Soft tissue

Vascular

Benign

Hemangioma - overview



Topic Completed: 6 January 2022

Minor changes: 6 January 2022

Copyright: 2002-2022, PathologyOutlines.com, Inc.

PubMed search: hemangioma [TI] soft tissue [TIAB] free full text [SB]

Qurratulain Chundriger, M.B.B.S.
Nasir Ud Din, M.B.B.S.
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Cite this page: Chundriger Q, Ud Din N. Hemangioma - overview. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuehemangioma.html. Accessed January 19th, 2022.
Definition / general
Essential features
  • Most cases arise in children, with equal gender distribution
  • Composed of small capillary sized blood vessels with a larger feeding vessel commonly present
  • Most cases are treated with topical or systemic beta blockers in isolation or in combination with other modalities like laser therapy, excision, etc.
  • Recurrence is uncommon and only exceptional examples show malignant transformation
Terminology
  • Hemangioma is commonly used with a qualifier (e.g. congenital hemangioma)
ICD coding
  • ICD-O: 9120/0 - hemangioma, NOS
  • ICD-11: 2E81.0Y - other specified neoplastic hemangioma
Epidemiology
Sites
Pathophysiology
  • Specific pathogenesis has not been fully understood
  • Currently regarded as a multifactorial condition, resulting in endothelial proliferation, with uncontrolled angiogenesis and abnormal function of downstream pathways (notably HIF1α, VEGF and PI3K / Akt) (Biomed Res Int 2021;2021:5695378)
Etiology
  • Not known
Clinical features
Diagnosis
  • Requires correlation of detailed history and physical examination with microscopic features, as the latter overlaps for various clinical presentations
Laboratory
  • Transient mild to moderate thrombocytopenia due to consumption may be seen in rapid growth phase (F1000Res 2019;8:F1000)
Radiology description
  • Mostly required for extracutaneous cases
  • Skeletal (bone) hemangiomas: radiology is highly nonspecific (BMC Musculoskelet Disord 2021;22:27)
  • Ultrasound: highly effective; shows well defined mass with high vascularity
  • Contrast enhanced ultrasonography (CEUS): gold standard for hepatic hemangiomas (Korean J Radiol 2000;1:191)
  • High flow in proliferative phase of infantile hemangioma; slow flow in involuted phase
  • Central high flow for NICH; slow flow for RICH
  • Fluttering sign: recently described feature for grayscale ultrasound in hepatic hemangiomas (Ultrasound Med Biol 2021;47:941)
  • MRI: useful in deep locations to estimate extent of tissue involvement
  • Infantile hemangioma: hyperintense on T2; isointense on T1 with postcontrast enhancement
  • Congenital hemangioma: heterogeneous appearance on MRI
  • CT scan: hypodense, with postcontrast enhancement of periphery (World J Gastroenterol 2020;26:11)
Radiology images

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Hepatic hemangioma Hepatic hemangioma

Hepatic hemangioma

Intrathoracic hemangioma

Intrathoracic hemangioma

Infantile thyroid hemangioma

Infantile thyroid hemangioma

Infantile hepatic hemangioma

Infantile hepatic hemangioma

Choroidal hemangioma (fundoscopy)

Choroidal hemangioma (fundoscopy)


Hemangioma in long bones Hemangioma in long bones

Hemangioma in long bones

Hemangioma in long bones Hemangioma in long bones Hemangioma in long bones

Hemangioma in long bones

Prognostic factors
Case reports
Treatment
Clinical images

Contributed by Nasir Ud Din, M.B.B.S.
Noninvoluting congenital hemangioma

Noninvoluting congenital hemangioma



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Infantile hemangioma

Infantile hemangioma

Caption

Infantile hemangioma with ulceration

Infantile hemangioma in beard distribution

Infantile hemangioma in beard distribution

Rapidly involuting congenital hemangioma

Rapidly involuting congenital hemangioma

Partially involuting congenital hemangioma

Partially involuting congenital hemangioma

Laryngeal hemangioma (laryngoscopic view)

Laryngeal hemangioma (laryngoscopic view)

