Objective:To summarize the clinical characteristics, diagnosis and treatment experience of salivary pleomorphic adenoma in children. Methods:Thirty patients with salivary pleomorphic adenomas treated in Beijing Childrens Hospital from January 2008 to December 2022 were retrospectively reviewed, including 11 boys and 19 girls, with the age ranging from 0.3 to 14.4 years（median age 10.4 years）. Initial presentation, medical history, imaging workups, surgical approaches, complications, rates of recurrence were evaluated. Results:Major salivary gland lesions were most common（n=24, 80%）; 53.3%（16 of 30） arising in the submandibular glands and 26.7%（8 of 30） in the parotid. Minor salivary gland lesions（n=6, 20%） were removed from the palate, tongue, face, trachea, nasopharynx, and upper mediastinumand. Preoperative imaging was reviewed in all patients and consisted of 26 ultrasound exams, 2 computerized tomography（CT） exams, and 15 magnetic resonance imaging（MRI） exams. Fine needle aspiration biopsy was performed in 12 patients. Surgical excision was performed in all patients. Postoperative complications included transient facial paresis（n=3）, Pneumonia and pleural effusion（n=1）. Average length of follow-up was 36.7 months; confirmed recurrence occurred in one patients. Conclusion:The symptoms of salivary gland pleomorphic adenoma in children are different according to the location of the tumor. The treatment is complete surgical resection, and a small amount of normal tissue around the tumor should be removed to reduce recurrence.
Humans ; Male ; Female ; Child ; Adenoma, Pleomorphic/diagnosis* ; Adolescent ; Retrospective Studies ; Salivary Gland Neoplasms/diagnosis* ; Child, Preschool ; Infant ; Neoplasm Recurrence, Local

Parotid gland tumors are usually solitary tumors, and multiple tumors of the parotid gland are extremely rare. We present a highly unusual case of bilateral and simultaneous pleomorphic adenoma and basal cell adenoma of the parotid gland. We review the literature and discuss the clinical manifestations, diagnosis, and treatment of these two rare tumors.
Adenoma, Pleomorphic ; Humans ; Parotid Gland ; Parotid Neoplasms/diagnosis*

PURPOSE: When there is a mass in the superior temporal orbit area, a lacrimal gland tumor should be suspected. We report a rare case of orbital lymphatic malformation that was histologically diagnosed in a patient with typical clinical features of the lacrimal gland. CASE SUMMARY: A 55-year-old female with no underlying disease and no ophthalmic history visited our clinic with a right upper eyelid edema associated with an enlarged painless eyelid mass 1 month prior to her visit. The patient stated that she discovered the mass 1 year previously.The palpebral lobe of the lacrimal gland protruded slightly with congestion of the surrounding conjunctiva. Enhanced computed tomography showed a 3 cm well-defined heterogeneous mass in the right lacrimal gland area and several well-defined round calcifications within the mass. Orbital tissue or bone involvement was not observed. The pleomorphic adenoma of the lacrimal gland was the most clinically suspicious, so complete resection of the mass was performed using lateral orbitotomy. Histopathologically, lymphangioma (lymphatic malformation) originating from the lacrimal gland was diagnosed. CONCLUSIONS: Orbital lymphatic malformation can occur in the lacrimal gland. The present case showed that differential diagnosis can reveal the presence of an adult lacrimal gland tumor.
Adenoma, Pleomorphic ; Adult ; Conjunctiva ; Diagnosis, Differential ; Edema ; Estrogens, Conjugated (USP) ; Eyelids ; Female ; Humans ; Lacrimal Apparatus ; Lymphangioma ; Middle Aged ; Orbit

Schwannoma is a benign tumor rarely found in the head and neck and much less commonly found in the intraparotid facial nerve. It is a slow-growing encapsulated tumor originating from the Schwann cells or axonal nerve sheath. It can occur anywhere along the course of the facial nerve. Patients may present with symptoms of facial palsy, but the most common presenting symptom is an asymptomatic swelling. Diagnosis is usually difficult before surgical removal and histopathological examination. We report a rare case of intraparotid facial nerve schwannoma in a 57-year-old female who had sustained a mass of the right preauricular area for 3 years. She reported no pain or facial muscle weakness. Enhanced computed tomography findings revealed the impression of pleomorphic adenoma. However, intraoperative gross findings were not characteristic of pleomorphic adenoma, and a frozen biopsy was performed resulting in the impression of a nerve sheath tumor. We performed an extracapsular surgical excision without parotidectomy. Permanent histopathology and immunohistochemistry reports diagnosed the mass as schwannoma. There were no complications including facial palsy after surgery. No recurrence was found at 6 months after surgery
Adenoma, Pleomorphic ; Axons ; Biopsy ; Diagnosis ; Facial Muscles ; Facial Nerve ; Facial Paralysis ; Female ; Head ; Humans ; Immunohistochemistry ; Middle Aged ; Neck ; Neurilemmoma ; Parotid Gland ; Recurrence ; Schwann Cells

