An uncommon vascular lesion over the right hand

A 45-year female presented with progressively enlarging, 2 × 1 cm, soft, mobile, non-tender swelling over the dorsum of the right hand around the wrist joint for 6 months. Fine needle aspiration cytology are shown in Figure 1.

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Figure 1:

(a) Isolated cell clusters with plump nuclei displaying “hobnailing” (May Grunwald-Giemsa stain ×100). (b) There is moderate pleomorphism with distinct nucleoli (Papanicolaou stain ×400). (c) A venous wall with intravascular organized thrombus (Hematoxylin and Eosin stain ×400). (d) Thrombus invaginated by anastomosing delicate papillae with a hyalinized core lined by endothelial cells (Hematoxylin and Eosin stained cell-block section ×100). (e) Plump endothelial cells with hobnail nuclei (Hematoxylin and Eosin stained cell-block section ×400).

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Q1. What is your interpretation?

1. Hemangioma

2. Angiosarcoma

3. Papillary endothelial hyperplasia (PEH)

4. Hobnail hemangioendothelioma.

Answer: c. Papillary endothelial hyperplasia (PEH)

Common sites of involvement by PEH include the head-and-neck, lip, tongue, buccal mucosa, and limbs. Usually presents as a reddish-blue nodular lesion in the skin or mucosa.^[1] On histology, they present as an intravascular lesion with residual organizing thrombus, anastomosing vascular channels, and papillary formations with hyaline or fibrous stalks lined by plump endothelial cells (histiocyte or epithelial-like).^[2-5]

Q2. Which among the following finding is characteristically seen in PEH

1. Hobnail

2. Thrombus

3. Nuclear atypia

4. Necrosis.

The differential diagnoses considered include vasoformative lesions such as hemangioma, angiosarcoma, and hobnail hemangioendothelioma. Hemangioma and angiosarcoma commonly involve the skin and subcutis of the head-and-neck region. Angiosarcomas exhibit marked nuclear atypia, necrosis, and mitoses. They are never confined to the intravascular location.^[1] Hobnail hemangioendothelioma (retiform type) is a rare intermediate-grade (locally aggressive) tumor of vascular origin affecting distal extremities. It consists of numerous elongated vessels (retiform) involving the dermis and extending into the subcutis. They display “hobnail” endothelial cells similar to PEH.^[6] However, they lack the organized thrombus seen in histology sections.^[6]

Q3. Which of the following is a reactive, non-neoplastic process.

1. Arteriovenous malformation

2. Hereditary hemorrhagic telangiectasia

3. PEH

4. Hobnail hemangioma.

Q4. Papillary endothelial hyperplasia is not a common feature of PEH:

1. Usually begins within a thrombosed blood vessel

2. They usually have abundant thrombotic material

3. They show papillae are lined by plump, normochromatic endothelial cells, in a single, non-stratified layer

4. PEH shows frequent mitotic figures.

Papillary endothelial hyperplasia is a reactive, non-neoplastic lesion representing an exuberant organization and recanalization of the thrombus.^[3] It occurs in normal vessels as well as in preexisting vascular lesions such as varices, hemorrhoids, pyogenic granulomas, hematomas, and angiosarcomas. The clinical presentation is non-specific, so histopathological examination is ultimately required to establish the diagnosis.