Background
RH is known as a rare kind of low-grade malignant angiosarcoma with high rate of local recurrence and low potential of metastasis which is a sparsely distributed disease counting 35 cases (Table
1) whereas none of them is related to pleura or lung [
1]. Given the lacking information of pleural RH, its clinical manifestation, HRCT presentation and thoracoscopic appearance of a female patient are presented. The definitive diagnosis was made due to pathological observation and immunochemical staining. The prognosis is unpredictable since the tumor can affect multiple organs [
2]. Unfortunately, consensus has not been reached on if there is effective treatment to this tumor.
Table 1
Summary of clinical features of reported RH cases
Calonje (15) | 9–78/9 F,6 M | 6:lower limbs | ND | 1/15 | ND | ND |
4:upper limbs |
3:Trunk |
1:Penis |
1:Scalp |
Fukunaga (1) | 75/F | Lower limb | CD31, vimentin, UEA-1 (all), CD34, f-VIII (part) | 0/1 | ND | ND |
Duke (1) | 30/F | Upper limb, trunk | CD31, f-VIII | 0/1 | Excision | >10 years |
Samz-Trelles (1) | 11/M | Lower limb | Vimentin, f-VIII | 0/1 | Excision | >4 years |
Schommer (1) | 73/F | Trunk | CD31,UEA-1, f-VIII | 0/1 | Excision, RT, IT | >1 year |
Darouti (1) | 32/F | Lower limb | f-VIII | ND | ND | >1.5 years |
Tan (1) | 19/F | Lower limb | ND | 0/1 | Excision | >14 months |
Ioannidon (1) | 55/F | Upper limb | CD31 | 0/1 | Excision | >4 years |
Parson (1) | 17–71/4 F,0 M | 1:upper limb | CD31(4/4), | ND | ND | ND |
1:trunk | D2-40(1/4), |
1:head | VEGFR3(0/4) |
1:lower limb |
Bhutoria (1) | 35/F | Trunk | ND | Lymph node | Excision | ND |
Emberger (1) | 17/M | Trunk | CD31,D2-40 | 0/1 | Excision | >3 years |
Zhang (1) | 61/F | Head | CD34, f-VIII, vimentin | 0/1 | Excision(twice) | 6 months |
Aydıngöz (1) | 60/F | Lower limb | ND | 0/1 | Excision | >2 years |
Hirsh (1) | 44/M | Trunk | CD31 | 0/1 | Resection,chemoradiation | >36 months |
Keiler (1) | 11/F | Upper limb | ND | 0/1 | Excision | ND |
O’Duffy (1) | 18/M | Head | ND | Lymph node | Excision, RT | ND |
Albertini(1) | 6/F | Trunk | D2-40 | Lung | Excision | 6 months |
Choi (1) | 20/M | Upper limb | f-VIII | ND | Excision | ND |
Couceiro (1) | 50/F | Upper limb | ND | ND | Excision | >4 years |
Mota (1) | 26/F | Trunk | CD31, D2-40 | ND | Excision | ND |
Al-Faky | 9/F | Head | CD31, CD34, D2-40, f-VIII | ND | Excision | >6 years |
Discussion
Hemangioendothelioma (HE), an intermediate lesion between hemangioma and angiosarcoma, has been incipiently delineated as a kind of vascular tumor highlighted by its marginal biological features [
3]. It generally appears on skin or in soft tissues and extensively includes HE of epithelioid, kaposiform, retiform, composite and pseudomyogenic (also known as epithelioid sarcoma-like HE) subgroups and papillary intralymphatic angioendothelioma (also known as Dabska tumor). Malignancy of pseudomyogenic HE has recently been re-recognized [
4]. Definitive diagnosis of varied types of this tumor is based on histopathlological morphology and immunohistochemical markers [
5]. HE on pleura are limitedly documented as majority of them have been confirmed as epithelioid hemangioendothelioma (EH). Though EH favorably links to lung and pleura according to published cases, there has never been a description of its counterpart-retiform hemangioendothelioma on pleura.
Retiform hemangioendothelioma is a low-grade variant angiosarcoma which has been first introduced by Calonje in 1994 [
6]. Hyperchromatic nuclei without mitosis suggests its intermediate characters [
7] and indicates discrepant prognosis.
RH demonstrates a predilection for female adults [
6]. The majority of RH cases have been described as lesions on trunk or limbs [
8] whereas penis and scalp were occasionally involved [
9]. Features of RH’s lesion vary among individual patients expressing as masses, which is hyperhidrotic, or erosion [
8,
10,
11]. Exophytic pattern seems to be more prevailing [
8]. All the lesions in this patient were surrounded by “poor circumscription” as a presentation of its histological character.
Histological diagnosis lays the foundation of clinical diagnosis also plays the key role in differentiating RH from angiosarcoma and other subtypes of hemangioendothelioma. RH comprises of blood vessels ”weaving a net” by infiltrating into bundles of collagen. Endothelial cells fashion intraluminal papillea with scarce cytoplasm [
8,
12]. However, pathological sample in this case for histological examination may accidentally be a single component of “composite hemangioendothelioma” which contains retiform HE, epithelioid HE, angiosarcoma, spindle cell hemangioma, lymphangioma, arteriovenous malformation [
13] or part of these. This assumption is drawn from the fact that excision of gross sample has not be performed taking the fact that EH tends to form in lung into consideration [
14].
Histochemical markers of RH include CD31, D2-40, CD34, factor VIII-related antigen [
15,
16] among which CD34, factor VIII-related antigen have been consistently reported. CD34 and CD31 symbolize endothelial lineage and D2-40 expresses on other kinds of lymphatic hemangioendothelioma or hemangioma [
17,
18]. Controversy, nevertheless, has been aroused by Amy Parson's demonstration of 3 negative cases of D2-40 in 4 RH patients [
19].
Though histological and clinical features permits differential diagnosis between RH and other vascular tumors [
20], it shares a number of similarities with hobnail hemangioma which has been considered as it benign counterpart. However, hobnail hemangioma features shortage of papillae protruding into lumina and restricted location [
21] while prominent lymphatic infiltration and extensive lesion (dermis and subcutis) are widely applied to make the RH diagnosis [
22]. Moreover, definitive diagnosis of a papillary lymphatic angioendothelioma with partial “netlike” camouflage and D2-40 positivity was in arguement [
21].
The etiology of RH remains obscure. Latent relations between malignant medical history and RH should be elucidated [
2,
9]. In this case no evidence supports the internal link between RH occurence and the past radiotherapy.
No tumor-related [
8] deaths in RHs are observed under the circumstance of low metastasis. However, a locally invasive RH in China and a metastatic RH in a six-year-old girl turned out to be lethal [
23]. Although adjuvant immunotherapy and radiotherapy have been proved to be effective in several cases, none agreed tumor-responding treatments are recommended unless a complete surgical excision is performed [
24‐
27]. Amanda and her colleagues, however, proposed specific appearance on dermoscopy as practical assistance to diagnosis and therapy [
28].
Competing interests
The author(s) declare that they have no competing interests.
Authors’ contributions
QL collected the information and drafted the manuscript. RO attended the patient, arranged the clinical examination. PC participated in the medical decisions. RZ revised the manuscript. All authors read and approved the final manuscript.
Qingqing Liu, Email: lqq.aileen@outlook.com
Ruoyun Ouyang, Email: ruoyun001@126.com
Ping Chen, Email: pingchen0731@sina.com
Rui Zhou, Phone: +86-731-5292100, Email: zhourui2355@sina.com