BACKGROUND: Recent findings in molecular pathology suggest that genetic translocation and/or overexpression of oncoproteins is important in salivary gland tumorigenesis and diagnosis. We investigated PLAG1, SOX10, and Myb protein expression in various salivary gland neoplasm tissues. METHODS: A total of 113 cases of surgically resected salivary gland neoplasms at the National Cancer Center from January 2007 to March 2017 were identified. Immunohistochemical staining of PLAG1, SOX10, and Myb in tissue samples was performed using tissue microarrays. RESULTS: Among the 113 cases, 82 (72.6%) were benign and 31 (27.4%) were malignant. PLAG1 showed nuclear staining and normal parotid gland was not stained. Among 48 cases of pleomorphic adenoma, 29 (60.4%) were positive for PLAG1. All other benign and malignant salivary gland neoplasms were PLAG1-negative. SOX10 showed nuclear staining. In normal salivary gland tissues SOX10 was expressed in cells of acinus and intercalated ducts. In benign tumors, SOX10 expression was observed in all pleomorphic adenoma (48/48), and basal cell adenoma (3/3), but not in other benign tumors. SOX10 positivity was observed in nine of 31 (29.0%) malignant tumors. Myb showed nuclear staining but was not detected in normal parotid glands. Four of 31 (12.9%) malignant tumors showed Myb positivity: three adenoid cystic carcinomas (AdCC) and one myoepithelial carcinoma with focal AdCC-like histology. CONCLUSIONS: PLAG1 expression is specific to pleomorphic adenoma. SOX10 expression is helpful to rule out excretory duct origin tumor, but its diagnostic value is relatively low. Myb is useful for diagnosing AdCC when histology is unclear in the surgical specimen.
Adenoma ; Adenoma, Pleomorphic ; Antibody-Dependent Cell Cytotoxicity ; Carcinogenesis ; Carcinoma, Adenoid Cystic ; Diagnosis ; Immunohistochemistry ; Oncogene Proteins ; Oncogene Proteins v-myb ; Parotid Gland ; Pathology, Molecular ; Salivary Gland Neoplasms ; Salivary Glands ; SOX Transcription Factors ; Translocation, Genetic

Oncocytic lipoadenoma is a rare salivary gland tumor composed of adipose tissue and oncocytic epithelial cells in varied proportions. We report a case of an oncocytic lipoadenoma of the submandibular gland, which presented as a submandibular gland mass. The patient was a 65-year-old woman with a right submandibular mass measuring 2 × 2 × 1.6 cm. As a sonographic evaluation and computed tomograph scan gave us the impression of benign submandibular gland tumor such as pleomorphic adenoma, we resected the right side submandibular gland. Grossly, the tumor was well circumscribed with yellow to brown cut surface. Microscopically, the tumor was surrounded by a thin, fibrous capsule and composed of oncocytic epithelial cells admixed with mature adipose tissue. Final diagnosis was an oncocytic lipoadenoma. We discussed here radiologic and pathologic finding of this rare salivary gland tumor.
Adenoma, Pleomorphic ; Adipose Tissue ; Aged ; Diagnosis ; Epithelial Cells ; Female ; Humans ; Salivary Glands ; Submandibular Gland ; Ultrasonography

Tracheal tumors are rare and difficult to diagnose. Moreover, delays in diagnosis are very common because the symptoms are nonspecific. As a result, tracheal tumors are commonly mistreated as chronic obstructive pulmonary disease or bronchial asthma. We report a case of a 49-year-old male who presented with a 3-month history of dyspnea and cough. Chest computed tomography scan showed a 1.5×1.3 cm homogenous tumor originating from the right lateral wall of the tracheobronchial angle into the tracheal lumen as well as a 0.5×0.4 cm round nodular lesion at the right upper lobe with multiple mediastinal lymph nodes enlargement. Bronchoscopic findings revealed a broad-based, polypoid lesion nearly obstructing the airway of the right main bronchus. The patient was diagnosed with pleomorphic adenoma which is the most common benign tumor of the salivary glands, but rarely appears in the trachea. Upon surgery, tracheal pleomorphic adenoma and co-existing active pulmonary tuberculoma that had been mistreated as bronchial asthma over 3 months was revealed. Following surgery, the patient underwent anti-tuberculosis treatment. No recurrence has been detected in the 3 years since treatment and the patient is now asymptomatic.
Adenoma, Pleomorphic ; Asthma ; Bronchi ; Cough ; Diagnosis ; Dyspnea ; Humans ; Lymph Nodes ; Male ; Middle Aged ; Pulmonary Disease, Chronic Obstructive ; Recurrence ; Salivary Glands ; Thorax ; Trachea ; Tracheal Neoplasms ; Tuberculoma ; Tuberculosis, Pulmonary

