Dermatology
BORDERLINE AND LOW-GRADE MALIGNANT VASCULAR NEOPLASMS
Built from Dermatology, 5th Edition

What’s inside
13 sections · 156 slides
Overview
- What this deck covers
- How pathologists sort vascular tumors
- A word on terms used throughout
Spindle Cell Hemangioma
A once-feared 'sarcoma' now understood to be a benign, thrombosis-prone vascular lesion
- Key features
- From 'sarcoma' to benign lesion
- Who gets it, and why
- Clinical appearance
- Spindle cell hemangioma: clinical appearance
- What the biopsy shows
- Spindle cell hemangioma: histopathology
- Spindle cell hemangioma: thrombosed and cellular zones
- Diagnosis and treatment
Kaposiform Hemangioendothelioma
An infiltrative infant tumor, its milder twin (tufted angioma), and the bleeding disorder both can trigger
- Key features
- A tumor once mistaken for infantile hemangioma
- What is Kasabach-Merritt phenomenon (KMP)?
- Why platelets get trapped
- The molecular picture
- Clinical presentation
- Kaposiform hemangioendothelioma with Kasabach–Merritt phenomenon
- Kaposiform hemangioendothelioma: large flank mass with Kasabach–Merritt phenomenon
- After treatment: three residual patterns
- What the biopsy shows
- Kaposiform hemangioendothelioma: histology
- Platelet trapping in kaposiform hemangioendothelioma (CD61 stain)
- Tufted angioma: the milder, shallower relative
- Tufted angioma: neonatal flank plaque
- Tufted angioma: thigh and knee plaque in an infant
- Distinguishing KHE from its mimics
- Treatment
Papillary Intralymphatic Angioendothelioma
The Dabska tumor: a rare childhood lesion built from lymphatic vessels lined by hobnail cells
- Key features
- From malignant to borderline
- Clinical picture
- Papillary intralymphatic angioendothelioma: histology
- Diagnosis and treatment
Retiform and Composite Hemangioendothelioma
A rete-testis-patterned tumor and its rare 'mixed' cousin, both driven by YAP1 gene fusions
- Key features
- Once thought to be a low-grade cancer
- The YAP1 gene fusion link
- Clinical course
- What the biopsy shows: RHE
- What the biopsy shows: CHE
- Diagnosis and treatment
Pseudomyogenic Hemangioendothelioma
A vascular tumor disguised as a muscle or epithelioid sarcoma, driven by FOSB gene fusions
- Key features
- A newly recognized entity
- Clinical presentation
- What the biopsy shows
- Diagnosis and treatment
Kaposi Sarcoma
One virus (HHV-8), four very different clinical faces - from an elderly man's ankle patch to fatal childhood disease
- Key features
- From an 1872 case series to a virus
- Four clinical variants, one virus
- Epidemiology of the four types
- Epidemiologic aspects of the four major types of Kaposi sarcoma (KS).
- Is it a cancer or an overgrowth reaction?
- How HHV-8 builds a Kaposi sarcoma lesion
- What drives growth once infected
- Classic KS: the original description
- Classic Kaposi sarcoma
- Kaposi sarcoma: violaceous plaque with superimposed papules
- African endemic KS: four different faces
- Iatrogenic KS: a drug-driven cancer
- AIDS-related KS: the most variable form
- Kaposi sarcoma in a patient with AIDS
- Pathology changes with the stage of the lesion
- Kaposi sarcoma: plaque-stage histology
- Kaposi sarcoma: patch-stage histology
- Kaposi sarcoma: nodular-stage histology and LANA-1
- The LANA-1 marker
- Telling KS apart from its mimics
- Treatment options for local disease
- Treatment for widespread or internal disease
Epithelioid Hemangioendothelioma
A true cancer of endothelial cells, but a far gentler one than angiosarcoma
- Key features
- Reclassified as a true cancer
- A defining gene fusion
- Clinical presentation
- ~30%
- What the biopsy shows
- Epithelioid hemangioendothelioma: histology
- Diagnosis and treatment
Angiosarcoma
The most aggressive vascular tumor of the skin - deceptively bruise-like at first, then rapidly lethal
- Key features
- Three historical descriptions, one disease
- Who gets it
- What drives the growth
- Other risk factors
- Where and how angiosarcoma arises
- Cutaneous angiosarcoma of the elderly
- Cutaneous angiosarcoma
- Angiosarcoma: ulcerated plaque with hemorrhage
- Lymphedema-associated and post-radiation forms
- Post-radiation angiosarcoma
- What the biopsy shows
- Angiosarcoma: histology
- Lymphatic-type and epithelioid variants
- Angiosarcoma with lymphatic features
- Angiosarcoma: c-MYC immunostain
- Angiosarcoma: ERG immunostain
- Diagnosis and prognosis
- Features of radiation-induced angiosarcoma of the breast (compared to post-radiation atypical vascular lesions of the skin).
- <1%
- Treatment
Glomus Tumors and Glomuvenous Malformations
Two disparate conditions that share one cell type: the glomus cell, a modified smooth muscle cell
- Key features
- Two different diseases, one shared cell
- Who gets each type
- Why the fingertip?
- The genetics of familial GVM
- Why a second genetic 'hit' is needed
- Glomus tumor: clinical picture
- Glomus tumor: subungual presentation
- MRI of a subungual glomus tumor
- Glomuvenous malformation: clinical picture
- Glomuvenous malformation: clustered papules
- Glomuvenous malformation: confluent plaque
- What the biopsy shows: glomus tumor
- Glomus tumor: histology
- When a glomus tumor turns malignant
- What the biopsy shows: GVM
- Glomuvenous malformation: histology
- Glomuvenous malformation: incomplete glomus cell rim
- Diagnosis and treatment
Infantile Hemangiopericytoma
A benign perivascular tumor of infancy that can look alarming yet often regresses on its own
- Key features
- Separated from the adult tumor of the same name
- Clinical presentation
- What the biopsy shows
- Diagnosis and treatment
Additional Figures From the Source
Figures from elsewhere in Bolognia's vascular tumor coverage, included here for completeness
- Where these figures fit
- Diffuse dermal angiomatosis: breast plaques
- Diffuse dermal angiomatosis: bulla and ulceration
- Targetoid hemosiderotic lymphatic malformation: target-like plaque
- Targetoid hemosiderotic lymphatic malformation: histology
- Pyogenic granuloma: nasal vestibule
- Pyogenic granuloma: lobular capillary pattern
- Pyogenic granuloma: exophytic nodule
- Pyogenic granuloma: lip papules
- Epithelioid hemangioma: scalp papulonodules
- Epithelioid hemangioma: vessel proliferation
- Epithelioid hemangioma: enlarged endothelial cells
- Genital angiokeratomas
Summary and References
The ten entities at a glance, key takeaways, and the source text's own reference list
- The ten entities, at a glance
- Malignant vs borderline: how they differ
- Take-home points
- References
- References
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- References
- References
- Dermatology, 5th Edition (2-Volume Set)