553665
research-article2014
IJSXXX10.1177/1066896914553665International Journal of Surgical PathologyAnila et al
Images in Pathology
Mucinous Cystic Neoplasm of Pancreas With Intermediate-Grade Dysplasia
International Journal of Surgical Pathology 2014, Vol. 22(8) 707–708 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1066896914553665 ijs.sagepub.com
K. R. Anila, DNB1, Arun Peter Mathew, DNB1, Anitha Mathews, MD1, and K. Jayasree, MD1 A 53-year-old woman presented with swelling abdomen of 2-month duration. She was a known diabetic on insulin and had hypothyroidism for which she was on medication (tab eltroxin). Computed tomography showed well-defined cystic lesion of size 17 × 15 × 8.8 cm arising from body and tail of pancreas. There were multiple enhancing septae and a small enhancing solid area in the wall. There was no infiltration to adjacent organs or bowel loop. The lesion was abutting the surface of stomach posteriorly. With a radiological diagnosis of mucinous cystadenoma, patient was taken up for surgery. The serum carcinoembryonic antigen was 7.3 ng/mL and CA 19-9 was 171 U/mL. Radical distal pancreaticosplenectomy was done. We received distal pancreaticosplenectomy specimen with a cystic mass. The cut surface showed multilocular cyst with the inner surface in areas having a trabeculated appearance (Figure 1). Microscopy showed a cystic neoplasm composed of columnar, mucin-producing epithelium, supported by ovarian-type stroma (Figure 2A). In areas there were papillary projections, crypt-like invaginations, cellular pseudo stratification, and occasional mitoses (Figure 2B). A diagnosis of mucinous cystic neoplasm of pancreas with intermediate dysplasia was made. Mucinous cystic neoplasms (MCNs) are defined as cystic epithelial neoplasms of pancreas occurring almost exclusively in women, showing no communication with the pancreatic ductal system and composed of columnar, mucin-producing epithelium, supported by ovarian-type stroma.1 MCNs can present with an associated invasive carcinoma. According to the grade of intraepithelial dysplasia, noninvasive tumors may be classified as MCN with low-, intermediate-, and high-grade dysplasia. The mean age at diagnosis is 49 years. MCNs typically present as a round mass with a smooth surface and a fibrous pseudo capsule with variable thickness and frequent calcifications. The cut surfaces demonstrate a unilocular or multilocular tumor with cystic spaces ranging from a few millimeters to several centimeters in diameter, containing either thick mucin or a mixture of mucin and hemorrhagic necrotic material. MCNs show two distinct components: an inner epithelial layer and an outer densely cellular ovarian-type stromal layer. The ovarian-type stroma is an entity defining feature of MCN and has become a requirement
Figure 1. Cut section showing multiloculated cystic neoplasm, with the inner surface in areas showing trabeculated appearance.
for diagnosing this entity.2 Ovarian-type stroma has been proposed as a requisite to distinguish MCN from intraductal papillary mucinous neoplasm. One view is that the stromal component of MCNs may be derived from the ovarian primordium. This view is supported by the stromal morphology, tendency to undergo luteinization, presence of hilar-like cells, and immunophenotypic sex cord– stromal differentiation. It has been hypothesized that ectopic ovarian stroma incorporated during embryogenesis in the pancreas, along the biliary tree or in the retro-peritoneum may release hormones and growth factors causing nearby epithelium to proliferate and form cystic tumors.3,4 The prognosis of noninvasive MCN, regardless of the degree of cellular atypia, is excellent if the tumor is completely removed. Our patient is on regular follow-up and is doing well after a period of 1 year from the initial diagnosis. 1
Regional Cancer Centre, Thiruvananthapuram, India
Corresponding Author: K. R. Anila, Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India. Email: [email protected]
708
International Journal of Surgical Pathology 22(8)
Figure 2. (A) Microscopy showed a cystic neoplasm composed of columnar, mucin-producing epithelium, supported by ovariantype stroma (hematoxylin and eosin, 100×). (B) Section from neoplasm showing papillary projections, crypt-like invaginations, cellular pseudo stratification, and occasional mitoses (hematoxylin and eosin, 100×).
References 1. Hamilton SR, Aaltonen LA, eds. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2006. 2. Tanaka M, Chari S, Adsay V, et al. International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology. 2006;6:17-32.
3. Erdogan D, Lamers WH, Offerhaus GJ, Busch OR, Gouma DJ, van Gulik TM. Cystadenomas with ovarian stroma in liver and pancreas: an evolving concept. Dig Surg. 2006;23: 186-191. 4. Izumo A, Yamaguchi K, Eguchi T, et al. Mucinous cystic tumor of the pancreas: immunohistochemical assessment of “ovarian-type stroma”. Oncol Rep. 2003;10:515-525.
