Primary Acinic Cell Carcinoma Research Articles

Primary acinic cell carcinoma in an adolescent female

Acinic cell carcinoma (ACC) is a rare, low-grade tumour, accounting for about 17% of all primary salivary gland malignancies in adults. ACC most commonly affects the parotid gland, predominating fifth and sixth decades of life and a female predilection. In the paediatric population, mucoepidermoid carcinoma is the most common parotid malignancy, followed by ACC.We report a case of a 16-year-old female, presenting with a slow-growing right parotid lump. Clinical examination revealed a small right parotid nodule, grade I House-Brackmann score and cervical lymphadenopathy. Radiological investigations revealed a well-circumscribed lobulated lesion in the anterior right parotid gland and reactive lymph nodes. Surgical management included a right partial parotidectomy. Immunohistochemistry was negative for S100, positive with DOG-1 and widespread PAS-D-resistant granules throughout the tumour cells, confirming a diagnosis of ACC.ACC is an important differential diagnosis to consider in salivary gland pathology in the female adolescent patient.

Primary acinar cell carcinoma of the body and tail of the pancreas in a child: A rare case report

Acinar cell carcinoma (ACC) of the pancreas is a rare neoplasm which is mostly diagnosed in late adulthood. This study aims to report an incidental finding of ACC in a child who was treated successfully with surgery. A 9-year-old male presented with signs of delayed growth. Physical examination revealed a swelling in the left upper abdomen, which was shown to be a pancreatic tumor on a CT scan. Total surgical excision of the neoplasm was performed and the histopathology report confirmed the diagnosis of ACC. Pancreatic ACC is very rarely reported in children and its signs and symptoms are non-specific. Its diagnosis needs a high index of suspicion and CT scan is a good imaging modality for detection. Resectable tumors are best managed with complete removal of the cancer, while unresectable neoplasms can be treated with chemotherapy. However, treatment guidelines and prognosis of pancreatic ACC are not clear in pediatric age groups due to scarcity of reports. Accidental finding of ACC involving the body and tail of the pancreas is extremely rare in children. Early detection and proper management provide an excellent outcome.

DOG-1 positive primary acinic cell carcinoma of the lung and investigation of molecular status

Primary acinic cell carcinoma (ACC) of the lung is an extremely rare neoplasm that more often arises near to a right bronchus. It is characterized by two populations of clear and dark eosinophilic cells, arranged in a glandular acinar pattern. Mitosis are rare and tumor cells show small and eccentric nuclei. Positive stain for PAS, PAS-D, cytokeratin, A1AT and A1ACT is reported, while TTF1, p40, synaptophysin, SMA, and S100 are substantially negative. DOG-1 positive stain was observed in ACC of the salivary glands and its negativity was proposed to distinguish between primary and metastatic ACC of the lung. Here, we report the 30th case of primary ACC of the lung, describing the immunohistochemical positivity for DOG-1 and the molecular status of the neoplasm for the first time.

1053 Primary Acinic Cell Carcinoma in a Young Female: A Case Report

Abstract Background Acinic cell carcinoma (ACC) is a rare, low-grade tumour, accounting for about 5% of all primary salivary gland malignancies. ACCs predominate in the parotid gland, seen in the fifth and sixth decades of life and has a female predilection. Well-differentiated and low-grade tumours are associated with a favourable prognosis. Case Description We report a case of a 16-year-old female, presenting with a right parotid lump, slowly increasing over the last year. Clinical examination revealed a 1.5cm right parotid nodule with no facial nerve involvement and presence of cervical lymphadenopathy. Past medical history was unremarkable. Findings Radiological investigations revealed a well-circumscribed lobulated lesion in the anterior right parotid gland with presence of reactive lymph nodes bilaterally. Differential diagnoses included Warthin's tumour, which would have been unusual in this age group, and the patient underwent a right partial parotidectomy. Histological examination showed a multinodular appearance with some marked granular cytoplasm with oncolytic and lymphoid infiltrate. Immunohistochemistry was negative for S100, positive with DOG-1. There were widespread PAS-D-resistant granules throughout the tumour cells, with ki67 proliferation index of approximately 5%. Results The tumour was completely excised, and the patient made a full recovery and considered to have a good long-term prognosis. Conclusion This is an unusual case of ACC seen in this age group and gender, where less than 4% of cases of ACC have been reported in patients younger than 20. It is important to consider differential diagnoses of salivary gland pathology, such as ACC in the young female patient.

