Papillary Endothelial Proliferation Research Articles

Cystic lymphangiomatous lesions of the adrenal gland: A clinicopathological study of 37 cases including previously unreported cysts with papillary endothelial proliferation

Published data regarding lymphangiomatous cysts of the adrenal glands (also known as adrenal cystic lymphangiomas) are limited to case reports and a few small case series. We analyzed the clinicopathologic features and histomorphologic spectrum of 37 cases of adrenal cystic lymphangiomatous lesions. There were 26 females and 11 males ranging from 12 to 67 years old (median, 34 years). Twenty two lesions (59.5%) were diagnosed incidentally on imaging studies for unrelated causes, while 15 cases (40.5%) were symptomatic: 8 patients presented with abdominal or flank pain and 7 patients presented with arterial hypertension. Clinically, 4 lesions (10.8%) were reported to have concurrent hormonal hypersecretion. Follow-up data were available for 23 patients (62.2%), ranging from 6 to 156 months (median, 52 mo). One of the 22 patients showed local recurrence at 12 months after partial adrenalectomy. The median size of the adrenal lymphangiomatous cysts was 4.5 cm (range, 1.5 to 10 cm). Based on the histopathologic findings these lesions were grouped into three, morphologically distinct types: typical multicystic lymphatic malformation (n = 16), typical unilocular lymphangiomatous cyst (n = 14) and lymphangiomatous cyst with papillary endothelial proliferation (n = 7). The median patient age of the first group was significantly higher than that of the other groups and calcifications in these cysts were more common than in the other two groups. The unilocular lymphangiomatous cysts were more frequently associated with a history of previous intra-abdominal surgical procedures and/or inflammatory processes than the other two groups. Cysts with papillary endothelial proliferation were significantly larger than other cysts and shared some microscopic features with a vascular neoplasm known as papillary intralymphatic angioendothelioma (PILA). In conclusion, adrenal lymphangiomatous cysts are usually asymptomatic, incidentally diagnosed lesions with a female predominance. They may imitate other adrenal tumors, both radiologically and clinically. Despite being non-functioning lesions, they should be considered as a possible cause of pseudopheochromocytoma. Although most adrenal lymphangiomatous cysts seem to be non-neoplastic, vascular abnormalities (malformations or lymphangiectasias), those with papillary endothelial proliferations may represent true neoplastic lesions.

Oral Intravascular Papillary Endothelial Hyperplasia Associated with an Organizing Thrombus: Case Report and Immunohistochemical Analysis.

Intravascular papillary endothelial hyperplasia (IPEH) is a benign lesion of the skin and mucosa of vascular origin characterized by reactive proliferation of endothelial cells. A 76-year-old woman was referred presenting a painless nodule on the lip. Intraoral examination revealed bluish submucosal nodular proliferation, measuring 10 × 5 × 5 mm, affecting the lower labial mucosa. The lesion had a firm consistency and it was not fixed to the adjacent tissues. The main differential diagnoses were mucocele/mucus retention cyst, sialolith, or salivary gland neoplasia. An incisional biopsy was performed and during the intraoperative procedure an encapsulated red-bluish nodular mass was observed. Microscopic analysis revealed papillary endothelial proliferation in the center of the lesion and fibrin admixed with inflammatory cells in organization peripherally. There was no nuclear atypia, mitotic figures, or necrosis. The endothelial cells were CD34 positive, with low Ki-67 proliferation index (4%). α-SMA highlighted the vessel walls, whereas negativity for D2-40 excluded lymphatic origin. Final diagnosis was IPEH associated with an organizing thrombus. Dentists should be aware about this rare benign vascular lesion, whose final diagnosis is achieved only after histopathology analysis. Surgical removal is the treatment of choice and no recurrence is expected.

Orbital intravascular papillary endothelial hyperplasia in a Nigerian child: a case report and review of the literature

IntroductionIntravascular papillary endothelial hyperplasia is a reactive proliferative lesion of endothelial cells in blood vessels. It typically presents as a painless, reddish purple lesion in the sites affected. The orbit remains an uncommon site of affectation of this relatively common disease. It is noteworthy that this is the first reported case, to the best of our knowledge, of orbital intravascular papillary endothelial hyperplasia in a Nigerian child.Case presentationThe case reported here is an orbital intravascular papillary endothelial hyperplasia causing non-axial proptosis and loss of vision in a 14-year-old Nigerian boy. We describe the clinical and histological findings of intravascular papillary endothelial hyperplasia in the orbit of this 14-year-old boy. The key distinguishing features are discussed and relevant literature is reviewed.ConclusionAlthough unusual in presentation, intravascular papillary endothelial proliferation should be considered in the list of differentials of proptosis due to mass lesion in young Nigerians and, possibly, Africans.

