|Year : 2020 | Volume
| Issue : 3 | Page : 145-148
Primary squamous cell carcinoma of head of the pancreas
Supratim Bhattacharyya, Padmalaya Devi, Subrat Samantara, K P Kunhi Mohammed
Department of Surgical Oncology, Acharya Harihar Postgraduate Institute of Cancer, Cuttack, Odisha, India
|Date of Submission||29-Aug-2020|
|Date of Acceptance||28-Sep-2020|
|Date of Web Publication||26-Nov-2020|
K P Kunhi Mohammed
Department of Surgical Oncology, Acharya Harihar Postgraduate Institute of Cancer, Cuttack - 753 007, Odisha
Source of Support: None, Conflict of Interest: None
Primary pancreatic malignancy with pure squamous cell histology is extremely rare and has aggressive behavior with poor prognosis. The management of primary squamous cell carcinoma of the pancreas (SCCP) is poorly defined due to the paucity of data. Preoperative histological diagnosis is difficult in the majority of cases. Curative resection is the main stay of treatment, but most of the cases were found inoperable due to delayed diagnosis and dissemination of the disease at the time of initial diagnosis. Herein, we report a case of primary SCCP in a 44-year-old female located at the head of the pancreas. Her contrast-enhanced computed tomography (CECT) scan image had the findings of pancreatic head mass along with the features of atrophic pancreatitis. Whipple's procedure was performed successfully for the case with the postoperative pathological staging of PT2N0CM0 and chronic pancreatitis. However, the patient died due to sepsis on the 32nd postoperative day. The presence of atrophic pancreatitis on CECT scan and postoperative pathological findings of chronic pancreatitis suggested the development of squamous metaplasia followed by subsequent development of SCCP.
Keywords: Pancreas, primary squamous cell carcinoma, Whipple's procedure
|How to cite this article:|
Bhattacharyya S, Devi P, Samantara S, Mohammed K P. Primary squamous cell carcinoma of head of the pancreas. Oncol J India 2020;4:145-8
| Introduction|| |
Ductal carcinoma is the most common exocrine neoplasm of the pancreas with adenocarcinoma is being the most common histological subtype. However, primary squamous cell carcinoma of the pancreas (SCCP) is an exceedingly rare histological subtype of ductal carcinoma. It constitutes 0.5%–2% of all pancreatic malignancies. A 2016 surveillance, epidemiology and end results (SEER) database review from 2000 to 2012 shows 0.2% incidence of primary SCCP among all the pancreatic cancers. Squamous cells are not naturally seen within the pancreas, and hence, the pathogenesis of SCCP is uncertain. The most common differentials for SCCP include adenosquamous type and secondaries.,,, Here, we present a case of primary SCCP in a 44-year-old female located at the head of the pancreas.
| Case Report|| |
A 44-year-old nonsmoker female presented to the department of surgical oncology with the chief complaints of vague upper abdominal pain radiating to back and weight loss for 2 months. There was no past history of jaundice or vomiting. She was a known case of Type-2 diabetes mellitus on oral hypoglycemic agents for 6 years. Routine blood and serum biochemistry did not show any significant abnormality. Coagulation profiles were within the normal range. Triphasic multidimensional contrast-enhanced computed tomography (CECT) of the whole abdomen revealed a pancreatic head mass of size 25 mm × 17 mm with atrophic pancreatitis, dilatation of common bile duct (CBD), and main pancreatic duct without any abutment or encasement of superior mesenteric artery (SMA) or superior mesenteric vein, and there was no evidence of retroperitoneal adenopathy or any other distant metastasis [Figure 1]. Serum marker CA 19-9 level was 117 U/mL. Further metastatic workup with a CECT scan of the thorax ruled out any lung metastasis or pleural effusion. Following preoperative optimization, the patient was planned for surgical exploration with Whipple's procedure.
|Figure 1: Contrast-enhanced computed tomography scan of the abdomen showing pancreatic head mass with dilated common bile duct and main pancreatic duct with hugely distended gall bladder|
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On exploration, no gross extrapancreatic disease was noted. Interaortocaval lymph nodes were found to be enlarged. Intraoperative imprint cytology of the nodes was negative for malignancy. The pancreatic head mass was approximately 4 cm × 4 cm in dimension, encasing the main portal vein trunk for a length of 2 cm. The SMA appeared to be free. There were no peritoneal deposits. The liver appeared grossly normal. No ascites were noted. Portal venous margin was resected en masse with the tumor and lateral venorrhaphy done. Whipple's pancreaticoduodenectomy was completed with no gross residual disease left. Retrocolic end to side invaginating double-layered pancreaticojejunostomy was performed over infant feeding tube number 5. End-to-side hepaticojejunostomy and gastrojejunostomy were done.
