J Gastrointest Canc (2014) 45 (Suppl 1):S88–S90 DOI 10.1007/s12029-013-9560-0
CASE REPORT
An Interesting Clinical Entity of Squamous Cell Cancer of the Pancreas with Liver and Bone Metastases: a Case Report and Review of the Literature David Thomas & Nihar Shah & Hamid Shaaban & Michael Maroules
Published online: 16 November 2013 # Springer Science+Business Media New York 2013
Introduction Squamous cell pancreatic carcinoma is an exceedingly rare malignancy. The prevalence is noted at less than 1 % of the overall recorded cases of pancreatic malignancy with very few cases represented in the literature [1–3]. With such few cases, there is some debate over the exact etiology and pathogenesis of this type of cancer. The presentation and many of the clinical findings are shared with other histologic types including the much more common ductal adenocarcinoma, while others still break away from expectations. Here, we present a case of squamous cell carcinoma of the pancreas with liver and skeletal metastases as well as a discussion with a brief review of the literature.
Case Report A 60-year-old African-American male with a longstanding history of alcohol abuse was referred to our medical center with complaints of a few weeks history of dull epigastric pain with radiation to the back and weight loss of 30 lb. Initial physical
examination revealed only minimal tenderness over the epigastrium, and blood tests were all normal including serum lipase (29 IU/L, reference 10–50 IU/L) and CA 19-9 (15 IU/mL, reference 0–37 IU/mL). Computed tomography (CT) of the thorax and abdomen revealed a 9-cm partially cystic mass in the tail of the pancreas and multiple subtle hypodense nonenhanced lesions in the liver. CT-guided biopsy of the liver lesions was performed, and preliminary histopathology revealed squamous cell carcinoma of pancreatobiliary origin. Endoscopic ultrasound (EUS) was subsequently performed which revealed a complex cystic mass with a large solid component in the tail of the pancreas measuring up to 93 mm. EUS-guided fine-needle aspiration (FNA) of both the liver and tail of pancreas lesions with extensive sampling was carried out, which revealed squamous cell carcinoma in both sites (Fig. 1). Bone scan revealed left humeral metastases, and the patient was administered intravenously with bisphosphonates. During hospitalization, the patient continued to deteriorate and was subsequently discharged to home hospice.
Discussion D. Thomas (*) Department of Medicine, St. Joseph’s Regional Medical Center, 703 Main St, Paterson, NJ 07503, USA e-mail:
[email protected] N. Shah Department of Gastroenterology, St. Joseph’s Regional Medical Center, Paterson, NJ, USA H. Shaaban Department of Hematology and Oncology, St Michael’s Medical Center, Newark, NJ, USA M. Maroules Department of Hematology and Oncology, St. Joseph’s Regional Medical Center, Paterson, NJ, USA
Pure squamous cell carcinoma of the pancreas is extremely rare. With the earliest reports in the English literature surfacing around the 1940s and yet very few cases seen in the past 70 years, it is considered one of the rarest pancreatic neoplasms with less than 1 % prevalence in all cases of pancreatic cancer [3–5]. Most commonly, pancreatic cancer manifests as a ductal adenocarcinoma which arises from the exocrine ductal cells in more than 75 % of cases [4]. The pancreas is devoid of squamous cells in its natural nondiseased state [2, 8]. As there have been very few reports in the literature, it is difficult to be certain of the mechanism of pathogenesis in this malignancy. The most popular theory appears to suggest that the
J Gastrointest Canc (2014) 45 (Suppl 1):S88–S90
Fig. 1 Pathologic findings revealing well-differentiated squamous cell carcinoma with evidence of cytoplasmic keratin pearls and intercellular bridges. (H&E, original magnification ×300)
tumors arise from squamous metaplasia of the exocrine ductal epithelium which is, like most metaplasia, caused by an ongoing inflammatory insult such as chronic pancreatitis [4, 5]. One alternative theory proposed that the origin could be a variant of an adenosquamous carcinoma in which the glandular component has disappeared [1]. This histologic type is frequently seen in surgical specimens and makes up roughly 25 % of pancreatic adenocarcinomas in the literature [4]. The extensive biopsy samples that we attained revealed no evidence of an adenocarcinoma component. The preferred theory for our patient is that his longstanding history of alcohol likely resulted in chronic pancreatitis-related squamous metaplasia which progressively resulted in pure squamous cell carcinoma. The cases that have been reported in the medical literature have shown no clinical correlation with a history of smoking, drinking, or pancreatitis [1–7]. In nearly all reported cases of squamous cell pancreatic cancer, a vague complaint of epigastric pain is the presenting symptom [3–8], making it very hard to diagnose at an early stage. The age range for this disease in the reviewed cases is roughly 40–70 years of age. One case involved a 33-year-old female patient who did not have any identifiable risk factors such as smoking or heavy alcohol consumption [8]. Weight loss is another important but nonspecific finding of pancreatic squamous cell cancer. In our own case, the patient presented with a 30-lb weight loss. This finding is likely due to compression of the duodenum and stomach by the masses resulting in delayed gastric emptying and an induced feeling of early satiety. This same process can result in other gastrointestinal symptoms such as nausea and vomiting. As was previously stated in our case, the complaint was only mild epigastric pain, radiating to the back, in addition to weight loss. Laboratory findings are often nonspecific. However, an elevated bilirubin due to obstructive jaundice is common particularly in cases involving the head of the pancreas. An elevated fecal elastase level is a well-known finding in cases of chronic pancreatitis, a possible precursor for this rare malignancy. Elevated tumor markers like CA 19-9 and CEA have
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been used to aid in the diagnosis, but, also more importantly, to follow the disease but they are nonspecific. On imaging, the mass tends to appear as an opacified mass on CT [6]. The mass itself is described as a fluid-filled cavity consisting of necrotic keratinous sediment [4, 8]. Another finding that is associated with this particular type of tumor is an angiographic hypervascularity or “blush” in the region of the tumor [2, 6]. However, this is not 100 % specific. In our own case, the CT revealed a partially cystic mass. Thus, these various methods of investigation should be taken together, and the clinical suspicion and laboratory evidence correlated to increase suspicion of malignancy. Diagnosis of this malignancy is similar to other types of pancreatic malignancy. In suspected cases, EUS-FNA should be performed for cytopathological confirmation. This technique has become the standard since the 1990s for diagnosis as it limits the need for unnecessary resection [4]. As mentioned, transgastric EUS-FNA was utilized in our case to attain a more accurate assessment of the pancreatic pathology and assessment of the liver lesions. Other methods described in the literature to aid in diagnosis include ERCP as well [6]. However, if excisable, the mass should be sent for pathologic section to obtain a final diagnosis as only one reported case in the literature was identified by EUS-FNA alone [4]. While only one other reported case described no associated lab abnormalities. There have been a few examples in the literature of atypical physical and laboratory manifestations of this disease. One case described a case with associated hypercalcemia. In our own case, there was no hypercalcemia despite bony metastasis in the left humerus on bone scan. The reviewed case in question was reported in 1982 and was the first case to the author’s knowledge with intractable hypercalcemia and normal PTH levels with normal glands at autopsy [3]. The case was fascinating because, unlike our case, there was no evidence of bony metastasis. Squamous cell cancers of the lung and head/neck can sometimes produce hypercalcemia, but this is often associated with some form of PTH or related peptide release stimulating the hypercalcemia. It should be noted that this finding is nonspecific, and hence, a search for this rare tumor is not warranted in a workup for hypercalcemia [3]. Treatment for this malignancy is difficult. Squamous cell carcinoma of the pancreas typically responds poorly to chemotherapy and radiation [5, 8]. One study, which included six patients with squamous cell pancreatic cancer, had reported no long-term survival with a range of 1–7 months of survival time [8]. The longest survivor in this study, who had multiple sites of metastases, underwent treatment with cisplatin and vinblastine. In one case of unresectable disease, the patient elected not to receive oncologic consultation and instead elected to undergo an unconventional immunotherapeutic regimen offered in California. At 9 months posttreatment,
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she presented with no symptoms and clinical improvement [8]. As the report does not entail any further information about this immunotherapy, no further conclusions can be drawn from this case.
Conflict of Interest The authors declare that they have no conflict of interest.
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