Gross description
Gross images

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Atrial hemangioma

Atrial hemangioma

Hepatic hemangioma

Hepatic hemangioma

Pulmonary hemangioma

Pulmonary hemangioma

Microscopic (histologic) description
  • Lobules of capillary sized vascular channels, lined by single layer of flattened endothelial cells
  • Large feeding vessel is usually seen at the deeper aspect
  • Associated lymphocyte infiltrate may be seen (Cardiovasc Pathol 2017;28:59)
  • Anastomosing hemangioma: anastomosing vascular channels lined by flattened endothelium; deep occurrence
  • Angiomatosis: involvement of multiple tissue planes with irregular and poorly circumscribed edges
  • Cavernous hemangioma: shows predominantly ectatic channels
  • Congenital hemangioma:
    • Solid appearance in rapid growth phase with poorly canalized vessels and mitotically active endothelium
    • Surrounding pericyte layer is present
    • With maturation, the lumina become prominent and blood flow ensues
    • Combination of solid and vascular areas in varying proportions may be seen
    • Noninvoluting congenital hemangioma (NICH) shows well formed capillaries and vascular channels
    • Involuting examples show thickening of basement membranes and fibrosis in the background
  • Epithelioid hemangioma:
    • Well formed small vessels are lined by plump endothelial cells with abundant eosinophilic cytoplasm and round enlarged nuclei, accompanied by abundant eosinophils in the background
    • Lobulated, well demarcated with maturation of vascular lumina at the periphery
  • Glomeruloid hemangioma: resembles glomerular capillaries
  • Hobnail hemangioma: hobnail nuclei protruding into vascular lumina; circumscribed
  • Infantile hemangioma:
    • Proliferation of capillary lobules; has a distinct natural history involving three stages (i.e., proliferation, partial regression and complete regression):
      • Early proliferative stage: lobules of immature dendritic type cells with intervening stroma, large feeding vessels and occasional presence of perineural involvement
      • Early regression: capillaries dilate and eventually start disappearing; apoptotic debris in basement membrane with increased numbers of pericapillary mast cells.
      • Late regression / end stage: ghosts of capillaries, rings of basement membrane with rare endothelial cells having immunophenotype of placental capillaries (GLUT1, LeY, CD15, CCR6, IDO and IGF2 positive)
  • Intramuscular angioma:
    • Arises within skeletal muscle in association with variable amount of mature adipose tissue, phleboliths and metaplastic bone formation
    • Shows a combination of lymphatics, variable sized veins and arteriovenous component
  • Lobular capillary hemangioma / pyogenic granuloma: ulceration with dense mixed inflammation
  • Microvenular hemangioma:
    • Poorly defined, in superficial and deep dermis
    • Small venule-like channels lined by single layer of endothelial cells which lack mitotic activity, surrounded by single layer of pericytes; these venules appear to dissect hyalinized collagen bundles of the dermis but lack multilayering and HHV8 positivity
  • Sinusoidal hemangioma:
    • Well demarcated proliferation of dilated, congested and thin vascular channels anastomosing in a sinusoidal manner
    • Intervening stroma is scant and scant smooth muscle may be present in the wall
    • Mitotic activity is not seen
  • Spindle cell hemangioma: proliferating spindle cells with intraluminal phleboliths
  • Verrucous hemangioma: hyperkeratosis and involvement of several tissue planes
Microscopic (histologic) images

Contributed by Nasir Ud Din, M.B.B.S.
Anastomosing hemangioma Anastomosing hemangioma

Anastomosing hemangioma

Skeletal hemangioma Skeletal hemangioma Skeletal hemangioma

Skeletal hemangioma

Cavernous hemangioma

Cavernous hemangioma


Hobnail hemangioma Hobnail hemangioma

Hobnail hemangioma

Hepatic hemangioma Hepatic hemangioma

Hepatic hemangioma

Infantile hemangioma Infantile hemangioma

Infantile hemangioma


Infantile hemangioma

Infantile hemangioma

Lobular capillary hemangioma Lobular capillary hemangioma Lobular capillary hemangioma

Lobular capillary hemangioma

Spindle cell hemangioma Spindle cell hemangioma

Spindle cell hemangioma


Spindle cell hemangioma

Spindle cell hemangioma

Phleboliths in spindle cell hemangioma

Phleboliths in spindle cell hemangioma

Synovial hemangioma

Synovial hemangioma

Verrucous hemangioma

Verrucous hemangioma

Virtual slides

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Extensive evolution

Extensive evolution

Involuting infantile hemangioma

Involuting infantile hemangioma

Congenital hemangioma

Congenital hemangioma

Cytology description
  • Aspiration is not advised, as there is high risk of uncontrolled bleeding
Positive stains
Negative stains
Electron microscopy description
  • Not required for diagnosis in routine cases
  • Endothelial cells show cytoplasmic folds on luminal surface, junctional complexes and cytoplasmic pinocytic vesicles (Cancer Res 1990;50:4787)
Electron microscopy images

