An indeterminate mucin-producing cystic neoplasm containing an undifferentiated carcinoma with osteoclast-like giant cells: a case report of a rare association of pancreatic tumors

Abstract

BackgroundOnly few case reports of mucinous cystic pancreatic neoplasm containing an undifferentiated carcinoma with osteoclast-like giant cells have been described in the literature. In the majority of cases this unusual association of tumors seems related to a favorable outcome. We present the second case of an indeterminate mucin-producting cystic neoplasm containing an area of carcinoma with osteoclast-like giant cells. The specific features of the two histotypes and the rapid course of the disease make our clinical case remarkable.Case presentationA 68 year old female came to our attention for a pancreatic macrocystic mass detected with ultrasonography. Her past medical history was silent. The patient reported upper abdominal discomfort for two months; nausea, vomiting or weight loss were not reported. Physical examination revealed a palpable mass in the epigastrium; scleral icterus was absent. Cross-sectional imaging showed a complex mass of the neck and body of the pancreas, characterized by multiple large cystic spaces separated by thick septa and an area of solid tissue located in the caudal portion of the lesion. The patient underwent total pancreatectomy with splenectomy. Pathological examination revealed a mucinous cystic neoplasm with a component of an undifferentiated carcinoma with osteoclast-like giant cells. Because of the absence of ovarian-type stroma, the lesion was classified as an indeterminate mucin-producing cystic neoplasm of the pancreas. The immunohistochemical studies evidenced no reactivity of osteclast-like giant cells to epithelial markers but showed a positive reactivity to histiocytic markers. Numerous pleomorphic giant cells with an immunohistochemical sarcomatoid profile were present in the undifferentiated carcinoma with osteoclast-like giant cells. A rapid tumor progression was observed: liver metastases were detected after 4 months. The patient received adjuvant chemotherapy (Gemcitabine) but expired 10 months after surgery.ConclusionOur case confirms that the presence of a solid area in a cystic pancreatic tumor at cross-sectional imaging should raise a suspicion of malignant transformation. The lack of ovarian-type stroma in a pancreatic mucinous cystic neoplasm and the presence of pleomorphic giant cells in an undifferentiated carcinoma with osteoclast-like giant cells could be a marker of a poor prognosis.

Undifferentiated carcinoma with osteoclast-like giant cells of pancreas

“…[11,12] Therefore, early radical resection is essential. Chemotherapy and radiation therapy may be effective in some advanced cases [13,14,16,20,23] ; however, experience and objective evidence are lacking throughout the literature.…”

Clinical Characteristics and Prognosis of Osteoclast-like Giant Cell Tumors of the Pancreas Compared with Pancreatic Adenocarcinomas: A Population-Based Study

“…Several studies [ 18 , 23 , 28 – 30 ] have reported OGTP to be associated lower rates of lymph node and distant metastasis relative to PA, and with better prognosis as a result. However, other reports have suggested the opposite, identifying poorer outcomes among OGTP patients relative to PA patients [ 21 , 22 ]. Given the limited number of OGTP cases reported to date, the prognosis of this condition thus remains unclear.…”

“…For example, some researchers have proposed that OGTP does not exhibit any sex-bias with respect to its development and that it has a relatively good prognosis as compared to other pancreatic tumors owing to the fact that OGTP typically only exhibits localized spreading, with metastasis to lymph nodes and distant sites being slow to develop and rarely reported [ 19 ]. In contrast, however, some researchers have reported OGTP to be more common in females [ 20 ] and to have a worse prognosis [ 21 , 22 ]. The site preferences for OGTP tumors similarly remains controversial.…”

Wirsung atraumatic rupture in patient with pancreatic pseudocysts: a case presentation

“…MCNs are thick-walled macrocystic tumors without ductal system communication [ 16 – 18 ]. Large cystic lesions detected on CT scan were coherent with MCN but MRCP findings excluded this diagnosis.…”