Cutaneous Metastasis From Parotid Tumors: A Report of 2 Cases and Literature Review.

ABSTRACT: Cutaneous metastases from parotid tumors are uncommon and imply a poor prognosis. In this article, we report 2 new cutaneous metastasis cases from 2 different parotid malignancies and 42 additional cases from a literature review. Clinical manifestations, localization, and outcomes of skin metastasis from parotid tumors are described. Although infrequent, parotid neoplasms may develop skin metastasis many years after the initial diagnosis, mostly in the head and neck region. Therefore, long-term follow-up and periodic skin examination of these patients is mandatory. Dermatologists and surgeons must have a high index of suspicion when evaluating any skin lesion arising on these patients because cutaneous metastasis from parotid neoplasms generally implies a poor prognosis.

Clinical Analysis of Acinar Cell Carcinoma of the Pancreas: A Single-Center Experience of 45 Consecutive Cases.

BACKGROUNDS: Acinar cell carcinoma of the pancreas is a rare malignancy, and its features remain unclear. We aimed to analyze the clinical characteristics, treatment and prognosis of acinar cell carcinoma with our institutional case series. METHODS: Patients diagnosed with acinar cell carcinoma in our hospital between 2005 and 2019 were reviewed. Investigations on clinicopathological features, treatment details and long-term survival were performed. RESULTS: A total of 45 pathologically confirmed acinar cell carcinomas were identified. The median age at diagnosis was 58 years with a male-to-female ratio of 3.1:1. There were 24 (53.3%) localized, 5 (11.1%) locally advanced and 16 (35.6%) metastatic cases, with a pancreatic head-to-body/tail ratio of 1:1.4 for all the primary lesions. In the localized group, there were 10 pancreatoduodenectomy, 12 distal pancreatectomy, 1 total pancreatectomy, and 1 distal pancreatectomy combined with proximal gastrectomy. Among the locally advanced and metastatic cases, 13 patients received chemotherapy, 1 received concurrent radiochemotherapy, 1 underwent synchronous resection of primary tumor and liver metastasis, 1 underwent palliative operation, 1 underwent exploratory laparotomy, and 4 required no treatment. The median overall survival of this series was 18.9 months with a 5-year survival rate of 19.6%. Moreover, the resected acinar cell carcinoma patients were associated with prolonged survival compared with the unresected cases (36.6 vs. 8.5 months, P < 0.001). CONCLUSIONS: Surgical resection could improve the long-term survival of acinar cell carcinoma patients, which might also improve the prognosis of selected metastatic cases. Large-scale studies are needed to further clarify the biological behavior and clinical features, and to seek the optimal treatments.

Pancreatic Acinar Cell Carcinoma with Multiple Liver Metastases Effectively Treated by S-1 Chemotherapy.

A 79-year-old woman was referred for pancreatic tail cancer with multiple liver metastases. The pancreatic tail tumor was diagnosed as acinar cell carcinoma (ACC) histologically by endoscopic ultrasound-guided fine-needle aspiration. Because of multiple liver metastases, S-1 chemotherapy was administered, resulting in a partial response to chemotherapy one year later. After approximately three years, liver atrophy and esophageal varices developed. We suspected S-1 as the cause of the liver cirrhosis. S-1 cessation minimized ascites and improved the esophageal varices. Although S-1 can potentially treat ACC, we should be watchful for liver cirrhosis caused by its long-term administration.

Surgical resection of hepatic and rectal metastases of pancreatic acinar cell carcinoma (PACC): a case report.

BACKGROUND: Pancreatic acinar cell carcinoma (PACC), a rare variant of pancreatic malignancy, is generally managed the same way as pancreatic ductal adenocarcinoma (PDAC). Surgical resection is the gateway to curing it; however, once it metastasizes (usually to the liver, lungs, lymph nodes, or peritoneal cavity), systemic chemotherapy has been the only option, but with unfavorable results. CASE PRESENTATION: A 67-year-old man with symptoms of loss of appetite and weight underwent surgery for malignancy of the pancreatic tail extending into the entire pancreas. The pathological diagnosis was PACC following total pancreatectomy. Twenty-four months after the pancreatectomy, a solitary liver metastasis was treated by partial hepatectomy, and, subsequently, 4 months later, he presented with melena. Further examination revealed a type-2 rectal tumor. Histological examination following biopsy revealed it to be rectal metastasis of PACC, and it was treated by abdominoperineal resection. Subsequently, the patient did not have tumor recurrence as of 40 months after pancreatectomy. CONCLUSIONS: This is a rare case of PACC presenting with metachronal metastases in the liver and rectum, and we successfully treated them by surgical resections. Since the malignant behavior of PACC is usually less than that of PDAC, surgical resection could be an option even for metastatic lesions when the number and extent of metastases are limited.

