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Background
These are malignant tumors of the salivary glands that are dervied from the salivary gland epithelium and characteristically forms glandular acini. It is notable for an unpredictable clinical course with both local recurrence and distant metastases. The vast majority (83%) occur within the parotid gland, usually as a slowly enlarging mass. Pain or tenderness may be present in up to 1/3 of patients.
OUTLINE
PATHOGENESIS CHARACTERIZATION CHROMOSOMAL ABNORMALITIES Genetic alterations in acinic cell carcinoma of the parotid gland determined by microsatellite analysis.
el-Naggar AK, Abdul-Karim FW, Hurr K, Callender D, Luna MA, Batsakis JG.
Department of Pathology, University of Texas, M. D. Anderson Cancer Center, Houston 77030, USA.
Cancer Genet Cytogenet 1998 Apr 1;102(1):19-24 Abstract quote
We investigated, for the first time, the genetic alterations at certain chromosomal loci in 25 primary parotid acinic cell carcinomas to define the most frequently altered chromosomal regions and their association with pathologic features and DNA content analysis.
Our results showed that 21 (84.0%) of the tumors had alteration in at least one of the loci tested. In general, chromosomal regions at chromosomes 4p, 5q, 6p, and 17p were more frequently altered than those on chromosomes 1p and 1q, 4q, 5p, and 6q. Certain markers at 4p15-16, 6p25-qter, and 17p11 regions showed the highest incidence of LOH, suggesting the presence of tumor suppressor genes associated with the oncogenesis of these tumors. LOH was significantly associated only with tumor grade. No apparent correlation between LOH and other clinicopathologic and DNA content characteristics was identified.
Our study broadly defined the chromosomal arms and loci that may be targeted for further localization of the minimally deleted regions involved in the tumorigenesis of these tumors.
RETINOBLASTOMA GENE PATHWAYS
Abnormal expression of Rb pathway-related proteins in salivary gland acinic cell carcinoma.
Liu T, Zhu E, Wang L, Okada T, Yamaguchi A, Okada N.
Section of Oral Pathology, Graduate School of Tokyo Medical and Dental University, Tokyo 113-8549, Japan; Section of Oral Pathology, Dental School, Dalian Medical University, Dalian 116027, China.
Hum Pathol. 2005 Sep;36(9):962-70. Abstract quote
Salivary gland acinic cell carcinoma (ACC) is a relatively rare neoplasm, and limited information is available regarding its molecular pathogenesis. Because the deregulation of Rb pathway is common to most human tumors, we immunohistochemically investigated the expression of Rb pathway-related proteins, including Rb, Rb proteins phosphorylated at serine 780 and 795 (pRb-S780 and pRb-S795, respectively), cyclin D1, and p16(INK4a) in 18 cases of ACC. The expression of topoisomerase II-alpha and Ki-67 was also examined to evaluate cell proliferation.
All the ACCs exhibited substantial numbers of positive cells against Rb antibody that recognizes both unphosphorylated and phosphorylated Rb proteins. The numbers of positive cells for pRb-S795 and cyclin D1 significantly increased in ACCs as compared with normal salivary glands. Double immunofluorescent staining demonstrated that pRb-S795 was colocalized with cyclin D1 in most tumor cells. However, neither significant change of the expression of Rb protein phosphorylated at serine 780 nor its colocalization with cyclin D1 was observed. The loss of p16(INK4a) is infrequent, but its expression was correlated with phosphorylated Rb proteins.
Our results suggest that serine 795 but not serine 780 is the preferred phosphorylation site induced by cyclin D1. This phosphorylation appeared to be critical for inactivation of Rb-mediated growth suppression and may play an important role in the pathogenesis of ACC.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES Cystadenocarcinoma Mucoepidermoid carcinoma Metastatic thyroid papillary carcinoma Polymorphous low-grade adenocarcinoma Epithelial-myoepithelial carcinoma Clear cell adenocarcinoma Clear cell oncocytoma Metastatic renal cell carcinoma
PROGNOSIS AND TREATMENT CHARACTERIZATION PROGNOSIS Poor factors include:
Short duration of symptoms
Incomplete excision
Frequent mitoses
Focal necrosis
Neural invasion
Pleomorphism
Infiltration
Stromal hyalinization
Large size
Involvement of the deep lobe of the parotid glandGENERAL Acinic cell carcinoma in Northern Ireland: a 10-year review.
Napier SS, Herron BT, Herron BM.
Division of Dental Surgery and Pathology, School of Clinical Dentistry, Queen's University, Belfast.
Br J Oral Maxillofac Surg 1995 Jun;33(3):145-8 Abstract quote
12 cases of acinic cell carcinoma diagnosed in Northern Ireland from 1942 to 1982 on which there was at least 10 years' follow-up were reviewed, by pooling cases from all regional pathology laboratories.