Right pneumonectomy for primary large acinic cell carcinoma (AciCC) with severe mediastinal deviation: a case report and literature review

BackgroundPrimary lung acinic cell carcinoma is very rare. Here we report a young female patient who suffered the largest primary lung acinic cell carcinoma with severe mediastinal deviation which has never been reported before. We also reviewed data and features of 20 previously reported cases of primary lung acinic cell carcinoma who underwent lobectomy.Case presentationA 27-year-old female patient presented with recurrent coughing and hemoptysis for more than 10 years came to our hospital. A chest computed tomography (CT) showed a giant space-occupying lesion in the hilum of right lung. After a thorough and detailed preoperative examination, the patient then was performed a radical right pneumonectomy with mediastinal lymph node dissection. The size of the tumor was about 8.6 × 4.5 × 4.4 cm. The pathological results demonstrated a primary acinic cell carcinoma of right lung. The immunohistochemistry of the tumor showed AE1/AE3 (+), Ki-67 (2% +), CK7 (+), Vimentin (+), CK19 (+), α1-ACT (+), AB-PAS (+), S-100 (−), TTF-1 (−). The patient was discharged less than 2 weeks after the operation. So far, the patient has been followed-up for 2 years, and no evidence of tumor recurrence or metastasis was observed.ConclusionsThe primary acinic cell carcinoma of lung in this case is the biggest one ever reported and also the first case treated with radical right pneumonectomy. In addition, the patient had a very rare condition of severe mediastinal deviation at the same time. After surgical treatment, the patient recovered uneventfully and had stable disease without recurrence and metastasis after 2 years of follow-up. This case together with the reported case indicate that primary acinic cell carcinoma of lung is of low malignancy, the prognosis and therapy effect of surgical treatment are relatively satisfactory.

P36.01 Primary Acinic Cell Carcinoma of Bronchial Ground Origin: A Case Report

Primary acinic cell carcinoma of the lung is an extremely rare disease. Also known as Fechner’s tumor, this condition was first reported by Fechner et al. in 1972. The accurate diagnosis of primary pulmonary acinic cell carcinoma is difficult and relies on comprehensive evaluation of clinical, histological and immunohistochemical features. A 49-year-old male patient was referred to our hospital because of an abnormal infiltration shadow in the left lower lung field detected on a chest radiograph during a medical check-up. Chest computed tomography revealed a nodal mass shadow 10mm in size, in segment 8 of the left lower lobe. Bronchoscopic examination revealed a polypoid tumor at B8. Transbronchial biopsy was performed and the tumor was diagnosed as a bronchial gland adenoma. The patient underwent video-assisted thoracic segmentectomy to achieve complete resection of the tumor. Macroscopically, the tumor was a solitary mass measuring 14×10×8 mm in size with substantial cut surface and showed no intraparenchimal growth. Histopathological examination revealed a polypoid projection in the bronchial space. Adeno-type cancer cells with monomorphous nuclei invade the bronchial wall tissue in association with tubule or acinus-like space formation. The cytoplasm of the tumor cells is plump and eosinophilic with granularity or clear. No direct invasion into the lung parenchyma was observed. Immunohistochemical staining revealed that the tumor cells were positive for PAS, lactoferrin, and HMG-45MI. The features are consistent with acinic cell carcinoma of bronchial gland origin. Electron microscopy showed that the acinus-forming tumor cell possess well-developed mitochondria and round-surfaced endoplasmic reticulum, in association with active luminal secretion. Rounded, electron-dense secretory exocrine granules, ranging from 150 to 340 nm in size, are noted mainly in the apical cytoplasm. Fat vacuoles are noted in some tumor cells. Microvillous intracytoplasmic lumina are occasionally noted. Bundles of intermediate filaments and desmosomal attachments are also seen. These features are consistent with acinar cell nature of the cells. Fourteen years since the surgical resection, no apparent recurrence or metastasis has been detected. We experienced an extremely rare case of primary bronchial acinic cell carcinoma diagnosed based on clinical information, histopathological features, immunogistopathological staining profile, and electron microscopy features. The limited number of reports on this type of tumor have revealed that the outcome after surgical resection is favorable due to its low-grade malignant potential. Likewise in this case, the patient remained free of recurrence for over 14 years after surgery.