Four-Year Survival of a Patient With Spinal Metastatic Acinic Cell Carcinoma After a Total En Bloc Spondylectomy and Reconstruction With a Frozen Tumor-Bearing Bone Graft.

Acinic cell carcinoma metastasizing to the spine is extremely rare. The authors present a case of acinic cell carcinoma of the parotid gland with subsequent lung and spinal metastases, treated with en bloc spondylectomy. A 41-year-old man presented with a left parotid mass. After being diagnosed with acinic cell carcinoma, he underwent a total parotidectomy. Imaging studies revealed a metastatic osteoblastic lesion in the T4 vertebral body and multiple lung metastases. Total en bloc spondylectomy and reconstruction with a frozen tumor-bearing bone graft were performed to treat the T4 metastasis. Lung metastases were treated with periodic radiofrequency ablation. At the 48-month follow-up, there was no local recurrence of the lesions, and the lung metastases were controlled. The bone graft had fused with the adjacent vertebrae, and the patient had full neurological function and normal daily activities. This report indicates satisfactory long-term outcomes of total en bloc spondylectomy and reconstruction with frozen tumor-bearing bone graft in a patient with acinic cell carcinoma with spinal metastasis. It also emphasizes the benefits of radical resection of spinal metastasis even in cases with multiple organ metastases. [Orthopedics. 2018; 41(5):e727-e730.].

[Multiple lung carcinoma: Primary or intrapulmonary metastasis?] / Adénocarcinomes pulmonaires multiples : primitifs ou métastases ?

Multiple lung carcinomas are 5 to 11,5% of lung carcinomas. The distinction between primary lung carcinomas from carcinomas with intrapulmonary metastasis is essential for optimal patient management. The histopathological analysis is very useful but it has to be completed by genotypic assessment using molecular biology (NGS). Molecular biology can also identify genetic alterations with therapeutic implications. We present the case of a patient with a history of surgery for multiple lung carcinomas diagnosed from 2013 to 2017.

Severe panniculitis and polyarthritis caused by acinar cell carcinoma arising from an ectopic pancreas.

The pancreatitis, panniculitis and polyarthritis (PPP) syndrome is a rare condition caused by pancreatic diseases, such as acute or chronic pancreatitis or pancreatic carcinoma. We report the first case of PPP syndrome caused by metastatic acinar cell carcinoma from an ectopic pancreas. The symptoms were successfully managed by the treatment of the metastatic carcinoma. Pancreatic cytosteatonecrosis should be always considered in a patient who is showing symptoms of panniculitis and polyarthritis.

Salivary gland epithelial neoplasms in pediatric population: a single-institute experience with a focus on the histologic spectrum and clinical outcome.

Salivary gland epithelial neoplasms are rare in children and adolescents, with only a handful of large series having been published. A retrospective study was conducted for 57 cases in patients 20 years or younger. The tumors were located in the parotid (n=36), submandibular gland (n=7), and minor salivary glands (n=14). Nineteen (33%) tumors were pleomorphic adenoma, whereas the remaining (67%) were malignant. The histologic types of carcinomas were mucoepidermoid carcinoma (MEC, n=19, 33%), acinic cell carcinoma (n=7, 12%), adenoid cystic carcinoma (n=6, 11%), secretory carcinoma (mammary analogue) (SC, n=4, 7%), and myoepithelial carcinoma (n=2, 4%). Ninety-three percent (13/14) of the minor and 58% (25/43) of the major salivary gland tumors were malignant. A 7-year-old girl (2%) with a high-grade MEC died from her disease because of uncontrollable locoregional recurrence. Seven patients (16%) developed recurrence including 2 distant metastases from adenoid cystic carcinoma and 6 locoregional recurrences (2 pleomorphic adenomas, 1 SC, 1 myoepithelial carcinoma, 1 adenoid cystic carcinoma, and 1 MEC). The following parameters were associated with decreased disease-free survival in malignant tumors: elevated mitotic index of >4/10 high-power fields (log-rank test, P<.001), and advanced American Joint Committee on Cancer pT (P=.029) and pN stage (P<.001). In conclusion, myoepithelial carcinoma and SC can occur in the pediatric population and should be considered in the differential diagnosis. Salivary gland malignancies in children appear to have better clinical outcome, associated with a 10-year recurrence-free survival rate of 74% and a 10-year disease-specific survival of 94%.