Chondroid synringoma (CS), pleomorphic adenoma of skin, is a benign tumor found in the head and neck region. CS was first reported in 1859 by Billorth for the salivary gland tumor. The usual presentation is an slowly growing, asymptomatic mass. A 53-year-old female with a history of chondroid synringoma had presented with multiple firm, nodular masses found in the left nostril area. The lesion had been excised 8 years prior and was diagnosed histopathologically, but had gradually recurred. Excision of the mass located in subcutaneous layer revealed four whitish, firm tumors surrounded with capsular tissue. Neither recurrence nor complications occurred during the 18 months follow-up period. In the head and neck region, chondroid syringoma should always be considered in differential diagnosis of soft tissue masses despite its rare incidence. For that reason, excisional biopsy with clear margin is the optimal diagnostic as well as therapeutic choice. We report a case of recurred chondroid syringoma on the nose in female patient.
Adenoma, Pleomorphic* ; Biopsy ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Head ; Humans ; Incidence ; Middle Aged ; Neck ; Nose ; Recurrence ; Salivary Glands ; Skin ; Skin Neoplasms

Chondroid syringoma is a rare mixed tumor of the skin which is composed of both mesenchymal and epithelial cells. Its incidence at less than 0.1% and is frequently located on the head and neck. Chondroid syringoma is easily confused with epidermal cysts. Since malignant forms of chondroid syringoma have been reported, accurate and timely diagnosis is important for proper management. We report clinical and histological features of chondroid syringoma in 5 patients treated at our institution. In most of the cases, chondroid syringoma presented as a round, firm, nodular or cystic lesion that had well marginated heterogeneity in sonography. Clinically, all of the lesions were removed by simple excision. Microscopically, all five tumors were well circumscribed and consisted of epithelial, myoepithelial, and stromal components. The epithelial component formed tubules lined by one or more rows of eosinophilic epithelial cells. The outer layer of tubules appeared to be flattened myoepithelial cells. The stroma is myxoid and contained spindle shaped myoepithelial cells. We expect that the clinical, sonographic, and histological data from our report may help clinicians who are confronted with various kinds of analogous facial lesions to decide the most proper management for their patients.
Adenoma, Pleomorphic* ; Diagnosis ; Eosinophils ; Epidermal Cyst ; Epithelial Cells ; Facial Neoplasms ; Head ; Humans ; Incidence ; Neck ; Population Characteristics ; Skin ; Skin Neoplasms ; Ultrasonography

Chondroid syringoma is a rare mixed tumor of the skin which is composed of both mesenchymal and epithelial cells. Its incidence at less than 0.1% and is frequently located on the head and neck. Chondroid syringoma is easily confused with epidermal cysts. Since malignant forms of chondroid syringoma have been reported, accurate and timely diagnosis is important for proper management. We report clinical and histological features of chondroid syringoma in 5 patients treated at our institution. In most of the cases, chondroid syringoma presented as a round, firm, nodular or cystic lesion that had well marginated heterogeneity in sonography. Clinically, all of the lesions were removed by simple excision. Microscopically, all five tumors were well circumscribed and consisted of epithelial, myoepithelial, and stromal components. The epithelial component formed tubules lined by one or more rows of eosinophilic epithelial cells. The outer layer of tubules appeared to be flattened myoepithelial cells. The stroma is myxoid and contained spindle shaped myoepithelial cells. We expect that the clinical, sonographic, and histological data from our report may help clinicians who are confronted with various kinds of analogous facial lesions to decide the most proper management for their patients.
Adenoma, Pleomorphic* ; Diagnosis ; Eosinophils ; Epidermal Cyst ; Epithelial Cells ; Facial Neoplasms ; Head ; Humans ; Incidence ; Neck ; Population Characteristics ; Skin ; Skin Neoplasms ; Ultrasonography