Intravascular papillary endothelial hyperplasia of the digit: MRI features with histological correlation

To clarify the magnetic resonance (MR) features of the pure form of intravascular papillary endothelial hyperplasia, MR images (MRIs) from five patients were retrospectively reviewed and compared with histological findings. The images showed a heterogeneous, iso- to slightly high signal intensity mass on T1-weighted images and a mass with a central heterogeneous, iso- to slightly high signal intensity area completely or incompletely surrounded by peripheral high signal intensity areas on T2-weighted images. Heterogeneous enhancement was observed after gadolinium administration. Histological studies indicated that the central heterogeneous area on T2-weighted images corresponded to thrombi (organized and/or hyalinized) and/or papillary endothelial proliferation, and also that the peripheral high signal intensity area corresponded to vascular blood space and/or papillary endothelial proliferation. The pure form of intravascular papillary endothelial hyperplasia showed relatively characteristic features on MRIs.

Florid Capsular and Pericapsular Papillary Endothelial Proliferation Associated With Poorly Differentiated Thyroid Carcinoma

Papillary endothelial hyperplasia (PEH) occurring within or around the capsule of thyroid neoplasms is rare. Such Masson-like lesions, which have been attributed to fine needle aspiration-induced injury and thrombosis, can mimic tumor angioinvasion or vascular neoplasms. This study reports the case of a poorly differentiated carcinoma of the thyroid that was accompanied by more than 10 foci of pericapsular and peritumoral papillary endothelial hyperplasia, one of which contained a tumor embolus. The vessels within the papillary fronds had CD31- and CD34-staining blood vessels but were lined by D2-40 staining endothelial cells, which were continuous with the lining of the spaces within which PEH had developed. This suggests that, in contrast to Masson's lesions that develop within blood vessels as a result of thrombosis, pericapsular PEH associated with thyroid neoplasms develops within lymphatics, possibly related to lymphangiogenic factors secreted by the thyroid neoplasms.

Serious Renal Hemorrhage in Masson Tumor

Intravascular papillary endothelial hyperplasia (IPEH; Masson tumor) is a vascular lesion of blood vessels, first described in 1923 by Masson, who termed it “hemangioendotheliome vegetant Intravasculaire.” This lesion consists of an exuberant, usually intravascular, proliferation of normal endothelial cells and is considered to be a reactive vascular proliferation after traumatic vascular stasis. The disease frequently occurs in skin and subcutaneous tissue; occurrence in solid organs is rare. We report a rare case IPEH that recurred as a possible consequence of an acute hypertensive arterial crisis in a patient with chronic kidney failure after kidney transplantectomy. Thirty days after transplantectomy, a 49-year-old white man receiving hemodialysis had pain in the left abdominal flank and acute anemia with serious hypovolemia after a hypertensive arterial crisis. An emergency non–contrast enhanced abdominal computed tomographic scan showed a massive retroperitoneal hemorrhage. A left nephrectomy was performed for evidence of a native kidney breach with toilette of the abdominal cavity. Histologic analysis revealed that the renal lesion had several important distinguishing characteristics that confirmed the diagnosis of IPEH. This lesion is considered to be an usual form of thrombus organization that is marked by excessive papillary endothelial proliferation. In rare cases, it is present in a solid organ of the abdominal cavity especially the kidney. Intravascular papillary endothelial hyperplasia is a benign lesion but can be dangerous. Clinical, radiologic, and histologic diagnosis of IPEH is difficult.

Intravascular Papillary Endothelial Hyperplasia of the Jejunum

Intravascular papillary endothelial hyperplasia is considered to be an unusual form of thrombus organization that is marked by an excessive papillary endothelial proliferation. This lesion has the propensity to occur in the skin and the subcutis. Occurrence in the gastrointestinal tract is very rare. The authors report an exceptional case of a 20-year-old young woman with intravascular papillary endothelial hyperplasia in the jejunum. The patient was referred to the hospital with a 1-week history of melena. The lesion did not recur after surgery. Histopathological examination revealed a papillary endothelial hyperplasia with an underlying arteriovenous malformation.

Extravascular Papillary Endothelial Hyperplasia of the Larynx: A Case Report and Review of the Literature

Papillary endothelial hyperplasia (PEH), a tumor that falls along the spectrum of reactive to neoplastic vascular lesions, must be diagnosed carefully because it can resemble an angiosarcoma. PEH is generally considered to be the result of an unusual form of thrombus organization, exhibiting excessive papillary endothelial proliferation that is usually confined to the lumen of preexisting vessels or vascular malformations. Most cases of PEH are of the intravascular type; extravascular PEH is rare. We describe what we believe is the first reported case of an extravascular PEH in the larynx.