Clinical outcomes of patients with resectable pancreatic acinar cell carcinoma.

OBJECTIVE: Given the rarity of the disease, the post-resection clinical course of localized pancreatic acinar cell carcinoma (ACC) is largely unknown. Therefore, we aimed to analyze the outcomes of patients with localized pancreatic ACC who underwent curative surgical resection. METHODS: We retrospectively analyzed the outcomes of 20 patients with resectable pancreatic ACC who underwent surgery. RESULTS: Altogether 20 patients were included in the study, with a median age of 57 years and a male predominance. There were eight pure ACC, 10 mixed acinar-neuroendocrine carcinomas and two mixed acinar-ductal adenocarcinomas. Among the 15 patients who were staged histologically, 3, 8 and 4 were at stages IB, IIA and IIB, respectively. Eleven patients received adjuvant chemotherapy (5-fluorouracil-based [n = 9]; gemcitabine [n = 1]; etoposide plus cisplatin [n = 1]). In a median follow-up period of 27.1 months, disease recurred in 10 patients, most commonly in the liver (90%). Median recurrence-free survival and overall survival were 16.9 months and 75.0 months, respectively. Elevation of cancer antigen 19-9 (CA19-9), lymph node metastasis and neural invasion were significantly associated with poor overall survival (P = 0.007, P = 0.027 and P = 0.016, respectively). CONCLUSIONS: Compared with ductal adenocarcinoma, resectable pancreatic ACC has a favorable prognosis after surgery. Considering that distant metastasis is the most common pattern of recurrence, further studies are necessary to define the role of adjuvant chemotherapy for improving survival outcomes.

Acinar cell carcinoma: a report of 19 cases with a brief review of the literature.

BACKGROUND: Acinar cell carcinoma (ACC) is a relatively rare pancreatic neoplasm with poorly defined prognosis. This study aimed to investigate this rare pancreatic neoplasm through comparing patients with ACC to pancreatic ductal cell adenocarcinoma (DCA). METHODS: Tianjin Medical University Cancer Institute and Hospital pathology database was reviewed from 1995 to 2015, and 19 patients with pathologically confirmed ACC were enrolled while 19 conventional DCA patients assigned randomly as control. Retrospective review and follow-up were performed for each patient. Regression methods were used to identify differences between ACC and DCA. RESULTS: In our study, most patients suffered from abdominal or back pain, and no lipase hypersecretion syndrome was observed. For ACC, resected cases had better survival than those without resection, and earlier staging was related to longer survival. Resection with postoperative adjuvant therapy had a better outcome than surgery alone. Twelve cases developed recurrence. Compared to DCA, ACC had earlier staging and better survival. The overall 1-, 2-, and 5-year survival rates for patients with ACC were 73.7, 26.3, and 5 %, respectively. CONCLUSIONS: ACC carries a better prognosis than DCA and a similarly high recurrence rate, while surgical resection proved the best first-line approach for it. A well-planned neoadjuvant or adjuvant chemoradiotherapy indeed benefit the patients with ACC.

[A Case of Curative Resection for Advanced Pancreatic Acinar Cell Carcinoma with Liver Metastasis and Involvement of the Superior Mesenteric Artery after Chemoradiotherapy Following Systemic Chemotherapy].

