Non-melanocytic Skin Tumors
Moderator: Dr. Philip E. LeBoit
Case 5-3 -
Atypical post-radiation vascular lesion
Dr. Eduoardo Calonje
Female, 81. Pleomorphic lobular carcinoma of the left breast in November 1992. Treated with
radiotherapy. Ductal carcinoma of the right breast in October 2004. treated with wide excision. Now, an
irregular patch has developed on left breast. Two weeks after the biopsy, the lesion appears to have
regressed and no recurrence has been noticed after follow-up of three months.
Case 5-3 - Slide 1
- A punch biopsy showing part of a dermal vascular proliferation mainly occupying the superficial part
of the dermis
- The epidermis is normal
- In the papillary and superficial reticular dermis and extending focally into the mid and deep
reticular dermis there are irregular, thin-walled, lymphatic-like, congested vascular spaces lined by a
single layer of endothelial cells
- In the surrounding dermis there is a mild to focally moderate, mononuclear inflammatory cell
infiltrate composed of lymphocytes
- Tiny papillary projections are focally present in the lumina of vascular channels
- Endothelial cells lining the vascular channels are slightly prominent with focal hyperchromatism but
cytological atypia is mild
- A few endothelial cells have a hobnail-like appearance
- Multilayering is not seen and mitotic figures are not seen in endothelial cells
Diagnosis: Atypical post-radiation vascular lesion
Atypical post-radiation vascular lesion (AVL)
In the past AVL has been designed in the literature under many different names including lymphangioma
circumscriptum, benign lymphangiomatous papule and benign lymphangioendothelioma (acquired progressive
lymphangioma). AVL usually present a few years or months after radiotherapy for breast cancer. Although
post-irradiation angiosarcomas tend to present many years after radiotherapy this is not always the case
and the time span between radiotherapy and the appearance of a vascular lesion cannot be used as a
reliable guide to distinguish between a benign and a malignant lesion. Although AVL characteristically
arises in irradiated skin for the treatment of breast carcinoma, cases have been described after
radiotherapy for multiple myeloma and ovarian and endometrial carcinoma.
Clinical lesions are not distinctive and vary from macules to papules. There is a 10% local
recurrence rate and additional new lesions arise in 15% of patients. In the
series published so far with limited follow-up most cases have a benign behavior.
Rare examples of AVL have progressed to angiosarcoma and this suggests that AVL may form a
morphologic continuum with low-grade cutaneous post-radiation angiosarcoma. This emphasises the need for
complete surgical excision and close clinical surveillance.
Histologically, irregular lymphatic-like vascular channels lined by a single layer of endothelial are
seen in the dermis. They do not usually extend into the subcutaneous tissue. Often vascular channels
mainly occupy the upper dermis as illustrated in this case. Endothelial cells may be plump or have a
hobnail appearance and papillary projections may also be found. Cytological atypia, multilayering and
mitotic activity is usually absent. As mentioned before, in a minority of cases, distinction from a
well-differentiated angiosarcoma may be extremely difficult and it has been suggested that AVL and the
latter form part of a spectrum.
None of the entities described below except post-irradiation angiosarcoma, tend to occur in the field
of previous radiotherapy.
Hobnail (targetoid hemosiderotic) hemangioma
Hobnail hemangioma (HH) is an acquired vascular lesion that presents on the trunk or extremities of
young or middle-aged adults with male predilection. Occasional lesions present in the oral cavity. This
tumor was previously known as targetoid haemosiderotic haemangioma to highlight what was considered to be
its typical clinical presentation consisting of a small round lesion with concentric ecchymotic halos.
Many lesions however, do not show this typical clinical appearance and the alternative name of hobnail
hemangioma has been suggested. Lesions identical to hobnail hemangioma may occur after trauma.
Histologically, in the superficial dermis, there are irregular dilated thin-walled
vascular channels lined by bland endothelial cells with occasional papillary projections. The
endothelial cells in the superficial blood vessels typically show a hobnail appearance. The lesion has a
wedge-shaped architecture as a result of the vascular channels disappearing as they descend further into
the deeper dermis. Deeper vascular channels display flattened endothelial cells. The surrounding dermis
shows extravasation of red blood cells and often, quite prominent hemosiderin deposition. Inflammatory
cells are not usually seen.
Benign lymphangioendothelioma (acquired progressive lymphangioma)
This is a fairly rare tumor that presents predominantly in middle-aged or old adults. There is an
equal sex incidence and predilection for the extremities, particularly the lower limbs. The clinical
presentation is that of a solitary macular or plaque-like erythematous or purplish lesion that evolves
over a long period of time. Spontaneous regression is occasionally seen. Local recurrence after
excision is very rare.
Histologically, lesions show dilated irregular thin-walled vascular spaces, which tend to
be parallel to the epidermis. Most vascular channels are in the superficial and mid reticular dermis but
extension into the deep dermis and superficial subcutis can be seen. The endothelial cells in the
vascular channels are flat and there is little or no cytological atypia. Proteinaceous material or red
blood cells can be seen in the lumina. Inflammatory cells are not usually seen.
This tumour is relatively rare and most
cases arise in the breast. The time from radiotherapy to presentation varies from 5 to 20 years. There
is usually no associated lymphedema and the latency period is shorter than that in Stewart-Treves
syndrome. Some cases of post-irradiation angiosarcoma of the breast may be associated with chronic
lymphedema and this may contribute to the development of the disease.
Prognosis is poor. Local recurrences and rapid dissemination (lymph nodes and lungs) are frequent.
A mortality rate of up to 80% of cases has been reported. Features associated with adverse prognosis
include size of the tumor, depth of invasion and mitotic rate.
The histological appearances are very similar regardless of the clinical setting. Tumors
vary from well to poorly differentiated. Irregular channels lined by atypical endothelial cells with
hyperchromatism and frequent mitotic figures infiltrate the dermis and subcutaneous tissue.
Multilayering is frequent and this may be associated with papillary projections. Tumor cells line and
dissect collagen bundles and this produces a characteristic appearance described as "dissection of
collagen bundles". Hemorrhage and hemosiderin deposition is often seen. Focal epithelioid change is
present in some cases. Less differentiated tumors become more solid and vascular differentiation is
focal or absent. Pleomorphism becomes more prominent.
As mentioned in the discussion of AVL, in some cases distinction between the latter and angiosarcoma
is almost impossible and there is a suggestion that both entities may be part of a spectrum.
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