Soft tissue

Vascular

Intermediate (locally aggressive / rarely metastasizing)

Retiform hemangioendothelioma



Last author update: 21 September 2023
Last staff update: 21 September 2023

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PubMed search: Retiform hemangioendothelioma

Qurratulain Chundriger, M.B.B.S.
Nasir Ud Din, M.B.B.S.
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Cite this page: Chundriger Q, Nusrat A, Ud Din N. Retiform hemangioendothelioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuehemangioendotheliomaretiform.html. Accessed April 14th, 2024.
Definition / general
  • Vascular neoplasm with locally aggressive behavior and characteristic morphology among other vascular lesions; exhibits a high recurrence rate and rarely has metastatic potential
Essential features
  • Locally aggressive, infiltrating vascular neoplasm composed of elongated arborizing vascular channels lined by endothelial cells with prominent hobnail nuclei with hyperchromasia but no atypia and rare mitotic activity
  • Presents as plaque-like areas of discoloration and high rate of local recurrence after incomplete excision
Terminology
  • Hobnail hemangioendothelioma (also used for papillary intralymphatic angioendothelioma / Dabska tumor)
ICD coding
  • ICD-O: 9136/1 - retiform hemangioendothelioma
  • ICD-11: 2B56.1 & XH64U8 - angiosarcoma of skin & retiform hemangioendothelioma
Epidemiology
Sites
Pathophysiology
  • Not known
Etiology
  • Not known
  • Case reports of lesions arising after low dose radiation and in association with lymphedema (Milroy disease)
Clinical features
  • Plaque-like, red to blue areas of discoloration
  • Size range: 3 - 12 cm
Diagnosis
  • Diagnosis is usually made on morphology alone
  • Immunohistochemical markers help confirm the vascular nature of the lesion
Laboratory
  • Not significant
  • Single case report of increased CA19-9 levels in a patient with splenic retiform hemangioendothelioma and concomitant hepatic amyloidosis (World J Clin Cases 2020;8:1108)
Radiology description
  • CT scan and MRI: solid enhancing mass lesion
  • Digital subtraction angiography (DSA): helps to identify feeding vessels for embolization
Radiology images

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Mediastinal masses

Mediastinal masses

External auditory canal lesion

External auditory canal lesion

Sphenoid bone origin

Sphenoid bone origin

Splenic lesion

Splenic lesion


Temporal bone lesion

Temporal bone lesion

Pre and postembolization DSA

Pre and postembolization DSA

Lymphography

Lymphography

Prognostic factors
  • Recurrence rates up to 60%
  • Margin involvement predisposes to recurrence
Case reports
Treatment
Clinical images

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Medial canthus lesion

Medial canthus lesion

Postauricular swelling

Postauricular swelling

Reddish plaques Reddish plaques

Reddish plaques


External auditory canal lesion

External auditory canal lesion

Scalp and postauricular lesions

Scalp and postauricular lesions

Raised nodular lesion on distal aspect of finger

Raised nodular lesion on distal aspect of finger

Gross description
Gross images

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Spinal lesion

Spinal lesion

External auditory canal lesion

External auditory canal lesion

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Nasir Ud Din, M.B.B.S. and Mowafak Hamodat, M.B.Ch.B., M.Sc. (Case #107)
Skin lesion

Skin lesion

Arborizing network Arborizing network

Arborizing network

Solid areas

Solid areas

Hobnailing

Hobnailing


Hobnailing

Hobnailing

Ectatic vessels

Ectatic vessels

Lymphoid aggregates

Lymphoid aggregates

Dabska tumor-like areas Dabska tumor-like areas

Dabska tumor-like areas


ERG

ERG

CD31 CD31

CD31

Cytology description
Positive stains
Negative stains
Molecular / cytogenetics description
  • 38% of cases in a single study were shown to harbor YAP1 gene rearrangements, with a subset showing YAP1::MAML2 fusions (Am J Surg Pathol 2020;44:1677)
Molecular / cytogenetics images