A 52-year-old man was initially diagnosed with an unresectable acinar cell carcinoma of the pancreas with liver metastasis and involvement of the superior mesenteric artery. After 5 courses of systemic chemotherapy(gemcitabine and S-1), the size of the pancreatic tumor had decreased from 31mm to 19mm and the liver metastasis had disappeared. We initiated chemoradiotherapy( CRT: S-1+56 Gy)for further local treatment. After CRT the size of the pancreatic tumor decreased to 13mm and his serum CA 19-9 level decreased from 677.2 U/mL to 38.1 U/mL. After 2months of S-1 administration, we performed subtotal stomach-preserving pancreaticoduodenectomy with D2 lymph node dissection. Histopathological examination revealed that most of the pancreatic cancer was replaced by fibrosis and only a few atypical epithelial cells existed. No cancer cells were found at the surgical margin. The patient remains alive without any signs of recurrence 59 months after the systemic chemotherapy and 51 months after the surgical resection.

Polyarthritis and pancreatic panniculitis associated with pancreatic carcinoma: review of the literature.

Pancreatic disorders, such as chronic or acute pancreatitis, and carcinoma may be infrequently accompanied or preceded by panniculitis or polyarthritis. This triad is known in the literature as the pancreatitis, panniculitis, and polyarthritis syndrome. Although the pancreatic disease of pancreatitis, panniculitis, and polyarthritis syndrome usually includes pancreatitis, here we review the literature with report of 1 additional case of polyarthritis and panniculitis occurring in the presence of pancreatic carcinoma. Given that the diagnosis is often difficult when abdominal symptoms are absent, knowledge of the association between panniculitis and polyarthritis with pancreatic disease may lead to a prompt diagnosis and management. The histopathology of the skin lesions can be a valuable clue for focusing attention to a pancreatic disease.

DNA mismatch repair abnormalities in acinar cell carcinoma of the pancreas: frequency and clinical significance.

OBJECTIVES: Acinar cell carcinoma (ACC), including its mixed variants, is a rare pancreatic malignancy. Recent reports have documented its occurrence in Lynch syndrome. Our aim was to evaluate the frequency and clinicopathologic significance of DNA mismatch repair (MMR) deficiency in ACCs in general. METHODS: Mismatch repair protein expression was evaluated by immunohistochemistry in a series of 36 ACC cases that were treated at our institution and had sufficient clinical information and pathologic material. RESULTS: Loss of MMR protein was observed in 5 ACCs (5/36, 14%): 2 lost MLH1/PMS2, 2 lost MSH2/MSH6, and 1 lost MSH6 alone. The 1 MSH6-deficient case and 1 of the 2 MSH2/MSH6-deficient cases had a known history of Lynch syndrome, carrying a germline mutation in MSH6 and MSH2, respectively. None of the 5 tumors showed distinctive morphology. Two of the 5 patients died of disease 6 and 21 months after diagnosis. In contrast, in the MMR-normal group, only 1 of 30 patients died of disease (median follow-up, 32.5 months). CONCLUSIONS: Mismatch repair protein deficiency is not uncommon in ACCs, occurring in 14% of the cases in this series. The MMR-deficient ACCs did not show distinctive morphologic features and were clinically no less aggressive than MMR-normal ACCs.

Excellent prognosis of lepidic-predominant lung adenocarcinoma: low incidence of lymphatic vessel invasion as a key factor.

AIM: To clarify the prognostic value of predominant histological subtypes for small-sized lung adenocarcinoma. MATERIALS AND METHODS: Sixty-four specimens of resected invasive lung adenocarcinoma less than 20 mm in diameter with no lymph node metastasis were studied. These specimens were microscopically classified into predominant histological subtypes (21 lepidic, 16 acinar, 24 papillary, and three solid) according to the International association for the study of lung cancer/American thoracic society/European respiratory society adenocarcinoma classification. The relationships between tumor relapse and predominant histological subtypes were statistically analyzed. In addition the relationships between several pathological factors and predominant histological subtypes were statistically assessed. RESULTS: Kaplan-Meier relapse-free curves showed a five-year relapse-free rate of 100% in 64 patients with lepidic-predominant adenocarcinoma, compared with a rate of 73.7% (p=0.035 by log rank test) in patients with non-lepidic-predominant adenocarcinoma (papillary, acinar, and solid). The only statistically significant pathological factor between lepidic-predominant and non-lepidic-predominant histological subtypes was lymphatic vessel invasion as assessed by logistic regression analysis. CONCLUSION: In small-sized lung adenocarcinoma, lepidic-predominant histological subtype is the best prognostic factor, and a low incidence of lymphatic vessel invasion in the histological subtype is a key factor for an excellent prognosis.