A Case of Primary Acinic Cell Carcinoma of the Breast

A Case of Primary Acinic Cell Carcinoma of the Breast

Perithyroidal Salivary Gland Acinic Cell Carcinoma: Morphological and Molecular Attributes of a Unique Lesion

Rarely, salivary gland tumors such as mucoepidermoid carcinoma, mammary analogue secretory carcinoma and mucinous carcinoma arise as primary tumors from ectopic or metaplastic salivary gland tissue adjacent to or within the thyroid gland. We report for the first time a case of primary salivary acinic cell carcinoma (AcCC) adjacent to the thyroid gland in a 71-year-old female patient with Crohns disease and a previous history of malignant melanoma. Following the development of a nodule adjacent to the left thyroid lobe, a fine-needle aspiration biopsy was reported as consistent with a follicular lesion of undetermined significance (Bethesda III). A left-sided hemithyroidectomy was performed. A circumscribed lesion measuring 33 mm was noted adjacent to the thyroid and trapping parathyroid, it was composed of solid nests and glands with microcystic and follicular patterns. The tumor was negative for thyroid, parathyroid and paraganglioma markers, but positive for pan-cytokeratins, CK7, CD10, CD117, androgen receptor and HNF-beta. A metastasis of a thyroid-like renal cell carcinoma was suspected but ruled out, and the patient had no evident lesions on extensive radiology of the urogenital, pulmonary and GI tracts. Based on the morphology, a diagnosis of AcCC was suggested, and confirmed with DOG1 and PAS-diastase staining. Molecular analyses pinpointed a constitutional ASXL1 variant of uncertain significance, but no fusion events. The patient had no radiological or clinical evidence of parotid, submandibular or sublingual tumors postoperatively, and the excised lesion was therefore assumed to be a primary tumor. We here detail the morphological and immunophenotypic profile of this previously undescribed perithyroidal tumor.

Une présentation inhabituelle d’un carcinome à cellules acineuses

Acinic cell carcinoma (ACC) is a low grade malignant tumor of the salivary glands. Primary ACC affects most frequently the parotid gland and can rarely arise in the minor salivary glands of the oral cavity, pharynx and larynx. It is extremely rare in the nasal cavity; to our knowledge only 18 cases of primary ACC of the nasal cavity are reported in the English-written literature. Herein we report a case of acinic cell carcinoma of the nasal cavity, describe the clinical, radiological and microscopic features of this uncommon presentation and finally provide a discussion in the light of relevant literature.

Primary Pulmonary Acinic Cell Carcinoma: A Clinicopathological Study of 6 Cases and Literature Review.

Primary pulmonary acinic cell carcinoma (ACC) is rare. The clinicopathological features are not identical to that of classic ACC that leads to misdiagnosis. In this article, we summarized the clinicopathological features of 25 such cases, including 6 cases in this series and additional 19 cases in the literature. Pulmonary ACCs showed an overwhelming solid growth pattern. The neoplastic cells had eosinophilic granular and clear cytoplasm in most cases and displayed basophilic cytoplasm in only 4 cases. Intratumoral fibrous septa, mitotic figure, necrosis, and psammoma bodies were observed in some cases. Prominent nuclear atypia and perineural invasion might suggest high-grade transformation, metastasis, and recurrence. The tumor cells were strongly positive for CK8/18 and negative for TTF-1, p63, S-100, mammaglobin, MUC5b, MUC5ac, and DOG1. CK7 was exclusively positive for neoplastic cells with ductal differentiation. Of the 25 included cases, 10 cases were initially misdiagnosed. The tumor was prone to involve the right bronchus. The patient outcome was favorable. The accurate diagnosis of primary pulmonary ACC relies on comprehensive evaluation of histological and immunohistochemical features and realization of the difference from classic ACC.