Clinical findings at presentation were established from case notes, together with details of eventual clinical outcome. Sections were examined to determine tumour size, morphological pattern and cytological constitution, the presence or absence of cytological atypia, mitotic activity, lymphocytic and desmoplastic responses, and to assess for infiltrative margins and adequacy of excision. The tumours arose in 8 females and 4 males, aged 22 to 86 years at presentation. Eleven tumours were in the parotid, one in the soft palate. Two patients suffered local recurrence, both more than 7 years after diagnosis. Five patients had regional lymph node metastasis, four at presentation. No patient suffered haematogenous metastasis or died of tumour. Of the features examined, incomplete excision increased the risk of local recurrence.
While numbers are small, the acinic cell carcinoma in Northern Ireland is an unpredictable low-grade malignant tumour which is capable of recurrence and metastasis. Adequate excision at presentation appears to be the most appropriate therapy.
Clinico-pathological predictors of recurrence for acinic cell carcinoma.
Timon CI, Dardick I, Panzarella T, Thomas J, Ellis G, Gullane P.
Department of Otolaryngology, Head and Neck Surgery, Toronto Hospital, Ottawa, Canada.
Clin Otolaryngol 1995 Oct;20(5):396-401 Abstract quote
The biological behaviour of acinic cell carcinomas, even if well differentiated, is unpredictable. We studied 45 patients with acinic cell carcinoma followed-up from 10 to 379 months (5 year recurrence-free and survival rate of 69% and 81% respectively), and compared clinico-pathological parameters with outcome.
The presence of a predominately solid architecture was strongly associated with a poor outcome (P < 0.01) and this was the only independent prognostic variable when log rank testing was performed.
Tumour size (> 2.75 cm) was a significant predictor of recurrent deep parotid lobe involvement, the presence of cervical nodal disease and lymphocytic infiltration, although not significant, factors showed a tendency towards recurrence. For acinic cell carcinoma, the predominant solid architecture would appear to be a strong predictor of recurrence.
PLOIDY ANALYSIS Acinic cell carcinoma of the salivary glands: the prognostic relevance of DNA cytophotometry in a retrospective study of long duration (1965-1987).
Hamper K, Mausch HE, Caselitz J, Arps H, Berger J, Askensten U, Auer G, Seifert G.
Institute of Pathology, University-Hospital Hamburg-Eppendorf, West Germany.
Oral Surg Oral Med Oral Pathol 1990 Jan;69(1):68-75 Abstract quote
Acinic cell carcinomas of the Salivary Gland Registry, Institute of Pathology, University of Hamburg, West Germany, from 1965 to 1980 (n = 55) were evaluated retrospectively with respect to histologic, cytophotometric, and clinical data.
The majority of the tumors (92.8%) were located in the parotid gland. Two thirds of the patients were female; one third were male. Mean age at primary diagnosis was 55.4 years. The tumors were graded into highly differentiated (76%) or less differentiated forms (24%) according to classic histologic and cytologic criteria. The clinical course was characterized by no recurrence in 15 cases; in 17 cases, recurrences developed, and 12 patients died of their tumor, some as late as 240 months after primary diagnosis. Differentiation showed a weak correlation with the clinical course. In 35 cases, nuclear DNA content of tumor cells was assessed cytochemically.
The tumors were "diploid" or "near-diploid" in 34 cases; DNA content showed no correlation to the clinical course. As a result of long-term follow-up, it becomes evident that acinic cell carcinoma is prone to develop recurrences and metastases.
Complete tumor removal during the primary operation seems to be important for controlling the disease inasmuch as the ostensible prognostic predictors evaluated here proved to be unreliable.
DNA flow cytometry of acinic cell carcinomas of major salivary glands.
el-Naggar AK, Batsakis JG, Luna MA, McLemore D, Byers RM.
Department of Pathology, University of Texas MD Anderson Cancer Center, Houston.
J Laryngol Otol 1990 May;104(5):410-6 Abstract quote
Fifteen acinic cell carcinomas from an equal number of patients were analysed for their DNA content and proliferative (S-phase) index by flow cytometry from archival tissues. Seven of the carcinomas manifested a diploid DNA content.
None of the patients with diploid acinic cell carcinomas died of their carcinomas and none developed metastases in follow-up periods extending for 10 or more years. Four of eight patients with aneuploid acinic cell carcinomas have died because of their malignancies within a 10 year period after the first surgical removal of the carcinoma. Five of the eight patients exhibited metastases.
Although the number of cases does not permit strong correlations between histopathological features, abnormalities in DNA content and outcome of patients, it was noted that carcinomas with prominent necrosis, tubuloductal differentiation and 'dedifferentiated' areas displayed more aggressive biological courses.
