BACKGROUND: Composite haemangioendothelioma is a rare vascular neoplasm with indolent to intermediate malignant potential. Diagnosis of this disease relays on histopathological identification of at least two different morphologically distinctive vascular components in proper clinical settings. Exceedingly rare cases of this neoplasm can exhibit areas resembling high-grade angiosarcoma, which does not change the biological behaviour. Such lesions tend to occur in the setting of chronic lymphoedema and thus, can mimic Stewart-Treves syndrome, which has a much worse clinical outcome and prognosis. CASE PRESENTATION: We present a case of 49 years old male suffering from chronic lymphoedema of the left lower extremity who had developed a composite haemangioendothelioma with high grade angiosarcoma-like areas mimicking the Stewart-Treves syndrome. Given the multifocality of the disease, the only potentially curable surgical treatment would be hemipelvectomy, which was refused by the patient. The patient has been followed-up, with no signs of local progression of the remaining disease, nor a distant spread outside the involved extremity for two years. CONCLUSIONS: Composite haemangioendothelioma represents a rare malignant vascular tumour, with significantly more favourable biological behaviour than angiosarcoma, even in cases where angiosarcoma-like areas are present. For that reason, composite haemangioendothelioma can be easily misdiagnosed as true angiosarcoma. The rarity of this disease unfortunately hampers the development of clinical practice guidelines and the implementation of treatment recommendations. Most of the patients with localized tumour are treated by wide surgical resection, without neo- or adjuvant radiotherapy or chemotherapy. However, in the case of this diagnosis, the watch-and-wait approach is better than mutilating procedure, highlighting the necessity of establishing of the correct diagnosis.

• Klíčová slova
• Angiosarcoma, Angiosarcoma-like areas, Case report, Composite haemangioendothelioma, Congenital lymphoedema, Stewart-Treves syndrome, Vascular neoplasm,
• MeSH
• hemangioendoteliom * diagnóza   MeSH
• hemangiosarkom * patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• lymfangiosarkom * diagnóza   etiologie   patologie   MeSH
• lymfedém * diagnóza   etiologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• mužské pohlaví MeSH
• Publikační typ
• časopisecké články MeSH
• kazuistiky MeSH

Lymphangiosarcoma, or Stewart-Treves Syndrome (STS), is a very rare skin angiosarcoma with poor prognosis, which usually affects the upper limbs of patients who underwent breast cancer surgery, including axillary dissection followed by radiotherapy (RT). Cutaneous lymphangiosarcomas, which account for approximately 5% of all angiosarcomas, usually originate in the limb with chronic lymphedema. Lymphatic blockade is involved in the onset of STS. RT contributes indirectly to an increased risk of developing STS by causing axillary-node sclerosis and resulting in a lymphatic blockade and lymphedema. Chronic lymphedema causes local immunodeficiency, which indirectly leads to oncogenesis. Currently, axillary nodes are no longer routinely irradiated after axillary dissection, which is associated with a reduction in the incidence of chronic lymphedema from 40% to 4%. The use of sentinel lymph node biopsy technique is also widespread and the associated risk of lymphedema is further reduced. Thus, the incidence of STS decreased significantly with improved surgical and radiation techniques. The overall prognosis of STS patients is very poor. Only early radical surgical removal, including amputation or disarticulation of the affected limb, or wide excision at an early stage offers the greatest chance of long-term survival. Only a few case reports and series with a small number of patients with lymphangiosarcoma can be found in the literature. We present a case report of the first diagnosed STS at our department in an effort to highlight the need of the consideration of developing lymphangiosarcoma in patients with chronic lymphedema.

• Klíčová slova
• Angiosarcoma, Breast cancer, Lymphangiosarcoma, Radiotherapy, Stewart-Treves syndrome,
• Publikační typ
• časopisecké články MeSH

Breast angiosarcomas (AS) are very rare neoplasms, which can be divided into primary (or sporadic), and secondary AS, the latter arising either on the base of lymphoedema after mastectomy (so called AS with Stewart-Treves syndrome- ASSTS), or skin AS after breast conservation surgery with subsequent radiotherapy for breast cancer (KPRAS). The authors present a case of a 55-year-old female patient with a 17mm tumour in nipple region, developing 8 years after primary diagnosis of tubulolobular carcinoma of the breast which was treated by breast conservation surgery and radiotherapy. A probatory bioptic examination of the lesion proved well-differentiated KPRAS. The patient underwent mastectomy. The authors analyse the basic clinical and morphological features of KPRAS, which distinguish it from other forms of AS, e.g. occurrence in older age, shorter period of latency after radiation therapy when compared to ASSTS, absence of lymphoedema, and rare involvement of the breast tissue. Prognosis of this entity is very poor; today the most reliable prognostic marker is histological grading. However, it will be needed to assess in the future new indicators of prognosis of patients with this rare disease.

• MeSH
• hemangiosarkom etiologie   patologie   MeSH
• lidé středního věku MeSH
• lidé MeSH
• nádory kůže etiologie   patologie   MeSH
• nádory prsu radioterapie   chirurgie   MeSH
• nádory vyvolané zářením patologie   MeSH
• prsy MeSH
• segmentální mastektomie MeSH
• sekundární malignity patologie   MeSH
• Check Tag
• lidé středního věku MeSH
• lidé MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH

BACKGROUNDS: The cancerogenic effect of ionizing radiation was documented just several years after it started to be used as a treatment option. Ionizing radiation produces a small but detectable risk of carcinoma as well as bone and soft tissue sarcomas. Over the past 20 years angiosarcomas arising from the area of the irradiated breast have been reported with increasing frequency as the number of women undergoing the breast conserving surgery with consecutive radiotherapy has increased also. Angiosarcomas can originate from either lymphatic or capillary endothelium, namely lymphangiosarcomas and haemangiosarcomas. The most of haemangiosarcomas arising from the breast skin developed in the irradiated area after breast conserving procedure--secondary angiosarcomas. Lymphangiosarcoma is typically associated with longstanding extremity lymphedema--Stewart-Treves syndrome. CASES: We report three cases of angiosarcomas which occured in this region after breast conserving treatment and we also review the literature. CONCLUSION: Paradoxically, the decrease in the use of radiotherapy to the post-mastectomy chest wall and the axillary area is expected to reduce the incidence of angiosarcomas, while the increase in the use of breast conserving procedure plus radiotherapy could lead to increased incidence of angiosarcomas in the residual breast tissue. Special attention should be paid to skin leasions and changes occuring after breast conserving treatment and especially to the ones with the skinthickening. The early detection and diagnosis has the crucial prognostic value.

• MeSH
• hemangiosarkom etiologie   MeSH
• lidé MeSH
• nádory kůže etiologie   MeSH
• nádory prsu radioterapie   chirurgie   MeSH
• nádory vyvolané zářením patologie   MeSH
• prsy * MeSH
• segmentální mastektomie MeSH
• sekundární malignity etiologie   MeSH
• senioři MeSH
• Check Tag
• lidé MeSH
• senioři MeSH
• ženské pohlaví MeSH
• Publikační typ
• anglický abstrakt MeSH
• časopisecké články MeSH
• kazuistiky MeSH