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

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.

The first recurrent intravascular papillary endothelial hyperplasia (Masson’s tumor) of the kidney

“…[7][8][9] Although recurrence rates in various skin cases have been documented in a range of 7-10%, any case with recurrent renal IPEH has not been reported in the English literature. [6][7][8] It has been considered that there is generally a concomitant vascular tumour in the recurrent cases. [13] In our case, we surprisingly detected a recurrent mass in the left kidney at the same localization with US, and MRI at the postoperative 3 rd months, which was not present on preoperative radiograms.…”

“…[5] Although IPEH may occur in any blood vessel throughout the body, the most common sites of these lesions are head, neck, fingers, and trunk. [5][6][7] Less frequently, it has been reported in the upper respiratory and gastrointestinal tracts, skeletal muscle, hepatic system, and rarely in the urogenital tract. [6,7] IPEH of the kidney is extremely rare, with only 8 cases previously reported in the English literature.…”

“…[5][6][7] Less frequently, it has been reported in the upper respiratory and gastrointestinal tracts, skeletal muscle, hepatic system, and rarely in the urogenital tract. [6,7] IPEH of the kidney is extremely rare, with only 8 cases previously reported in the English literature. [7][8][9] In this article, we present a novel case of recurrent IPEH in the kidney.…”

Angiosarcoma of the adrenal gland with concurrent contralateral advanced renal cell carcinoma: A diagnostic and management dilemma

“…IPEH is a benign, reactive process that cannot be differentiated radiologically from other benign or malignant lesions. 13 It can mimic malignancy, such as angiosarcoma, in particular in the context of underlying reactive changes related to treatment (sunitinib therapy). IPEH can also occur concomitantly with frank angiosarcoma, making the distinction between these two entities even more challenging, in particular if there is a lack of pathohistologic findings of marked cellular and nuclear pleomorphism, and significant mitotic activity.…”

“…7,11,14 Prognosis of these two neoplasms are dramatically different. 13,14 Angiosarcoma is an aggressive high-grade malignancy with a poor prognosis. 1,6,7 The mortality rate within the first year of diagnosis is over 50%.…”