Survival About 16% die from the tumor Recurrence 35%
Usually within first 5 years after resection of the primary tumorMETASTATIC
Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Indianapolis, IN, USA.
Acinic cell carcinoma is a rare, low-grade malignant salivary gland neoplasm. Approximately, 20% of patients experience local recurrence and 10% distant metastasis, often many years after initial presentation.
We present a case of widespread cutaneous metastasis from acinic cell carcinoma arising in a 59-year-old woman with a history of acinic cell carcinoma of the parotid gland 20 years prior to her cutaneous disease.
To our knowledge, is the first report of widespread cutaneous metastasis from acinic cell carcinoma.Acinic cell carcinoma of salivary origin. A clinicopathologic study of 67 cases.
Spiro RH, Huvos AG, Strong EW.
Cancer 1978 Mar;41(3):924-35 Abstract quote
This study reviews a 30 year experience with acinic cell carcinoma.
The tumor arose in the parotid gland in 64 patients, the submaxillary gland in one and minor salivary glands in two. In untreated patients with small tumors, clinical findings usually suggested a benign mixed tumor and a subtotal parotidectomy which spared the facial nerve was highly effective therapy.
In contrast, local recurrence and death was the rule in those few who had locally extensive disease, regardless of how radical an operation was performed. Determine "cure" rates for the entire group were 76, 63 and 55% at 5, 10 and 15 years, respectively. Cervical lymph node metastasis occurred in 16% of the patients, and distant metastasis in 12%.
Survival was most directly influenced by the clinical extent of the primary tumor, and also correlated with certain histologic features which are described.
Acinic cell carcinoma of the salivary glands: the prognostic relevance of DNA cytophotometry in a retrospective study of long duration (1965-1987).
Hamper K, Mausch HE, Caselitz J, Arps H, Berger J, Askensten U, Auer G, Seifert G.
Institute of Pathology, University-Hospital Hamburg-Eppendorf, West Germany.
Oral Surg Oral Med Oral Pathol 1990 Jan;69(1):68-75 Abstract quote
Acinic cell carcinomas of the Salivary Gland Registry, Institute of Pathology, University of Hamburg, West Germany, from 1965 to 1980 (n = 55) were evaluated retrospectively with respect to histologic, cytophotometric, and clinical data.
The majority of the tumors (92.8%) were located in the parotid gland. Two thirds of the patients were female; one third were male. Mean age at primary diagnosis was 55.4 years. The tumors were graded into highly differentiated (76%) or less differentiated forms (24%) according to classic histologic and cytologic criteria. The clinical course was characterized by no recurrence in 15 cases; in 17 cases, recurrences developed, and 12 patients died of their tumor, some as late as 240 months after primary diagnosis. Differentiation showed a weak correlation with the clinical course. In 35 cases, nuclear DNA content of tumor cells was assessed cytochemically. The tumors were "diploid" or "near-diploid" in 34 cases; DNA content showed no correlation to the clinical course.
As a result of long-term follow-up, it becomes evident that acinic cell carcinoma is prone to develop recurrences and metastases. Complete tumor removal during the primary operation seems to be important for controlling the disease inasmuch as the ostensible prognostic predictors evaluated here proved to be unreliable.
TREATMENT Complete surgical excision Radiation may improve survival in patients for whom complete excision cannot be achieved Acinic cell carcinoma of the salivary glands. A long term follow-up study of 15 cases.
Oliveira P, Fonseca I, Soares J.
Servico de Patologia Morfologica, Instituto Portugues de Oncologia de Francisco Gentil-Centro de Lisboa.
Eur J Surg Oncol 1992 Feb;18(1):7-15 Abstract quote
Fifteen cases of acinic cell carcinoma of the salivary glands were evaluated retrospectively with respect to histological and clinical data. DNA content assessment was carried out in six cases by cytophotometry.
The majority of tumors were located in the parotid gland and were Stage I at presentation. There was a female predominance and the mean age at primary diagnosis was 51.2 years for females and 41.0 for males. The solid-acinar cell pattern was the most frequently observed and the tumors were 'diploid' in all the six cases studied. Surgery was the therapeutic modality in all cases (enucleation in seven, superficial parotidectomy in three and total parotidectomy in five) and, in four of them, was complemented with radiotherapy. The clinical course was characterized by recurrence in 10 cases, metastases occurred in three patients and one patient died of the tumor. Of the seven recurrent cases, six were treated by enucleation and one by superficial parotidectomy. The histological pattern showed no correlation with the clinical course or DNA content. Acinic cell carcinoma has a significant morbidity with a high recurrence rate which seems to be largely influenced by the type of surgery employed.
Wide surgical excision of the neoplasia, which includes total parotidectomy in the parotid cases, is recommended in order to reduce the frequency of recurrence of the tumor.
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Last Updated June 19, 2008
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