Treatment Challenges of Primary Acinic Cell Carcinoma of the Breast

We present a rare case of primary acinic carcinoma in the breast, which is almost always associated with salivary glands. We will discuss the multidisplinary approach to treatment, highlighting the role of chemotherapy and radiation. Additionally, this tumor was seen on magnetic resonance imaging (MRI), which is not a readily used diagnostic test for breast disease, emphasizing its diagnostic sensitivity in these patients. J Curr Surg. 2019;9(1):11-12 doi: https://doi.org/10.14740/jcs373

Primary acinic cell carcinoma of the esophagus

Primary acinic cell carcinoma of the esophagus

A primary pure pancreatic-type acinar cell carcinoma of the stomach: a case report

BackgroundAcinar cell carcinoma represents only 1–2% of exocrine pancreatic neoplasms. On exceptionally rare occasions, primary acinar cell carcinoma can occur in ectopic locations. Herein, we report a case of pure pancreatic-type acinar cell carcinoma arising in the stomach.Case presentationA 54-year-old male presented with a gastric submucosal mass detected by endoscopic examination. Laparoscopic wedge resection was performed. Macroscopically, the 2.7 cm yellowish mass was located in the submucosa of the stomach. Microscopically, the tumor was well circumscribed and had a homogeneous acinar architecture. The tumor cells were small and had a minimal amount of cytoplasm. The nuclei of the tumor cells were round to oval with finely dispersed chromatin. The tumor cells were strongly positive for α1-antitrypsin, chymotrypsin, and α1-antichymotrypsin immunostaining, consistent with pancreatic exocrine differentiation. There was no clinical or radiologic evidence of primary pancreatic or head and neck tumors. After surgical resection of the tumor, there was no recurrence or metastasis during 33 months follow-up.ConclusionIn this report, we have presented a rare case of primary pure pancreatic-type acinar cell carcinoma arising in the stomach and suggest that it could be helpful if the pathologist were aware that pancreatic-type acinar cell carcinoma could arise in the stomach as a polypoid submucosal tumor in the routine diagnostic field of gastric endoscopy.

Ultrasound and computed tomography features of primary acinic cell carcinoma in the parotid gland: A retrospective study

ObjectiveThe aim of this study was to characterize the ultrasound (US) and computed tomography (CT) findings of primary acinic cell carcinoma (AciCC) of the parotid gland. Subjects and methodsSeventy patients (70 lesions) with histopathologically proven AciCC underwent US or CT examination. The following characteristics were assessed on US images: size, shape, border, echogenicity, echotexture, internal structure, distal acoustic enhancement, and vascularity. The following characteristics were evaluated on CT images: size, shape, border, density, CT values on plain and contrast-enhanced scans, enhancement pattern, enhancement degree, and surrounding bone destruction. ResultsOn US images, lesions were irregular, well-defined, hypoechoic, heterogeneous, and poorly vascularized. On CT images, lesions were regular and well-defined, and showed slight heterogeneous enhancement. ConclusionThese findings suggest that most primary AciCCs show benign features on US and CT.

Primary Acinic Cell Carcinoma of the Breast

Acinic cell carcinoma (ACC) of the breast is a rare tumor that is listed in the 2003 World Health Organization (WHO) classification of tumors of the breast. Pure form ACC of the breast is even rarer. To date, only 12 cases have been reported in the English-language literature. A case of primary ACC of the breast in a 38-year-old woman is presented in this report. Histologically, the neoplastic cells are characterized by widespread acinci cell-like differentiation with a eosinophilic granular or clear cytoplasm, resembling acinic cells of the parotid gland. In addition, the neoplastic cells are positive for lysozyme, EMA, S-100, CD68 and GCDFP-15 and negative for α-anti-chymotrypsin, synaptophysin, ER, PR and Her-2/neu. This primary breast tumor was confirmed by clinical, morphological, and immunohistochemical studies. Characteristic features and differential diagnosis of this tumor were discussed in the light of pertinent literature.

