, gavage-fed infants at 2 to 6 weeks postnatal age. The occurrence of sialadenitis was observed in temporal relation to changes in enteral feeding routines in the unit which were initiated with the objective of promoting full breast feeding at discharge. Clinical presentation consisted of a tender palpable mass over one of the salivary glands, low-grade (37.5–38.3°C) fever in conjunction with clinical malaise. C-reactive protein and white blood cell count were only moderately elevated. No suppuration from salivary ducts was present. Bacterial cultures were invariably negative. Viral investigations were not carried out. Aseptic sialadenitis was suggested as the cause of the symptoms. The clinical course was benign and the local tender mass over the salivary gland vanished shortly after intravenous fluids and antibiotics were started. Conclusion Long-term exclusive oro-gastric feeding may result in reduced reflex salivary gland stimulation, saliva production and hence ductal clearance of mucoid saliva, leading to functional ductal obstruction and local inflammation.     

Primary Gougerot-Sj?gren syndrome in a 13-year-old girl]

Sj?gren's syndrome is uncommon in children, and occurs most often in association with autoimmune diseases (secondary Sj?gren's syndrome). We describe the clinical and biological features of a 13-year-old girl with primary Sj?gren's syndrome, revealed by recurrent parotitis. CASE REPORT: This adolescent girl was referred for investigation of multiple episodes of bilateral parotid swelling since age nine, without systemic symptoms. Examination was unremarkable except for enlarged and painless parotid glands. Laboratory investigations, measurement of saliva production, parotid sialography, labial salivary gland biopsy, revealed Sj?gren's syndrome without associated disease. Hydroxychloroquine was prescribed with clinical improvement. CONCLUSION: Recurrent parotitis in children is an uncommon condition. The onset of parotid swelling at five years or over deserves screening for dysimmune disorders, sarcoidosis or Sj?gren's syndrome. Diagnosis of Sj?gren's syndrome is based on laboratory evidence of autoimmune disorders and minor salivary gland biopsy.     

Surgical treatment of salivary incontinence in cerebral palsy]

Swallowing dysfunction is the main cause of drooling in cerebral palsy. Medical treatment is inefficient. Surgical treatment involves neurectomy, translocation of the salivary duct or salivary gland resection. Following this, reeducation is advisable. Indications are based on the degree of drooling and on the degree of histories mental retardation. The authors present 2 case histories of drooling in patients with cerebral palsy treated by submandibular gland resection and parotid duct ligation.     

Preliminary experience with chemotherapy in advanced salivary gland neoplasms

Eight patients with advanced incurable salivary gland carcinoma were treated with the combination of cyclophosphamide, doxorubicin, and cisplatin (CAP). There were three clinical complete responses and two excellent partial responses. One of the three nonresponders had symptomatic improvement without any response in the measurable disease sites. Side effects were acceptable. All patients had moderate nausea and some degree of vomiting, which were adequately controlled by antiemetics. These results are preliminary but when considered with other reported trials using the same combination suggest an active treatment program for advanced neoplasms of the salivary glands.     

Aldosterone in cystic fibrosis: measurement in saliva and correlation with disease severity

Aldosterone was measured in the saliva of 20 patients with cystic fibrosis and a group of 20 normal children matched for age and sex. Mean levels were higher in the patient group but overall differences were small and statistically not significant. For the first time a link between aldosterone level and disease severity in patients with cystic fibrosis was established using a simple scoring system to assess disease activity. Urinary and salivary electrolytes were similar in the two groups. The results do not support the proposed hypothesis that the salivary glands of patients with cystic fibrosis are insensitive to aldosterone.