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Vol. 4, No. 1, 2010
Issue release date: January – April
Open Access Gateway
Case Rep Gastroenterol 2010;4:124–132
(DOI:10.1159/000294148)

Intraabdominal Intravascular Papillary Endothelial Hyperplasia (Masson’s Tumor): A Rare and Novel Cause of Gastrointestinal Bleeding

Meadows M.C.a, d · Sun X.b · Dardik M.b · Tarantino D.R.a · Chamberlain R.S.a, c, d
Departments of aSurgery and bPathology, Saint Barnabas Medical Center, Livingston, N.J., and cDepartment of Surgery, University of Medicine and Dentistry of New Jersey, Newark, N.J., USA; dSt. George’s University School of Medicine, Grenada, West Indies
email Corresponding Author

Abstract

Intravascular papillary endothelial hyperplasia (IPEH), or Masson’s tumor, a rare benign vascular lesion, occurs mainly in the head, neck, and hands in the human population. Aberrant tumor locations have been rarely reported. We present a case of a patient with chronic abdominal pain and melena of variable severity due to a Masson’s tumor, with no apparent Masson’s tumor-associated comorbidities, along with a comprehensive review of the literature. Using PubMed, a search engine provided by the U.S. National Library of Medicine and the National Institutes of Health, we searched for all reports of Masson’s tumor limited within the abdominal cavity. Furthermore, keywords such as ‘intravascular papillary endothelial hyperplasia’, ‘renal’, ‘gastrointestinal’, ‘hepatic’ and ‘intraabdominal’ were used to facilitate the search. We thus found fourteen cases of intraabdominal Masson’s tumors published. Six (42.9%) of these were located in the renal vein, 4 (28.6%) were reported in the gastrointestinal tract, 1 (7.1%) in the adrenal gland, 1 (7.1%) in the liver, and 1 (7.1%) instance with multiple lesion sites including the renal hilum and retroperitoneum. Among these patients, 9 (64.3%) were female and 5 (35.7%) male, with a mean age of 38.9 years (7–69). IPEH is a reactive process, having three subtypes, all involving the proliferation of epithelial cells around a thrombus in the setting of venous stasis. In its pure form, the organized thrombus is solely localized within the vascular lumen. Mixed-form IPEH is formed in preexisting vascular lesions (such as arteriovenous malformation, hemangioma, pyogenic granuloma, etc.). The rarest form is the extravascular variety, which arises in hematomas often from recent trauma to the area. In its pure form, IPEH has a zero recurrence rate when an R0 resection is performed; all mixed and extravascular forms show the highest recurrence rates. The exact histogenesis of these epithelial cells remains a mystery and multiple theories have been offered. Although difficult to distinguish from malignant angiosarcomas solely on presentation and radiologic work-up, Masson’s tumors occur more frequently in women, demonstrate no local invasion, do not metastasize, are commonly located intravascularly, and are associated with a significantly more favorable prognosis than angiosarcomas. Only four Masson’s tumors have been reported in the gastrointestinal tract, two of these cases were related to microvascular thrombosis secondary to paroxysmal nocturnal hemoglobinuria and two were formed secondary to arteriovenous malformations. Our case lacked solitary evidence of either of these comorbidities. An incidental finding of an enlarged ovary, which was removed during our exploratory laparoscopy, plus strong demographic statistics that suggest women have an increased prevalence of this lesion may help support a hormonal theory of pathogenesis.


 Outline


 goto top of outline Abstract

Intravascular papillary endothelial hyperplasia (IPEH), or Masson’s tumor, a rare benign vascular lesion, occurs mainly in the head, neck, and hands in the human population. Aberrant tumor locations have been rarely reported. We present a case of a patient with chronic abdominal pain and melena of variable severity due to a Masson’s tumor, with no apparent Masson’s tumor-associated comorbidities, along with a comprehensive review of the literature. Using PubMed, a search engine provided by the U.S. National Library of Medicine and the National Institutes of Health, we searched for all reports of Masson’s tumor limited within the abdominal cavity. Furthermore, keywords such as ‘intravascular papillary endothelial hyperplasia’, ‘renal’, ‘gastrointestinal’, ‘hepatic’ and ‘intraabdominal’ were used to facilitate the search. We thus found fourteen cases of intraabdominal Masson’s tumors published. Six (42.9%) of these were located in the renal vein, 4 (28.6%) were reported in the gastrointestinal tract, 1 (7.1%) in the adrenal gland, 1 (7.1%) in the liver, and 1 (7.1%) instance with multiple lesion sites including the renal hilum and retroperitoneum. Among these patients, 9 (64.3%) were female and 5 (35.7%) male, with a mean age of 38.9 years (7–69). IPEH is a reactive process, having three subtypes, all involving the proliferation of epithelial cells around a thrombus in the setting of venous stasis. In its pure form, the organized thrombus is solely localized within the vascular lumen. Mixed-form IPEH is formed in preexisting vascular lesions (such as arteriovenous malformation, hemangioma, pyogenic granuloma, etc.). The rarest form is the extravascular variety, which arises in hematomas often from recent trauma to the area. In its pure form, IPEH has a zero recurrence rate when an R0 resection is performed; all mixed and extravascular forms show the highest recurrence rates. The exact histogenesis of these epithelial cells remains a mystery and multiple theories have been offered. Although difficult to distinguish from malignant angiosarcomas solely on presentation and radiologic work-up, Masson’s tumors occur more frequently in women, demonstrate no local invasion, do not metastasize, are commonly located intravascularly, and are associated with a significantly more favorable prognosis than angiosarcomas. Only four Masson’s tumors have been reported in the gastrointestinal tract, two of these cases were related to microvascular thrombosis secondary to paroxysmal nocturnal hemoglobinuria and two were formed secondary to arteriovenous malformations. Our case lacked solitary evidence of either of these comorbidities. An incidental finding of an enlarged ovary, which was removed during our exploratory laparoscopy, plus strong demographic statistics that suggest women have an increased prevalence of this lesion may help support a hormonal theory of pathogenesis.