A rare case of iliac crest metastasis from acinic cell carcinoma of parotid gland.

A case of acinic cell carcinoma of the right parotid gland metastasizing to the right iliac crest is presented. Generally, for this rare low-grade malignant salivary gland neoplasm, 20% of cases may have local recurrences whereas about 10% of cases have distant metastases. They may arise many years after the initial presentation of the original tumor. The most frequent locations are the cervical lymph nodes, liver, lungs, contralateral orbit and bones. Occurrence in the appendicular skeleton is very rare and in our knowledge this is the first report of metastases to the pelvis.

"Chronic" metastatic pancreatic acinar cell carcinoma.

Acinar cell carcinoma (ACC) of the pancreas is a rare exocrine tumour for which there is very limited information about chemotherapy regimens and prognosis. Even though there are clinical guidelines for management of ductal cell carcinoma, a definitive and specific regime has not yet been agreed for this type of pancreatic cancer. We report a case of metastatic ACC of pancreas who has been treated with a multimodal approach, including novel combinations of different targeted drugs with conventional chemotherapy, surgery and radiofrequency ablation since the last 11 years. This degree of long term survival has not been reported so far in such a case of metastatic ACC of the pancreas. This case highlights the importance of a personalised multidisciplinary therapeutic strategy, employing locoregional therapies along with combinations of established and novel systemic therapies to control the disease, and the importance of flexibility when instigating new treatment paradigms for progressive cancer. Also, this case demonstrates that complete tumour eradication may not be the sole purpose of surgical oncology.

A case report of a patient with advanced acinar cell carcinoma of the pancreas: long-term survival with regional, systemic and targeted therapy.

Acinar cell carcinoma of the pancreas is an uncommon malignancy for which there are no prospective, randomized data to guide therapy. We describe the clinical course and management of a patient with advanced pancreatic acinar cell carcinoma who is alive seven years after diagnosis using systemic and regional chemotherapies as well as molecularly targeted agents.

Prognostic value of CA 19-9, CEA, CRP, LDH and bilirubin levels in locally advanced and metastatic pancreatic cancer: results from a multicenter, pooled analysis of patients receiving palliative chemotherapy.

PURPOSE: CA 19-9 is the only established tumor marker in pancreatic cancer (PC); the prognostic role of other serum markers like CEA, CRP, LDH or bilirubin has not yet been defined. METHODS: We pooled pre-treatment data on CA 19-9, CEA, CRP, LDH and bilirubin levels from two German multicenter randomized phase II trials together with prospective patient data from one high-volume German Cancer Center. Marker levels were assessed locally before the start of palliative first-line therapy for advanced PC and serially during treatment (for CA 19-9 only). Clinical and biomarker data (overall 12 variables) were correlated with the efficacy endpoints time-to-progression (TTP) and overall survival (OS) by using uni- and multivariate Cox models. RESULTS: Data from 291 patients were included in this pooled analysis; 253 patients (87 %) received treatment within prospective clinical trials. Median TTP in the study cohort was 5.1 months and median OS 9.0 months. In univariate analysis, pre-treatment CA 19-9 (HR 1.55), LDH (HR 2.04) and CEA (HR 1.89) levels were significantly associated with TTP. Regarding OS, baseline CA 19-9 (HR 1.46), LDH (HR 2.07), CRP (HR 1.69) and bilirubin (HR 1.62) were significant prognostic factors. Within multivariate analyses, pre-treatment log [CA 19-9] (as continuous variable for TTP) and log [bilirubin] as well as log [CRP] (for OS) had an independent prognostic value. A CA 19-9 decline of ≥25 % during the first two chemotherapy cycles was predictive for TTP and OS, independent of the applied CA 19-9 assay. CONCLUSION: Baseline CA 19-9 and CA 19-9 kinetics during first-line chemotherapy are prognostic in advanced PC. Besides that finding other serum markers like CRP, LDH and bilirubin can also provide prognostic information on TTP and OS.