Long-term survival after resection of sarcomatoid carcinoma of the pancreas: an updated experience

Abstract

Background Sarcomatoid carcinoma of the pancreas (SCP) is a rare histologic subtype of undifferentiated pancreatic carcinoma. Historically, this has been associated with a worse overall prognosis than adenocarcinoma. However, the clinical course and surgical outcomes of SCP remain poorly characterized owing to its rarity. Methods A single-institution, prospectively maintained database was queried for patients who underwent pancreatic resection with a final diagnosis of SCP. We describe their histology, clinicopathologic features, and perioperative outcomes. Survival data are highlighted, and common traits of long-term survivors are examined. Results Over a 25-year period, 7009 patents underwent pancreatic resection at our institution. Eight (0.11%) were diagnosed with SCP on final histopathology. R0 resection was achieved in six patients (75%). Four patients had early recurrence leading to death (<3 months). Two (25%) experienced long-term survival (>5 years), with the longest surviving nearly 16 years despite the presence of lymph node metastasis. There were no deaths attributed to perioperative complications. Both long-term survivors had disease in the body/tail of the pancreas and received adjuvant radiotherapy. One also received adjuvant gemcitabine-based chemotherapy. Conclusions SCP is a rarely appreciated subset of pancreatic malignancy that does not necessarily portend to a uniformly dismal prognosis. Although some have rapid recurrence and an early demise, long-term survival may be possible. Future studies are needed to better define the cohort with potential for long-term survival so that aggressive therapies may be tailored appropriately in this patient subset.

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

“…UCP is a subtype of PDAC representing a set of rare tumors that accounts for as many as 5% of all pancreatic malignancies[ 11 ]. 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 ]…”

“…Owing to the rarity of the disease, the clinical course, surgical outcomes, and optimal treatment strategies for SCP are poorly characterized[ 5 ].…”

“…Although treatment for PDAC remains challenging, complete R0 surgical extirpation is the only chance of cure[ 5 ]. Although SCP shares similar molecular carcinogenesis with PDAC, its prognosis is much worse[ 6 ].…”

“…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.…”

“…Sarcomatoid carcinoma of the pancreas (SCP) is among these variants. SCP is an extremely uncommon tumor that accounts for 0.1% to 5.7% of all pancreatic malignancies[ 5 ]. It is an undifferentiated carcinoma that shares similar molecular pathogenesis with PDAC and therefore a similarly poor prognosis[ 2 ].…”