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Large ectopic pancreas (Heinlich 3) of ileum causing intussusceptions and ileus
Human Pathology: Case Reports (2014) 1, 29–30
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Letter to the Editor Large ectopic pancreas (Heinlich 3) of ileum causing intussusceptions and ileus To the Editor: Ectopic pancreas (EP) is classified into three types: Heinlich type 1 (pancreatic islets + acinus + ducts), type 2 (pancreatic acinus + ducts), and type 3 (only pancreatic ducts). EP commonly occurs in stomach and duodenum, but EP in ileum is very rare. Further, large EP of ileum causing intussusceptions and obstructive ileus has rarely been reported [1]. A 68-year-old man was emergently admitted to our hospital because of abdominal pain and nausea. Abdominal X-P and CT revealed free gas and niveau. Emergency endoscopy revealed no significant changes in duodenum, stomach, esophagus, colon and rectum. Blood laboratory data showed mild leukocytosis. Emergency laparotomy was performed, which showed intussusceptions and ileus of the ileum. Resection of the diseased ileum was performed. The macroscopic diagnosis of surgeons was submucosal tumor; i.e. lymphoma or GIST. The resected ileum showed polypoid submucosal tumor measuring 2.5 × 2.5 × 3 cm (Fig. 1A and B). The tumor was well defined and white. Microscopically, the tumor was composed of ductal element and smooth muscle element; the appearances were the so-called adenomyoma (Fig. 2A). The ductal element was free from atypia and contained mucins (Fig. 2B). The smooth
Fig. 1
muscle element consisted of mature smooth muscles without atypia. No endocrine cells, islet cells, acinar cells, chief cells, parietal cells, or other specific cells were seen. Mucin stains (PAS, d-PAS, and Alcian blue at pH 2.5 and 1.0) showed neutral, carboxylated and sulfated mucins in the cytoplasm. An immunohistochemical analysis was performed by the Dako Envision method and its modifications [2-5]. Immunohistochemically, the ductal cells showed the following cytokeratin (CK) profile: AE1/3 (+++) CK CAM5.2 (+++), CK34BE12 (−), CK7 (+++), CK8 (++), CK14 (−), CK18 (++), CK19 (+++), and CK20 (−). They showed the following MUC profile: MUC1 (−), MUC2 (−), MUC5AC (++), and MUC6 (+++). The ductal cells were positive for CA125 (+), CA19-9 (++), EMA (+), synaptophysin (+, only focal), and CD56 (+, only focal). They were negative for p53, p63, vimentin, CD34, smooth muscle actin (SMA), desmin, CDX-2, estrogen receptor (ER), progesterone receptor (PgR), TTF-1, KIT, S-100 protein, CD68, and surfactant apoprotein A. The Ki-67 labeling was 10%. In contrast, the smooth muscle element was positive for SMA, vimentin, and desmin and negative for p53, CD34, KIT, and S100; the findings were compatible with benign nature of the element. The Ki-67 labeling was 1%. The histology of the present tumor is compatible with EP of type 3. The CK profile is also of pancreatic-biliary type. However, the MUC and CDX-2 pattern is suggestive of gastric foveolar type. Although the author thinks that the lesion is EP, the possibility that the lesion is
Gross features of the tumor (arrow). A: Gross features. B: Cut surface.
2214-3300/© 2014 Published by Elsevier Inc.
30
Letter to the Editor
Fig. 2 Microscopic features of the ileal tumor. The tumor is composed of ductal and smooth muscle elements. No atypical features are seen. HE: A: × 15. B; × 100.
hamartoma of biliary and gastric phenotypes cannot be excluded. Hamartoma of colorectal epithelium is unlikely. Therefore it can be more appropriate to label this lesion simply as “adenomyoma”. The present tumor is not endometriosis because of the histologies and negative ER and PgR. It is not GIST because of negative KIT and CD34. It is also interesting that the ductal and smooth muscle elements of the lesion have proliferative fraction, as revealed by Ki-67 labeling. EP of the ileum is very rare, and gut large EP causing intussusception and obstructive ileus is very are. The patient is now well, and is free from symptoms. The author has no conflict of interest. Tadashi Terada M.D., Ph.D Department of Pathology, Shizuoka City Shimizu Hospital Miyakami 1231 Shimizu-Ku, Shizuoka 424-8636, Japan
Tel.: + 81 4 336 1111; fax: + 81 54 334 1173 E-mail address: [email protected] http://dx.doi.org/10.1016/j.ehpc.2014.09.006
References [1] Fujita N, Kimura R, Yamamura J, Akazawa K, Kasugai T, Tsukamoto F. Leiomyosarcoma of the breast: a case report and review of the literature about therapeutic management. Breast 2011;20:389-93. [2] Terada T, Kawaguchi M, Furukawa K, Sekido Y, Osamura Y. Minute mixed ductal-endocrine carcinoma of the pancreas with predominant intraductal growth. Pathol Int 2002;52:740-6. [3] Terada T. Ductal adenoma of the breast: immunohistochemistry of two cases. Pathol Int 2008;58:801-5. [4] Gall bladder adenocarcinoma arising in Rokitansky-Schoff sinuses. Pathol Int 2008;58:806-9. [5] Terada T. Intraductal tubular carcinoma, intestinal type, of the pancreas. Pathol Int 2009;59:53-8.
