Nonmucinous Cystadenomas of the Pancreas With Pancreatobiliary Phenotype and Ovarian-Like Stroma (original) (raw)
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Surgical Case Reports
Background: Diagnosis of cystic tumor of the pancreas is based on the World Health Organization criteria that classify pancreatic cystadenomas into four types: intra-ductal papillary mucinous neoplasms, mucinous cystic neoplasms (MCNs), serous cystic neoplasms, and solid pseudo-papillary neoplasms depending on their secretion and presence of ovarian-like stroma. Recently, Albores-Saavedra identified non-mucinous cystadenomas of the pancreas with pancreato-biliary phenotype and ovarian-like stroma. This precipitated examination of the proportions of these rare tumors in patients treated at Tokushima University Hospital. Case presentation: Case 1 was a 40-year-old woman with a cystic tumor in the tail of the pancreas. Computed tomography (CT) revealed a diffuse and non-enhanced cystic tumor in the tail of the pancreas. This tumor was diagnosed as a simple cyst at this point. However, 2 years later, the tumor had increased in size by 3 cm. Thus, laparoscopic distal pancreatectomy was performed. The content of the cyst was serous. The epithelial cells were lined with a single layer of cuboidal cells and the tumor had ovarian-like stroma pathologically. The final pathological diagnosis was non-mucinous cystadenoma of the pancreas with ovarian-like stroma. In Case 2, a cystic tumor in the pancreas was found by medical examination in a woman in her sixties who presented without symptoms. CT showed a 1.5-cm cystic tumor in the tail and body of the pancreas and a septum in the cyst. Nine years later, the tumor had grown to 2.4 cm in diameter and had a clear septum in the cyst. This tumor was diagnosed preoperatively as MCN. Thus, laparoscopic distal pancreatectomy was performed. The cyst contained serous fluid. Microscopic examination showed no ovarian-like stroma and the epithelial cells were lined by a single layer of cuboidal cells. The final pathological diagnosis was non-mucinous cystadenoma of the pancreas with ovarian-like stroma. Conclusions: Accurate preoperative diagnosis of this type of pancreatic cystic tumor may be difficult, although it occurs more often than expected. Non-mucinous cystadenomas of the pancreas with ovarian-like stroma need to be considered as a differential diagnosis.
The American Journal of Surgical Pathology, 1999
Mucinous cystic naopIasms (MCNs) of the p c r e a s are uncommon tumors. The classifntion and biologic potential of these neoplasms n m a h the subject of controversy. Attempts to classify t h~ tumors in a sirnilat manner to ovarian MCNs remains controversial, as even ~t o l o g i d y bmignappe.amg pmcrealic MCNs metastasize and art IethaI. One hundred cases of MCNs were identXed in the files of the trine Pathology Tumor R c w of the Armed P m s Institute of PathoIogy from the YCBF~ 1979 to 1993. The pathologic features, including hematoxylin and Basin s w n g , bistochemishy, i m m u 1 1~h e m i s k y (XHC), ocll cycle analysis, and K-ms oncogene detamhation were review&. These finwere correlated with the clinical follow-up obtained in all cases. There were 130 women, aged 2 M 5 years (mean age at the outset, 44.6 y w ) . Tbt m t s had vague abdominal pain, fullness, or abdominal masses. More than 95% of the tumors were in the m c tail or body and were p d o m h n t l y m u l t i l~. The tumors xanged in size from 1.5 to 36 cm in greatest dimension, with the average tumor measuring >I0 cm. A specttum of hbtomorphologic changes were present within the same case and from case to we. A single layer of blandappearing, sialomucin-producing columnar epithelium Iining the cyst wall wouId abruptly change to a complex papillary architecture, with and without cytologic atypia, and with and without s t c o d invasion. Ovarian-type smma was a c b teristic and requisite f e w . Focal sclerotic hyalhhtion of the siroma was noted. This ovarian-type smma reacted with *nth, smooth muscle actin, progesterone, w cstmgen re-From the -t of E d m h e and OtorJholaryn@c-Head & Neck Pathology &D.RT., C F . k , CS-H), the -t ofC?IlW Pathology, Mole& Division (RMP.). and the Deparment of Cellular Pathology, Division of Quantitative Pathology (R.C.B.), ArmGd Forces Mtute of Pathology, Washington, DC. Address ~~c e and reprint quests to Dr. Lester D.R Thompson, Dcpammt of Bndocrint and OtorhinohyngmBd & Nezk Pathology, BuMmg 54, Room GO661 1, hmd Forcss Institute of Pathology, 6825 16th Sh-cct NW, Washugmi, DC 2M066000, U.S.A. h n t e d at the 86th Annual Metting of tbe United Stares d Can a a n Academy of PaihoIogy, Orlando, FIoria March 1-8, 1997.
