Sarcomatoid carcinoma of the pancreas with rare long-term survival: a case report

Abstract

Background Sarcomatoid carcinoma of the pancreas (SCP) tends to have similar or even worse prognosis than that of conventional pancreatic ductal adenocarcinoma. The clinical and pathological features of SCP remain poorly characterized owing to its rarity. Case presentation A 58-year-old man was admitted to our hospital with the chief complaints of upper abdominal pain and weight loss. Abdominal contrast computed tomography revealed a 5-cm low-density mass in the pancreatic body. Magnetic resonance cholangiopancreatography revealed an obstruction of the main pancreatic duct and a dilation of the distal main pancreatic duct. Based on the clinicoradiological findings, pancreatic body cancer was suspected, and the distal pancreatectomy was performed. A pathological examination revealed that the tumor was composed of an area of invasive ductal adenocarcinoma and an area of sarcomatous spindle-shaped cells; the latter component predominated. The spindle cells were immunohistochemically positive for both cytokeratin and vimentin, and thus, a pathological diagnosis of SCP was made. In addition, immunohistochemical analysis suggested the sarcomatous component might be derived from the adenocarcinoma component via the process of epithelial-mesenchymal transition. After the operation, the patient received 6 months of chemotherapy with gemcitabine. At 10 years after the operation, the patient is alive with no recurrence. Conclusions The current case study presented a SCP patient with long-term survival after the operation. It was worth noting that the sarcomatous component of the tumor pathologically showed lower MIB-1 labeling index compared with those in previously reported SCP cases, which might account for the long-term survival of the patient.

Sarcomatoid carcinoma of the pancreas — a rare tumor with an uncommon presentation and course: A case report and review of literature

“…Several terms have been used to describe this malignancy, including carcinosarcoma, pseudosarcoma, pseudocarcinoma, and spindle cell carcinoma[ 9 ]. The multiple names demonstrate the varied understanding of this disease, which these terms have been often used interchangeably, and their definitions vary among the reports[ 10 ]. According to the WHO classification (5 th edition, 2019) of pancreatic tumors assigns SCP under the category of undifferentiated carcinomas (UCP)[ 4 ].…”

“…UPC is categorized into two different types: undifferentiated carcinoma [with three variants: anaplastic undifferentiated carcinoma, sarcomatoid carcinoma (SCP), and carcinosarcoma] and undifferentiated carcinoma with osteoclast-like giant cells[ 5 , 12 ]. Hence, we present a case of SCP that is an extremely rare type of tumor, with only a few cases reported in the literature[ 5 , 9 , 10 , 13 - 21 , 31 - 36 ]…”

“…Given its aggressive biological behavior and poor prognosis, it is of prime importance to make early diagnoses for patients with SCP[ 22 ]. Although some patients have a rapid recurrence and early death, long-term survival has been reported[ 5 , 10 ]. Blair et al [ 5 ] reported 8 cases of SCP, of which two experienced long-term survival (> 5 years), with the longest surviving nearly 16 years despite the presence of lymph node metastasis representing the longest survival time of SPC patients in the literature.…”

“…One of them received adjuvant chemotherapy with gemcitabine who remained free of tumor recurrence and metastasis for 10 years but after this period the patient presented a colonic obstruction due to metastatic disease[ 18 ]. In the other case of SCP with a stage T3N1M0, after surgery the patient completed a 6-month course of adjuvant chemotherapy with gemcitabine and was then followed up with abdomen CT. At 10 years after the operation, the authors report he is alive with no recurrence[ 10 ].…”

Does cellular senescence play an important role in the prognosis of sarcomatoid carcinoma of the pancreas?

“…The survival benefit of surgery for SCP remains uncertain, and no therapeutic strategies for SCP have been established. We have already reported a rare case of SCP with long-term survival [ 3 ]. In that article, we concluded that the low proliferative activity of sarcomatous cells might have played a role in the prolonged survival of the patient.…”