www.humanpathologycasereports.com
Letter to the Editor Large ectopic pancreas (Heinlich 3) of ileum causing intussusceptions and ileus To the Editor: Ectopic pancreas (EP) is classified into three types: Heinlich type 1 (pancreatic islets + acinus + ducts), type 2 (pancreatic acinus + ducts), and type 3 (only pancreatic ducts). EP commonly occurs in stomach and duodenum, but EP in ileum is very rare. Further, large EP of ileum causing intussusceptions and obstructive ileus has rarely been reported [1]. A 68-year-old man was emergently admitted to our hospital because of abdominal pain and nausea. Abdominal X-P and CT revealed free gas and niveau. Emergency endoscopy revealed no significant changes in duodenum, stomach, esophagus, colon and rectum. Blood laboratory data showed mild leukocytosis. Emergency laparotomy was performed, which showed intussusceptions and ileus of the ileum. Resection of the diseased ileum was performed. The macroscopic diagnosis of surgeons was submucosal tumor; i.e. lymphoma or GIST. The resected ileum showed polypoid submucosal tumor measuring 2.5 × 2.5 × 3 cm (Fig. 1A and B). The tumor was well defined and white. Microscopically, the tumor was composed of ductal element and smooth muscle element; the appearances were the so-called adenomyoma (Fig. 2A). The ductal element was free from atypia and contained mucins (Fig. 2B). The smooth
Fig. 1
muscle element consisted of mature smooth muscles without atypia. No endocrine cells, islet cells, acinar cells, chief cells, parietal cells, or other specific cells were seen. Mucin stains (PAS, d-PAS, and Alcian blue at pH 2.5 and 1.0) showed neutral, carboxylated and sulfated mucins in the cytoplasm. An immunohistochemical analysis was performed by the Dako Envision method and its modifications [2-5]. Immunohistochemically, the ductal cells showed the following cytokeratin (CK) profile: AE1/3 (+++) CK CAM5.2 (+++), CK34BE12 (−), CK7 (+++), CK8 (++), CK14 (−), CK18 (++), CK19 (+++), and CK20 (−). They showed the following MUC profile: MUC1 (−), MUC2 (−), MUC5AC (++), and MUC6 (+++). The ductal cells were positive for CA125 (+), CA19-9 (++), EMA (+), synaptophysin (+, only focal), and CD56 (+, only focal). They were negative for p53, p63, vimentin, CD34, smooth muscle actin (SMA), desmin, CDX-2, estrogen receptor (ER), progesterone receptor (PgR), TTF-1, KIT, S-100 protein, CD68, and surfactant apoprotein A. The Ki-67 labeling was 10%. In contrast, the smooth muscle element was positive for SMA, vimentin, and desmin and negative for p53, CD34, KIT, and S100; the findings were compatible with benign nature of the element. The Ki-67 labeling was 1%. The histology of the present tumor is compatible with EP of type 3. The CK profile is also of pancreatic-biliary type. However, the MUC and CDX-2 pattern is suggestive of gastric foveolar type. Although the author thinks that the lesion is EP, the possibility that the lesion is
Gross features of the tumor (arrow). A: Gross features. B: Cut surface.
2214-3300/© 2014 Published by Elsevier Inc.
30
Letter to the Editor
Fig. 2 Microscopic features of the ileal tumor. The tumor is composed of ductal and smooth muscle elements. No atypical features are seen. HE: A: × 15. B; × 100.
hamartoma of biliary and gastric phenotypes cannot be excluded. Hamartoma of colorectal epithelium is unlikely. Therefore it can be more appropriate to label this lesion simply as “adenomyoma”. The present tumor is not endometriosis because of the histologies and negative ER and PgR. It is not GIST because of negative KIT and CD34. It is also interesting that the ductal and smooth muscle elements of the lesion have proliferative fraction, as revealed by Ki-67 labeling. EP of the ileum is very rare, and gut large EP causing intussusception and obstructive ileus is very are. The patient is now well, and is free from symptoms. The author has no conflict of interest. Tadashi Terada M.D., Ph.D Department of Pathology, Shizuoka City Shimizu Hospital Miyakami 1231 Shimizu-Ku, Shizuoka 424-8636, Japan
Tel.: + 81 4 336 1111; fax: + 81 54 334 1173 E-mail address: [email protected] http://dx.doi.org/10.1016/j.ehpc.2014.09.006
References [1] Fujita N, Kimura R, Yamamura J, Akazawa K, Kasugai T, Tsukamoto F. Leiomyosarcoma of the breast: a case report and review of the literature about therapeutic management. Breast 2011;20:389-93. [2] Terada T, Kawaguchi M, Furukawa K, Sekido Y, Osamura Y. Minute mixed ductal-endocrine carcinoma of the pancreas with predominant intraductal growth. Pathol Int 2002;52:740-6. [3] Terada T. Ductal adenoma of the breast: immunohistochemistry of two cases. Pathol Int 2008;58:801-5. [4] Gall bladder adenocarcinoma arising in Rokitansky-Schoff sinuses. Pathol Int 2008;58:806-9. [5] Terada T. Intraductal tubular carcinoma, intestinal type, of the pancreas. Pathol Int 2009;59:53-8.