Gross examination of the resected specimen revealed a reddish-brown tumor at the head of the pancreas [Figure 2]. On histopathological examination of the resected specimen, the pleomorphic tumor cells were arranged in sheets with the adjacent stroma having inflammatory cell infiltrate, and the tumor cells were having round-to-oval nuclei, prominent nucleoli with abundant cytoplasm suggesting the features of SCCP with well-differentiation grading [Figure 3] and [Figure 4]. The lesion was extending to the second part of the duodenum and distal CBD. On further histological examination, lymphovascular space invasion and perineural invasion was found to be positive. The depth of invasion of the tumor was up to the muscularis of the duodenal wall. Pancreatic resection margin, CBD resection margin, and SMA groove margin were free of malignancy. Adjacent pancreatic parenchyma showed the features of chronic pancreatitis. A total of nine lymph nodes were resected, and all were found to be reactive. With the above findings, the patient was staged as SCCP PT2PN0CM0.
|Figure 2: Gross image of Whipple's pancreaticoduodenectomy specimen showing a reddish-brown tumor at the head of the pancreas|
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|Figure 3: Histopathological examination revealing the tumor cells arranged in sheets with the adjacent stroma having inflammatory cell infiltrate (H and E, ×10)|
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|Figure 4: Histopathological examination revealing the pleomorphic tumor cells having round-to-oval nuclei, prominent nucleoli with abundant cytoplasm (H and E, ×20)|
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The patient was clinically improving in the postoperative period for the initial 1 week. She was started oral feeding on the 4th postoperative day and intravenous fluid support was taken off after a week of surgery. However, after the 10th postoperative day, the patient started developing high output transudative ascites with hemodynamic instability. The ascites were negative for pancreatic enzymes and chylomicron on three different occasions. She was started on supplemental intravenous fluid and nutrient support with enteral feeding. However, her glycemic control worsened, and she developed sepsis. Blood culture revealed multidrug resistant Klebsiella pneumonia sensitive to meropenem. Despite all broad-spectrum antibiotic coverage, inotropic and ventilatory support, the patient went into multi-organ dysfunction syndrome and finally died on the postoperative day 32.
| Discussion|| |
Primary SCCP is a rare entity with unknown etiology. Squamous cells are naturally not found in the pancreas. Squamous metaplasia is noted in the setting of chronic pancreatitis and due to the placement of pancreatic duct stents. However, the transformation of squamous metaplasia to SCCP is unlikely. Adenosquamous carcinoma is an important differential diagnosis for pure SCCP with a higher incidence of 0.5 cases per 100,000. But the genetic markers for ductal origin will be expressed in adenosquamous carcinoma., Moreover, most of the SCCP cases are metastatic in nature and have been reported from squamous esophageal and lung cancer.,
The histogenesis of SCCP is unknown. Several theories have been suggested such as: a primitive cell capable of differentiating into either squamous or glandular carcinoma undergoes malignant change; squamous change in a pre-existing adenocarcinoma; malignant transformation from a squamous metaplasia of the ductal epithelium or an aberrant squamous cell undergoes a malignant change.
A recent systematic review conducted by Ntanasis-Stathopoulos et al. found that majority of SCCP were located in the head of the pancreas (52.9%) followed by tail (21.6%) and body (5.9%). This systematic review reported the median age at the diagnosis of 63 years and majority of them are male. The clinical picture of SCCP is similar to that of adenocarcinoma. However, early nodal and distant metastasis has been reported in the majority of cases. A SEER database reported that up to 56% of SCCP were noted to be metastatic at the time of initial presentation and 61% being stage T3–T4. Our case was pathologically stage T2 disease.
There are no specific imaging studies for the diagnosis of SCCP. Endoscopic ultrasound-guided fine-needle aspiration and computed tomography-guided core needle biopsy has been used for the diagnosis in unresectable cases., The histopathological characteristic features of SCCP include keratinization with eosinophilic cytoplasm on hematoxylin and eosin staining, formation of the whorls or “pearls” with intercellular bridges, and irregularly shaped nests and cords of epithelial cells. Desmoplastic reaction secondary to ductal obstruction may be observed. The background of severe chronic pancreatitis, along with tumor focus may be seen like in our case, indicating the origin of metaplastic process. In our case, the presence of chronic atrophic pancreatitis on CECT scan and postoperative histopathological finding of chronic pancreatitis is the possible risk factor for the development of squamous metaplasia followed by SCCP which needs further evaluation.
There is no standard treatment approach for primary SCCP due to the rarity of cases. Surgical resection with microscopic negative margin is the best therapeutic approach for operable cases, but majority of cases are metastatic or locally advanced at presentation, resulting curative resection impossible., A study by Makarova-Rusher et al. reported median survival of 10 months for SCCP treated with curative surgery than that of 3 months for those SCCP treated in palliative intent. They also found median survival of 18 months for curative resection versus 5 months for palliative treatment among adenocarcinoma of the pancreas suggesting survival is poorer among SCCP than adenocarcinoma of the pancreas.
| Conclusion|| |
Primary SCCP is an extremely rare event which mandates a thorough search for the possibility of other more common primary sources such as the lungs and esophagus. The treatment of such entity is challenging and may only be improved by centralized national registries.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]