Cutaneous Metastasis of a Mucoepidermoid Carcinoma of the Pancreas: First Reported Case

Pandey, Prasamsa; Al‐Rohil, Rami N.; Goldstein, Jennifer; Tetzlaff, Michael T.; Barnes, Tamara G.; Baldwin, Sarah E.; Fogelman, David R.; Abraham, Susan C.; Piha-Paul, Sarina A.

doi:10.1097/dad.0000000000000604

Cited by 10 publications

(12 citation statements)

References 22 publications

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“…Curative surgery with R0 resection margin seems to be the basis to guarantee an improved survival outcome, as the failure to undergo radical operation always resulted in an inferior survival outcome as described in our current literature review. Postoperative multimodal treatment may be a prognostic factor to improve survival outcome, which was identified by Pandey et al [ 8 ] and re-confirmed by us in the present series. In that study, the patient received 6 months chemotherapy by using adjuvant gemcitabine and cisplatin first; then further FOLFIRINOX was also employed, and after that several chemotherapy or biotherapy were also used so as to prevent disease progression.…”

Section: Discussionsupporting

confidence: 87%

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Zhou²,

Liu³

et al. 2018

Medicine

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Rationale:Pancreatic mucoepidermoid carcinoma (MEC) is a rare disease with no more than 10 cases reported in literature. The prognosis is poor and few patients can survival more than 1 year.Patient concerns:We presented a case of patient manifested as left upper abdominal pain. Computed tomography demonstrated a low-density shadow measuring 2.1 × 2.4 cm situated at the transition area of neck and body of the pancreas with obscure boundary and irregular enhancement. The preoperative symptoms and imaging features were unspecific.Diagnoses:Pancreatic MEC.Interventions:Curative surgery of distal pancreatectomy was conducted. Postoperatively, the patient subsequently underwent 8 cycles of chemotherapy using cisplatin (25 mg/m2) on day 1 to day 3; and gemcitabine (1000 mg/m2) on day 1 and day 8, repeated every 21 days.Outcomes:The patient was monitored on a regular basis at our outpatient department and survived 23 months after surgery.Lessons:Preoperative diagnosis of pancreatic MEC is difficult. Patients with pancreatic MEC may have a survival benefit from the multimodal treatment of curative surgery combined with chemotherapy.

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Section: Discussionsupporting

confidence: 87%

“…[ 6 ] Since first reported by Franz in 1959, about 10 cases have been reported in literature. [ 4 , 6 – 8 ] We summarized all published series about pancreatic MEC which are presented in Table 1 . Taking together, a majority of patients tend to be male (70%), with ages between 40 and 70 years.…”

Section: Discussionmentioning

confidence: 99%

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Zhou²,

Liu³

et al. 2018

Medicine

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“…English papers published from 1959 to 2020 were searched using the PubMed database with the search terms "mucoepidermoid carcinoma", "MEC", "pancreas" and "pancreatic", and only eleven cases were found. 4,5,[15][16][17][18][19] Clinicopathologic characteristics of these patients, including age, sex, treatment and prognosis, were collected for a better understanding of pancreatic MEC (Table 2). To date, the gene mutation sites in pancreatic MEC are unknown.…”

Section: Discussionmentioning

confidence: 99%

“…Meanwhile, liver lesions appeared as hypointense on T1-weighted image and as hyperintense on T2weighted image with ring enhanced during the arterial dominant phase of contrast-enhanced MRI, and metastatic lesions could not be excluded (Figure 2A-C). Whole-body 18 F-fluorodeoxyglucose (FDG) positron emission tomography (PET)/CT examination revealed a moderate FDG uptake nodule with the size of 4×4.5 cm in front of the pancreatic tail (SUVmax 6.2) and mild FDG uptake nodules in the liver (SUVmax 2.1 and 3.05) with normal scans of the head, neck, chest, and colon (Figure 2D). Subsequently, liver metastases were biopsied with ultrasonography navigation and were interpreted by histopathology as suspected mucinous tumors.…”

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confidence: 99%

Targeted-Gene Sequencing and Bioinformatics Analysis of Patients with Pancreatic Mucoepidermoid Carcinoma: A Case Report and Literature Review

Chen

Zhang

Huang

et al. 2021

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Background Primary pancreatic mucoepidermoid carcinoma (MEC) is an extremely rare malignant tumor with unclear etiology and pathogenesis. There are only eleven reported cases in English papers published from 1959 to 2020. MEC generally occurs in the salivary gland, but cases in the pancreas have also been reported. Although being considered as a low-grade indolent carcinoma, pancreatic MEC always invades the surrounding lymph node and metastasizes. The prognosis of pancreatic MEC is unsatisfactory. To date, the genetic alterations, mechanistic relationships among mutated genes and signaling pathways of pancreatic MEC are unclear. Patient and Methods This paper presents a case of rare primary pancreatic MEC in a 56-year-old male patient with liver metastasis. Radical surgery of distal pancreatectomy and radiofrequency ablation (RFA) of two liver metastatic lesions is conducted. Targeted-gene sequencing and bioinformatics analysis tools, including STRING, DAVID, cBioPortal, DGidb and Human Protein Atlas database (HPA), are used to clarify the biological functions and features of mutated genes in pancreatic MEC. Results Eight gene mutations (TP53, KRAS, ATR, FLI1, FLT4, MAGI2, RBM10, and TNFAIP3) were observed, and a tumor mutation burden (TMB) of 5.6 muts/Mb was calculated in the pancreatic MEC using targeted-gene sequencing. The patient subsequently underwent adjuvant chemotherapy and died three months after surgery. Gene–gene interaction network was constructed, which showed the significant interactions among eight mutated genes. In terms of the functions and pathways of eight gene mutations based on GO and KEGG, 20 tumor-related results are presented in this paper, Furthermore, the biological functions and features of pancreatic MEC are further compared with pancreatic ductal adenocarcinoma. Conclusion Pancreatic MEC requires early and effective treatment, and lymph node metastases and multiple organ metastases were unfavorable prognostic factors. Pancreatic MEC can be compared with other pancreatic cancers that have characteristic clinical phenotype, molecular alterations, functional information and enrichment pathway.

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“…[ 1 ] It is most commonly seen in salivary glands; however, mucoepidermoid carcinoma of the pancreas is an extremely rare entity which has been reported on only four cases in English literature. [ 2 3 4 5 ] Because of the rarity of mucoepidermoid carcinoma of the pancreas, the imaging features of the tumor have not been revealed, and all previous reports have not illustrated endoscopic ultrasound (EUS) findings.…”

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confidence: 99%

Contrast-enhanced endoscopic ultrasonography features of a mucoepidermoid carcinoma of the pancreas

2018

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Cutaneous Metastasis of a Mucoepidermoid Carcinoma of the Pancreas: First Reported Case

Abstract: Mucoepidermoid pancreatic cancer is an exceedingly rare subtype of pancreatic cancer, with very little information regarding its diagnosis, treatment, and patterns of metastases. Here, the authors present the first reported case of cutaneous metastases of mucoepidermoid pancreatic cancer.

Cited by 10 publications

References 22 publications

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Targeted-Gene Sequencing and Bioinformatics Analysis of Patients with Pancreatic Mucoepidermoid Carcinoma: A Case Report and Literature Review

Contrast-enhanced endoscopic ultrasonography features of a mucoepidermoid carcinoma of the pancreas

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