Copyright of International Journal of Surgical Pathology is the property of Sage Publications Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
research-article2014
IJSXXX10.1177/1066896914553665International Journal of Surgical PathologyAnila et al
Images in Pathology
Mucinous Cystic Neoplasm of Pancreas With Intermediate-Grade Dysplasia
International Journal of Surgical Pathology 2014, Vol. 22(8) 707–708 © The Author(s) 2014 Reprints and permissions: sagepub.com/journalsPermissions.nav DOI: 10.1177/1066896914553665 ijs.sagepub.com
K. R. Anila, DNB1, Arun Peter Mathew, DNB1, Anitha Mathews, MD1, and K. Jayasree, MD1 A 53-year-old woman presented with swelling abdomen of 2-month duration. She was a known diabetic on insulin and had hypothyroidism for which she was on medication (tab eltroxin). Computed tomography showed well-defined cystic lesion of size 17 × 15 × 8.8 cm arising from body and tail of pancreas. There were multiple enhancing septae and a small enhancing solid area in the wall. There was no infiltration to adjacent organs or bowel loop. The lesion was abutting the surface of stomach posteriorly. With a radiological diagnosis of mucinous cystadenoma, patient was taken up for surgery. The serum carcinoembryonic antigen was 7.3 ng/mL and CA 19-9 was 171 U/mL. Radical distal pancreaticosplenectomy was done. We received distal pancreaticosplenectomy specimen with a cystic mass. The cut surface showed multilocular cyst with the inner surface in areas having a trabeculated appearance (Figure 1). Microscopy showed a cystic neoplasm composed of columnar, mucin-producing epithelium, supported by ovarian-type stroma (Figure 2A). In areas there were papillary projections, crypt-like invaginations, cellular pseudo stratification, and occasional mitoses (Figure 2B). A diagnosis of mucinous cystic neoplasm of pancreas with intermediate dysplasia was made. Mucinous cystic neoplasms (MCNs) are defined as cystic epithelial neoplasms of pancreas occurring almost exclusively in women, showing no communication with the pancreatic ductal system and composed of columnar, mucin-producing epithelium, supported by ovarian-type stroma.1 MCNs can present with an associated invasive carcinoma. According to the grade of intraepithelial dysplasia, noninvasive tumors may be classified as MCN with low-, intermediate-, and high-grade dysplasia. The mean age at diagnosis is 49 years. MCNs typically present as a round mass with a smooth surface and a fibrous pseudo capsule with variable thickness and frequent calcifications. The cut surfaces demonstrate a unilocular or multilocular tumor with cystic spaces ranging from a few millimeters to several centimeters in diameter, containing either thick mucin or a mixture of mucin and hemorrhagic necrotic material. MCNs show two distinct components: an inner epithelial layer and an outer densely cellular ovarian-type stromal layer. The ovarian-type stroma is an entity defining feature of MCN and has become a requirement
Figure 1. Cut section showing multiloculated cystic neoplasm, with the inner surface in areas showing trabeculated appearance.
for diagnosing this entity.2 Ovarian-type stroma has been proposed as a requisite to distinguish MCN from intraductal papillary mucinous neoplasm. One view is that the stromal component of MCNs may be derived from the ovarian primordium. This view is supported by the stromal morphology, tendency to undergo luteinization, presence of hilar-like cells, and immunophenotypic sex cord– stromal differentiation. It has been hypothesized that ectopic ovarian stroma incorporated during embryogenesis in the pancreas, along the biliary tree or in the retro-peritoneum may release hormones and growth factors causing nearby epithelium to proliferate and form cystic tumors.3,4 The prognosis of noninvasive MCN, regardless of the degree of cellular atypia, is excellent if the tumor is completely removed. Our patient is on regular follow-up and is doing well after a period of 1 year from the initial diagnosis. 1
Regional Cancer Centre, Thiruvananthapuram, India
Corresponding Author: K. R. Anila, Department of Pathology, Regional Cancer Centre, Thiruvananthapuram, Kerala 695011, India. Email: [email protected]
708
International Journal of Surgical Pathology 22(8)
Figure 2. (A) Microscopy showed a cystic neoplasm composed of columnar, mucin-producing epithelium, supported by ovariantype stroma (hematoxylin and eosin, 100×). (B) Section from neoplasm showing papillary projections, crypt-like invaginations, cellular pseudo stratification, and occasional mitoses (hematoxylin and eosin, 100×).
References 1. Hamilton SR, Aaltonen LA, eds. WHO Classification of Tumours of the Digestive System. Lyon, France: IARC Press; 2006. 2. Tanaka M, Chari S, Adsay V, et al. International consensus guidelines for management of intraductal papillary mucinous neoplasms and mucinous cystic neoplasms of the pancreas. Pancreatology. 2006;6:17-32.
3. Erdogan D, Lamers WH, Offerhaus GJ, Busch OR, Gouma DJ, van Gulik TM. Cystadenomas with ovarian stroma in liver and pancreas: an evolving concept. Dig Surg. 2006;23: 186-191. 4. Izumo A, Yamaguchi K, Eguchi T, et al. Mucinous cystic tumor of the pancreas: immunohistochemical assessment of “ovarian-type stroma”. Oncol Rep. 2003;10:515-525.
Copyright of International Journal of Surgical Pathology is the property of Sage Publications Inc. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use.