Stromal reaction and prognosis in acinic cell carcinoma of the salivary gland

Primary acinic cell carcinoma (ACC) is an uncommon malignant neoplasm of the salivary gland (SG), which usually presents as slow growing tumor. We reported a 69-year-old woman with tumor in the right parotid gland with a 5-year progress. Biopsy sections revealed a hybrid form of ACC with a low- and high-grade component and prominent lymphoid tissue in tumor stroma. Immunohistochemistry was performed to define the molecular profile of this unusual ACC, with special interest for stromal influence on to the proliferative activity of ACC with dedifferentiation. We detected that the level and the type of stromal lymphoid reaction (particularly CD8+/CD4+ ratio) had a significant influence on to Ki-67 index in the high-grade component of ACC, as well as the involvement of the CXCR4 signaling axis in the stromal reaction influence. We suggest that tumor stroma may be a source of potential new tumor biomarkers which can determine the aggressivity of this tumor.

Primary Acinic Cell Carcinoma of the Breast: A Clinicopathological and Immunohistochemical Study

Acinic cell carcinoma of the breast is an extremely rare, malignant neoplasm characterized by widespread acinar cell-like differentiation and clinically low-grade malignancy. Herein, we report a case of acinic cell carcinoma of the breast in a 41-year-old woman. The tumor was poorly demarcated but had a firm consistency. It was removed with lumpectomy, and sentinel lymph node biopsy was performed to check for metastasis. Microscopically, the tumor showed an infiltrative growth pattern with a combination of solid, trabecular, and microglandular areas. Many of the tumor cells had abundant clear vacuolated cytoplasm containing zymogen-typed granules which resemble acinar cells of the salivary glands. The immunohistochemical profile of the tumor was also similar to that of salivary gland acinic cell carcinoma: the tumor cells were positive for amylase, lysozyme, α-1-antichymotrypsin, S-100 protein, and epithelial membrane antigen and negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2. She received postoperative chemoradiation therapy and has been well for 3 years since surgery. As studies on large series are lacking, further studies are needed to elucidate the biological characteristics of acinic cell carcinoma of the breast.

Primary Acinic Cell Carcinoma of the Breast: A Rare Lesion with Good Prognosis

Primary Acinic Cell Carcinoma of the Breast: A Rare Lesion with Good Prognosis

Skull Base Involvement by Acinic Cell Carcinoma of the Parotid Gland

Objective Describe the clinical course and outcomes of patients with primary acinic cell carcinoma (ACC) of the parotid gland with skull base invasion or metastasis. Design Retrospective case series (1995-2011) at a single institution. Results Ten patients met study criteria. Mean and median time from initial diagnosis of parotid ACC to development of skull base disease were 14.6 and 10.2 years, respectively. Two patients demonstrated skull base disease on initial presentation. Those who pursued further treatment after developing disease at the skull base underwent surgery (4/7), stereotactic radiosurgery (4/7), or external beam radiation (3/7). The 10-year Kaplan-Meier estimated overall survival after initial diagnosis of parotid ACC was 80%. Once skull base invasion occurred, 2-year estimated overall survival was 50%. Conclusion Although primary ACC of the parotid generally caries an excellent prognosis, tumor control with cranial base disease is difficult and the majority of patients present with late aggressive recurrences. Our observations underscore the importance of long-term follow-up in this patient group.

Skull Base Involvement by Acinic Cell Carcinoma of the Parotid Gland

Background: Acinic cell carcinoma (ACC) represents approximately 3% of all adult parotid cancers. Reports in the literature describing skull base ACC are uncommon and limited to case reports. Herein, we describe the presentation, treatment strategies, and characteristics of disease progression for a series of 10 patients who developed primary parotid ACC with skull base involvement.