The American Journal of Surgical Pathology, 1999
Mucinous cystic naopIasms (MCNs) of the p c r e a s are uncommon tumors. The classifntion and biologic potential of these neoplasms n m a h the subject of controversy. Attempts to classify t h~ tumors in a sirnilat manner to ovarian MCNs remains controversial, as even ~t o l o g i d y bmignappe.amg pmcrealic MCNs metastasize and art IethaI. One hundred cases of MCNs were identXed in the files of the trine Pathology Tumor R c w of the Armed P m s Institute of PathoIogy from the YCBF~ 1979 to 1993. The pathologic features, including hematoxylin and Basin s w n g , bistochemishy, i m m u 1 1~h e m i s k y (XHC), ocll cycle analysis, and K-ms oncogene detamhation were review&. These finwere correlated with the clinical follow-up obtained in all cases. There were 130 women, aged 2 M 5 years (mean age at the outset, 44.6 y w ) . Tbt m t s had vague abdominal pain, fullness, or abdominal masses. More than 95% of the tumors were in the m c tail or body and were p d o m h n t l y m u l t i l~. The tumors xanged in size from 1.5 to 36 cm in greatest dimension, with the average tumor measuring >I0 cm. A specttum of hbtomorphologic changes were present within the same case and from case to we. A single layer of blandappearing, sialomucin-producing columnar epithelium Iining the cyst wall wouId abruptly change to a complex papillary architecture, with and without cytologic atypia, and with and without s t c o d invasion. Ovarian-type smma was a c b teristic and requisite f e w . Focal sclerotic hyalhhtion of the siroma was noted. This ovarian-type smma reacted with *nth, smooth muscle actin, progesterone, w cstmgen re-From the -t of E d m h e and OtorJholaryn@c-Head & Neck Pathology &D.RT., C F . k , CS-H), the -t ofC?IlW Pathology, Mole& Division (RMP.). and the Deparment of Cellular Pathology, Division of Quantitative Pathology (R.C.B.), ArmGd Forces Mtute of Pathology, Washington, DC. Address ~~c e and reprint quests to Dr. Lester D.R Thompson, Dcpammt of Bndocrint and OtorhinohyngmBd & Nezk Pathology, BuMmg 54, Room GO661 1, hmd Forcss Institute of Pathology, 6825 16th Sh-cct NW, Washugmi, DC 2M066000, U.S.A. h n t e d at the 86th Annual Metting of tbe United Stares d Can a a n Academy of PaihoIogy, Orlando, FIoria March 1-8, 1997.
Mucinous Cystic Neoplasm of the Pancreas is Not an Aggressive Entity
Annals of Surgery, 2008
Objective-Mucinous cystic neoplasms (MCNs) of the pancreas have often been confused with intraductal papillary mucinous neoplasms. We evaluated the clinicopathologic characteristics, prevalence of cancer, and prognosis of a large series of well-characterized MCNs in 2 tertiary centers. Methods-Analysis of 163 patients with resected MCNs, defined by the presence of ovarian stroma and lack of communication with the main pancreatic duct. Results-MCNs were seen mostly in women (95%) and in the distal pancreas (97%); 25% were incidentally discovered. Symptomatic patients typically had mild abdominal pain, but 9% presented with acute pancreatitis. One hundred eighteen patients (72%) had adenoma, 17 (10.5%) borderline tumors, 9 (5.5%) in situ carcinoma, and 19 (12%) invasive carcinoma. Patients with invasive carcinoma were significantly older than those with noninvasive neoplasms (55 vs. 44 years, P = 0.01). Findings associated with malignancy were presence of nodules (P = 0.0001) and diameter ≥60 mm (P = 0.0001). All neoplasms with cancer were either ≥40 mm in size or had nodules. There was no operative mortality and postoperative morbidity was 49%. Median followup was 57 months (range, 4-233); only patients with invasive carcinoma had recurrence. The 5year disease-specific survival for noninvasive MCNs was 100%, and for those with invasive cancer, 57%. Conclusions-This series, the largest with MCNs defined by ovarian stroma, shows a prevalence of cancer of only 17.5%. Patients with invasive carcinoma are older, suggesting progression from adenoma to carcinoma. Although resection should be considered for all cases, in low-risk MCNs (≤4 cm/no nodules), nonradical resections are appropriate. Mucinous cystic neoplasms (MCNs) usually are large, septated, thick-walled mucinous cysts that lack communication with the ductal system, and occur almost exclusively in the
Pancreatic mucinous cystic neoplasm of the main pancreatic duct
Archives of pathology & laboratory medicine, 2011
Mucinous cystic neoplasm and intraductal papillary mucinous neoplasm are 2 types of cystic pancreatic mucinous tumors, each with its own distinct clinicopathologic features and pathogenetic mechanisms. We report here an unusual pancreatic mucinous neoplasm with features of both a mucinous cystic neoplasm and an intraductal papillary mucinous neoplasm in a 40-year-old woman who underwent total pancreatectomy. The endoscopic retrograde cholangiopancreatogram and gross examination demonstrated a mucin-producing intraductal neoplasm involving the length of the main pancreatic duct, typical of main duct intraductal papillary mucinous neoplasm, but histology of the main duct showed involvement by a biphasic tumor composed of columnar epithelium overlying ovarian-type stroma, characteristic of a mucinous cystic neoplasm. Immunohistochemistry confirmed that the stromal cells expressed estrogen and progesterone receptors, inhibin, and calretinin. Pancreatic mucinous cystic neoplasm involving...
