| Abstract|| |
In this case report, we present a case of a 47-year-old man with sinusoidal hemangioma with secondary intravascular papillary endothelial hyperplasia arising in the subcutaneous tissue. To the best of our knowledge, there are only two cases of sinusoidal hemangioma with secondary intravascular papillary endothelial hyperplasia reported till date, both of which were intramuscular hemangiomas. These are rare entities that also have overlapping histomorphological features with that of various other vascular tumors making histopathological diagnosis troublesome at times. Careful histopathological examination aided by immunohistochemical studies in difficult cases is essential to avoid these diagnostic pitfalls.
Keywords: Benign vascular tumor, hemangioma, intravascular papillary endothelial hyperplasia, Masson's tumor, sinusoidal hemangioma
|How to cite this article:|
Gurumurthy RY, Shankar NS, Mohan Raj C S, Sriram N. Sinusoidal hemangioma with secondary intravascular papillary endothelial hyperplasia. Indian J Pathol Microbiol 2020;63:279-81
|How to cite this URL:|
Gurumurthy RY, Shankar NS, Mohan Raj C S, Sriram N. Sinusoidal hemangioma with secondary intravascular papillary endothelial hyperplasia. Indian J Pathol Microbiol [serial online] 2020 [cited 2021 Jun 13];63:279-81. Available from: https://www.ijpmonline.org/text.asp?2020/63/2/279/282685
| Introduction|| |
Sinusoidal hemangioma was first described by Calonje E and Fletcher CD in 1991 as a distinctive benign vascular neoplasm within the group of cavernous hemangioma. Intravascular papillary endothelial hyperplasia (IPEH) is a reactive condition characterized by exuberant proliferation of endothelial cells. In this case report, we present the case of a 47-year-old man with sinusoidal hemangioma with secondary IPEH arising in the subcutaneous tissue. To the best of our knowledge, there are only two cases of sinusoidal hemangioma with secondary IPEH reported till date, both of which were intramuscular hemangiomas.,
| Case Report|| |
A 47-year-old man came with the complaints of a painless, gradually progressive growth over anterior abdominal wall. On examination, a solitary swelling was noted over right side of the anterior abdominal wall that was nodular, firm, and non-tender. Excision biopsy of the growth was performed.
On gross examination of the surgical specimen, a grey-brown mass measuring about 4 × 3 cm2 was noted. Cut surface of the specimen was spongy and hemorrhagic. Microscopic examination of the mass, showed a tumor with characteristic features of sinusoidal hemangioma as shown in [Figure 1]. Large areas of degenerative changes like hyalinization were noted along with many vascular channels showing organizing and recanalizing thrombi. A dilated vascular channel with organizing thrombi also showed features of IPEH as shown in [Figure 2]. The endothelial cells of the vascular channels in the tumor were immunopositive for CD31, CD34, and ERG and immunonegative for D2-40 as shown in [Figure 3]. The report was signed out as sinusoidal hemangioma with secondary IPEH.
|Figure 1: Sinusoidal Hemangioma. (a) Photomicrograph of the tumor showing well-circumscribed lobules of vascular channels. (b) Photomicrograph showing gaping, dilated and congested, thin-walled blood vessels arranged in back-to-back pattern with very little intervening stroma. (c and d) Photomicrograph showing septations and interconnecting blood vessels. (e) Photomicrograph showing pseudopapillary structures. (f) Photomicrograph showing a recanalizing thrombus|
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|Figure 2: Intravascular Papillary Endothelial Hyperplasia. (a) Photomicrograph showing organizing and recanalizing thrombi in a large vascular channel with multiple papillary structures lined by hyperplastic endothelium. (b and c) Photomicrograph showing papillary structures lined by hyperplastic epithelium|
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|Figure 3: Immunohistochemical Studies. Vascular endothelial cells showing (a) immunopositivity for CD31. (b) immunopositivity for CD34. (c) immunopositivity for ERG. (d) immunonegativity to D2-40|
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| Discussion|| |
Sinusoidal hemangiomas usually present as subcutaneous or deep dermal lesions as seen in the present case. They characteristically have a nodular appearance as in our case, although rarely they present as plurinodular polypoidal formations or cystic structures covered by normal skin. On gross examination, tumors usually vary in size from 1 to 3.5 cm, although the largest reported tumor measured 11.5 cm. On cut section, they will be well circumscribed, spongy and hemorrhagic.
Histologically sinusoidal hemangiomas have a predominantly lobular architecture, which peripherally may show ill-defined spread into the subcutaneous tissue. They are characterized by dilated and congested, interconnecting, thin-walled blood vessels arranged in back-to-back pattern with very little intervening stroma. The vascular channels frequently show pseudopapillary patterns/septations. The vascular channels are lined by single-layered endothelium, which show focal pleomorphism and hyperchromasia. Some of the tumors may show thrombosis, hyalinization, dystrophic calcification, ossification, and even infarction. On immunohistochemistry, they show positivity for markers such as CD31, CD34. Present case had all the classical histomorphological features of sinusoidal hemangioma with some degenerative changes. An unusual finding in the present case was the presence of secondary IPEH.
IPEH can be divided into three types: (1) pure/primary IPEH, which arises denovo in dilated vascular channels, (2) reactive/mixed/secondary IPEH, which presents as a focal change in a pre-existing vascular lesion as in present case, (3) extravascular IPEH, which is a rare form seen in hematomas. On histopathology, the characteristic feature of IPEH is papillary structures lined by hyperplastic endothelial cells within the vascular lumen.
In 2018, Diaz-Flores et al. reported that sinusoidal hemangioma and IPEH are interrelated processes. In their study, they noted that both sinusoidal hemangiomas and IPEH have papillae covered by CD31 and CD34 positive endothelial cells and a core formed by either type 1 collagen and SMA positive cells or thrombotic components. The major difference between these two entities are the number, arrangement, and substrate of papillae: Myriad, densely grouped, parietal and thrombotic papillae are seen in IPEH whereas a linear arrangement of predominant parietal papillae is noted in sinusoidal hemangioma. They also demonstrated that both these entities have a similar piecemeal angiogenic mechanism in papillary formation, including sprouting of intimal endothelial cells towards the vessel wall itself or intravascular thrombi, formation of vascular loops that encircle and separate vessel wall or thrombus components and parietal or thrombotic papillae development.
Sinusoidal hemangiomas have overlapping histomorphological features with that of many other vascular neoplasms such as cavernous hemangioma, spindle cell hemangioma, juvenile hemangioma, and angiosarcoma. Well-differentiated angiosarcoma is also considered to be a differential diagnosis of IPEH and thus these entities should be carefully differentiated. Angiosarcoma is a interstitial lesion, which typically shows diffuse infiltration, atypical mitosis and multilayered endothelial cells. Immunohistochemical studies with Ki-67 can be used in troublesome cases.
| Conclusion|| |
Sinusoidal hemangioma and IPEH are rare entities and have histopathological features overlapping with that of various benign and malignant vascular neoplasms. A careful histopathological examination aided by immunohistochemical studies in difficult cases is required to avoid diagnostic pitfalls.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.
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Radhika Yajaman Gurumurthy
Department of Histopathology, Bhagavan Pathology Laboratory, #1116, 5th Cross, 1503, Srirampet, Vinoba Road, Mysore - 570 001, Karnataka
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3]