Copyright © 2010 S. Karger AG, Basel


 goto top of outline Author Contacts

Ronald S. Chamberlain, MD, MPA, FACS
Saint Barnabas Medical Center, 94 Old Short Hills Road, Suite 1172
Livingston, NJ 07039 (USA)
Tel. +1 973 322 5195, Fax +1 973 322 2471, E-Mail rchamberlain@sbhcs.com


 goto top of outline Article Information

Published online: March 20, 2010
Number of Print Pages : 9
Number of Figures : 3, Number of Tables : 1,


 goto top of outline Publication Details

Case Reports in Gastroenterology

Vol. 4, No. 1, Year 2010 (Cover Date: January - April)

Journal Editor: Urrutia R. (Rochester, Minn.)
ISSN: NIL (Print), eISSN: 1662-0631 (Online)

For additional information: http://www.karger.com/CRG


Open Access License / Drug Dosage / Disclaimer

Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

Abstract

Intravascular papillary endothelial hyperplasia (IPEH), or Masson’s tumor, a rare benign vascular lesion, occurs mainly in the head, neck, and hands in the human population. Aberrant tumor locations have been rarely reported. We present a case of a patient with chronic abdominal pain and melena of variable severity due to a Masson’s tumor, with no apparent Masson’s tumor-associated comorbidities, along with a comprehensive review of the literature. Using PubMed, a search engine provided by the U.S. National Library of Medicine and the National Institutes of Health, we searched for all reports of Masson’s tumor limited within the abdominal cavity. Furthermore, keywords such as ‘intravascular papillary endothelial hyperplasia’, ‘renal’, ‘gastrointestinal’, ‘hepatic’ and ‘intraabdominal’ were used to facilitate the search. We thus found fourteen cases of intraabdominal Masson’s tumors published. Six (42.9%) of these were located in the renal vein, 4 (28.6%) were reported in the gastrointestinal tract, 1 (7.1%) in the adrenal gland, 1 (7.1%) in the liver, and 1 (7.1%) instance with multiple lesion sites including the renal hilum and retroperitoneum. Among these patients, 9 (64.3%) were female and 5 (35.7%) male, with a mean age of 38.9 years (7–69). IPEH is a reactive process, having three subtypes, all involving the proliferation of epithelial cells around a thrombus in the setting of venous stasis. In its pure form, the organized thrombus is solely localized within the vascular lumen. Mixed-form IPEH is formed in preexisting vascular lesions (such as arteriovenous malformation, hemangioma, pyogenic granuloma, etc.). The rarest form is the extravascular variety, which arises in hematomas often from recent trauma to the area. In its pure form, IPEH has a zero recurrence rate when an R0 resection is performed; all mixed and extravascular forms show the highest recurrence rates. The exact histogenesis of these epithelial cells remains a mystery and multiple theories have been offered. Although difficult to distinguish from malignant angiosarcomas solely on presentation and radiologic work-up, Masson’s tumors occur more frequently in women, demonstrate no local invasion, do not metastasize, are commonly located intravascularly, and are associated with a significantly more favorable prognosis than angiosarcomas. Only four Masson’s tumors have been reported in the gastrointestinal tract, two of these cases were related to microvascular thrombosis secondary to paroxysmal nocturnal hemoglobinuria and two were formed secondary to arteriovenous malformations. Our case lacked solitary evidence of either of these comorbidities. An incidental finding of an enlarged ovary, which was removed during our exploratory laparoscopy, plus strong demographic statistics that suggest women have an increased prevalence of this lesion may help support a hormonal theory of pathogenesis.



 goto top of outline Author Contacts

Ronald S. Chamberlain, MD, MPA, FACS
Saint Barnabas Medical Center, 94 Old Short Hills Road, Suite 1172
Livingston, NJ 07039 (USA)
Tel. +1 973 322 5195, Fax +1 973 322 2471, E-Mail rchamberlain@sbhcs.com


 goto top of outline Article Information

Published online: March 20, 2010
Number of Print Pages : 9
Number of Figures : 3, Number of Tables : 1,


 goto top of outline Publication Details

Case Reports in Gastroenterology

Vol. 4, No. 1, Year 2010 (Cover Date: January - April)

Journal Editor: Urrutia R. (Rochester, Minn.)
ISSN: NIL (Print), eISSN: 1662-0631 (Online)

For additional information: http://www.karger.com/CRG


Open Access License / Drug Dosage

Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) (www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.