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YAP1 gene rearrangements

YAP1 gene rearrangements

Videos

Hemangioendotheliomas

Pathology of hemangioendotheliomas

Sample pathology report
  • Thigh lesion, shave excisional biopsy:
    • Vascular neoplasm with locally aggressive course, consistent with retiform hemangioendothelioma (see comment)
    • Comment: These tumors are known to recur (recurrence rate up to 60% in the literature), particularly when resection margins are not clear.
Differential diagnosis
  • Hobnail hemangioma:
    • Well circumscribed cutaneous lesion
    • Superficial vessels are more hobnail as compared to deep ones
    • No local recurrence reported
  • Papillary intralymphatic angioendothelioma (PILA / Dabska tumor):
    • Intraluminal papillary formations are diffusely present with perivascular and papillary core hyalinization
    • Similar areas may be seen in some examples of retiform hemangioendothelioma but this entity lacks typical retiform architecture
    • D2-40 positivity in endothelial cells
    • Adjacent lymphangioma-like areas and hemosiderin deposition may be seen
  • Composite hemangioendothelioma:
    • Shows mixture of more than hemangioendothelioma types, including Dabska tumor, retiform hemangioendothelioma and hobnail hemangioendothelioma, etc. in various combinations
    • Whenever possible, thorough sampling should be done to exclude a possibility of composite hemangioendothelioma when any hemangioendothelioma pattern is encountered
  • Kaposi sarcoma:
    • Mostly arises in HIV patients with typical clinical presentation
    • Composed of slit-like vessels with spindle cells, hyaline globules and appreciable mitotic activity
    • Immunohistochemistry for HHV8 is positive
  • Angiosarcoma:
    • Ill defined lesion with infiltrative appearance, mostly in older patients
    • Shows various architectural patterns with well formed vascular spaces, spindle cells with extravasated red blood cells, all showing prominent nuclear atypia and brisk mitotic activity
  • Atypical vascular lesion:
    • Develops in skin a few years after radiation
    • Mostly confined to dermis
    • Interconnected vascular channels lined by flattened to hobnail cells
    • May resemble retiform hemangioendothelioma in small biopsies; clinical history is important in distinguishing the two
Board review style question #1

A 30 year old man presents with a red to purple plaque involving the right thigh. A punch biopsy through the lesion shows light microscopic features as depicted in the photomicrograph above. Which of the following is the most likely diagnosis in this case?

  1. Atypical vascular lesion
  2. Intramuscular angioma
  3. Kaposi sarcoma
  4. Papillary intralymphatic angioendothelioma
  5. Retiform hemangioendothelioma
Board review style answer #1
E. Retiform hemangioendothelioma. The photomicrograph shows a vascular lesion with retiform vessels lined by a single layer of endothelial cells with hobnailing. A lymphoid aggregate is also present. Answers B - D are incorrect because other characteristic essential features of any of the given options, except atypical vascular lesion, are not seen here. Answer A is incorrect because the clinical scenario is not appropriate for a diagnosis of atypical vascular lesion. Therefore, the correct diagnosis is retiform hemangioendothelioma.

Comment Here

Reference: Retiform hemangioendothelioma
Board review style question #2
A 45 year old HIV positive woman presents with a violaceous papule on her neck. Punch biopsy of the lesion shows crushing artifacts. Preserved areas show interconnected rete testis-like vascular spaces lined by a single layer of endothelial cells with hobnailing. Which of the following is the most important immunohistochemical stain to differentiate the diagnosis from other possibilities in the scenario?

  1. CD34
  2. Claudin5
  3. D2-40
  4. HHV8
  5. Ki67
Board review style answer #2
D. HHV8. In the given clinical scenario, the most important differential diagnostic consideration here is Kaposi sarcoma. HHV8 is known to be positive in Kaposi sarcoma but not in retiform hemangioendothelioma, which is the correct diagnosis. Answers A and B are incorrect because CD34 and claudin5 are positive in both retiform hemangioendothelioma and Kaposi sarcoma. Answer C is incorrect because D2-40 can be positive in some cases of both. Answer E is incorrect because Ki67 or MIB1 proliferative index is not a diagnostic marker for either diagnosis.

Comment Here

Reference: Retiform hemangioendothelioma
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