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Endothelial differentiation

Endothelial differentiation

Cutaneous hemangioma

Cutaneous hemangioma

Molecular / cytogenetics description
Molecular / cytogenetics images

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Sanger sequencing for <i>RASA1</i>

Sanger sequencing for RASA1

Videos

Spindle cell hemangioma

Cutaneous vascular tumors

Sample pathology report
  • Liver, lobectomy:
    • Benign vascular lesion, consistent with hemangioma (see comment)
    • Comment: The tumor is completely excised with free margins. Evidence of embolization is seen, with presence of acellular material within the lumina of large caliber vessels, accompanied by areas of infarction within the lesion.
Differential diagnosis
  • Granulation tissue:
    • Sites of trauma, commonly cutaneous or mucosal
    • Shows associated inflammatory infiltrate and edema
    • Feeding vessels are not seen commonly
    • Lobular capillary hemangioma is common mimicker
    • Correlation with clinical history is important
  • Hemangioendothelioma:
    • Mimics proliferating phase of congenital hemangioma
    • Retiform type mimics hobnailing seen in proliferating phase of congenital hemangioma
    • Not well circumscribed
    • Clinical presentation with consumptive coagulopathy is common in both
    • May be associated with other anomalies
    • Requires more aggressive treatment
  • Kaposi sarcoma:
    • Mimics proliferating phase of congenital hemangioma due to compact cellularity and slit-like spaces
    • Eosinophilic hyaline globules are not seen in hemangioma
    • HHV8 association is not seen in hemangioma
    • Requires aggressive treatment
  • Angiosarcoma:
    • Malignant vascular tumor with infiltrative growth
    • Marked nuclear pleomorphism and brisk mitotic activity
    • Visceral location is more common compared to hemangioma
Board review style question #1

A newborn shows a nodular growth on his scalp. Over the next couple of weeks, the lesion starts shrinking. Despite reassurances from the pediatrician, the parents insist on excision. Upon pathological examination, it shows a network of capillary sized vessels with large feeding vessels near the base, shown in the above photomicrograph. The diagnosis in this case is

  1. Infantile hemangioma
  2. Noninvoluting hemangioma
  3. Partially involuting hemangioma
  4. Rapidly involuting hemangioma
Board review style answer #1
A. Infantile hemangioma. This case is an example of infantile hemangioma, which typically shows regression after birth, unlike congenital hemangioma, which grows with the patient, at least to some extent. Within the umbrella of congenital hemangioma, there is partial involution over time with shrinkage and secondary changes. Noninvoluting examples grow with the baby and rapidly involuting ones regress at a much faster rate, ultimately disappearing completely.

Comment Here

Reference: Hemangioma
Board review style question #2

A 9 year old boy presents with a purple nodular / bosselated lesion on the left leg, with involvement of the plantar and dorsal aspects of foot (as shown in the clinical photograph). He has had this lesion since birth and it has grown with him since then. The microscopic examination shows well formed vascular channels of variable size lined by a single layer of endothelial cells. The most likely diagnosis in this case is

  1. Birth mark
  2. Infantile hemangioma
  3. Kaposiform hemangioendothelioma
  4. Lobular capillary hemangioma
  5. Noninvoluting congenital hemangioma
Board review style answer #2
E. Noninvoluting congenital hemangioma. Noninvoluting congenital hemangioma is present at birth and grows with the child. As the name implies, there is no microscopic evidence of involution if a biopsy is performed. Birth marks commonly present with a flat, pigmented / reddish appearance of the skin rather than the appearance given in the clinical photograph, which shows prominent vascular marking, like the appearance of the lesion with some areas showing bosselation. Infantile hemangioma rapidly regresses after birth. Lobular capillary hemangioma presents as a nodular growth, commonly associated with ulceration of the skin or mucosa. Kaposiform hemangioendothelioma clinically presents commonly on the head and neck and trunk region, with a strong association with Kasabach-Merritt syndrome (consumptive coagulopathy). Microscopically, it shows glomeruloid proliferation and spindle cell morphology with slit-like spaces on microscopic examination.

Comment Here

Reference: Hemangioma
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