Cystadenomas and Cystadenocarcinomas of the Pancreas
Annals of Surgery, 1999
To review the features of patients with benign and malignant cystadenomas of the pancreas, focusing on preoperative diagnostic accuracy and long-term outcome, especially for nonoperated serous cystadenomas and resected cystadenocarcinomas. Summary Background Data Serous cystadenomas (SCAs) are benign tumors. Mucinous cystic neoplasms should be resected because of the risk of malignant progression. A correct preoperative diagnosis of tumor type is based on morphologic criteria. Despite the high quality of recent imaging procedures, the diagnosis frequently remains uncertain. Invasive investigations such as endosonography and diagnostic aspiration of cystic fluid may be helpful, but their assessment is limited to small series. The management of typical SCA may require resection or observation. Survival after pancreatic resection seems better for cystadenocarcinomas (MCACs) than for ductal adenocarcinomas of the pancreas.
International Journal of Surgery Case Reports, 2019
INTRODUCTION: Mucinous cystic neoplasms (MCN) are rare premalignant neoplasms of the pancreas that are typically found as single lesions in the pancreatic body and tail of women in the fifth and sixth decade of life, do not communicate with the pancreatic ductal system and are characterized by mucin-producing epithelium supported by ovarian-type stroma. PRESENTATION OF CASE: We present here a case of diffuse pancreatic involvement by MCN in a 64year-old woman with chronic pancreatitis. Pre-operative suspicion for MCN was low due to the multicentric nature of the lesions and imaging/biochemical fluid analysis demonstrating connection with the pancreatic ductal architecture. The patient underwent total pancreatectomy with pathology showing multiple cysts lined by flat epithelium with focal ovarian-type stroma, consistent with low-grade MCN. DISCUSSION: The presence of ovarian stroma on histological analysis is one of the defining characteristics of MCNs per WHO guidelines, and is mandatory for its diagnosis. Only one case of diffuse MCN has been previously described in the literature; however, in this case the authors were not able to reach a definitive histological diagnosis based on WHO criteria. CONCLUSION: To our knowledge, this is the first report of diffuse histology-proven MCN of the pancreas.
Clinical and Pathologic Correlation of 84 Mucinous Cystic Neoplasms of the Pancreas
Annals of Surgery, 2000
To determine whether the long-term behavior of cystic mucinous neoplasms of the pancreas could be predicted using a novel, precisely defined classification of benign mucinous cystadenomas, noninvasive proliferative cystic mucinous neoplasms, and invasive mucinous cystadenocarcinomas. The primary interest was to obtain long-term follow-up after complete resection to determine the recurrence rates based on this objective classification.
Mucinous cystic neoplasm of the pancreas: a case report
Chirurgia italiana
Cystic neoplasms of the pancreas account for only a small percentage of pancreatic tumours. They include mucinous cystic tumours, which have a higher incidence in females in their forties or fifties. Cystic neoplasms of the pancreas can present in a benign, borderline or malignant form. These tumours have a natural evolution from a benign (mucinous cystadenoma) to a malignant form (cystadenocarcinoma). It is not always easy to diagnose cystic tumours, including mucinous cystic tumours of the pancreas, and the final diagnosis is often reached only after the surgical procedure, which is the gold standard treatment of this disease. We present the case of a 56-year-old woman affected by a mucinous cystic tumour of the body-tail of the pancreas, who underwent distal splenopancreasectomy. She was discharged on postoperative day 12. After an 18-month followup, she is in good general condition and disease-free.