Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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.
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PMID:Treatment of metastatic sialoblastoma with chemotherapy and surgery. 1651 17

Sialoblastoma is a rare tumor of the salivary gland that commonly occurs in the parotid gland and occasionally in the sub-mandibular gland. The malignant potential of sialoblastoma has been documented in only 3 of 32 cases of sialoblastoma reported thus far. In the last 15 years, we have encountered 2 cases of sialoblastoma, in a newborn and in a 15-year-old boy, both arising within the parotid gland. Case 1 has been previously reported and although there were 2 recurrences, at 1 and 9 years post resection, it has shown benign biological behavior. Case 2 is unusual since the patient presented with metastases. We reviewed the 2 cases, including the 2 recurrences from the first case, for histologic and immunohistochemical differences. Although both cases showed similar cytomorphologic features, there was a significant difference in Ki-67 expression: 20% in case 1 (original tumor), <2% in case 1 (recurrent tumor), and nearly 70-80% in the recent malignant case. The difference is remarkable when combined with p53 expression, which was focally positive in the first case but diffusely positive in the second. This report highlights the potential utility of proliferation markers such as Ki-67 in concert with p53 expression to better predict the biological behavior of a rare but locally aggressive neoplasm.
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PMID:Sialoblastoma: utility of Ki-67 and p53 as a prognostic tool and review of literature. 2000 35

Sialoblastoma is a very rare congenital salivary gland tumor. No consensus has been reached concerning the treatment of this tumor due to its rarity. The treatment of reference is surgery, which can be mutilating, in the case of a locally invasive tumor. The treatment of metastatic disease is also controversial. The authors report a new case of a 6-year-old girl with a progressively growing left parotid mass since birth. The first cytological diagnosis was that of pleomorphic adenoma. Due to local progression, superficial parotidectomy was performed at the age of 3.5 years and revealed a diagnosis of sialoblastoma. Six months later, local recurrence and lung metastasis were treated by neoadjuvant chemotherapy with a very good partial response on the local recurrence and the lung metastasis, allowing complete parotidectomy with sacrifice of the facial nerve. Bilateral lung biopsies after adjuvant chemotherapy showed total necrosis. No recurrence was observed with a follow-up of 1 year. This case and a review of the literature confirm the very good chemosensitivity of this tumor and argue in favor of neoadjuvant chemotherapy for locally invasive tumors rather than extensive mutilating surgery.
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PMID:Sialoblastoma of salivary glands in children: chemotherapy should be discussed as an alternative to mutilating surgery. 2055 35

Sialoblastoma is a rare salivary neoplasm which presents either congenitally or during early infancy. It was originally considered a benign neoplasm, however a number of reported cases have documented locoregional recurrence and distant metastases. Currently, there is no consensus on the appropriate treatment for this neoplasm. We report on long term follow-up of a patient with metastatic sialoblastoma, and a brief discussion of the possible treatment modalities currently being considered.
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PMID:Sialoblastoma- long-term follow-up and remission for a rare salivary malignancy. 2176 12