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Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome.

Yamada S, Yamada Y, Kobayashi M, Hino R, Nawata A, Noguchi H, Nakamura M, Nakayama T - Diagn Pathol (2014)

Bottom Line: Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well.Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%.Therefore, we finally made a diagnosis of BL of the skin.

View Article: PubMed Central - PubMed

Affiliation: Departments of Pathology and Cell Biology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-8555, Japan. sousuke@med.uoeh-u.ac.jp.

ABSTRACT
Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. The biopsy specimen showed an ill-defined lesion composed of a proliferation of irregular and sometimes anastomosing vascular structures in the dermis, lined by endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no mitotic figures. As the lesion grew within deeper dermis, these proliferating vessels dissected dermal collagenous bands, occasionally arranged in low-papillary projections and/or characteristic hobnail cytomorphology. We first interpreted it as low-grade angiosarcoma following chronic lymphedema due to the operation, i.e., the so-called Stewart-Treves syndrome. Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well. Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%. Therefore, we finally made a diagnosis of BL of the skin. MIB-1 labeling index might be useful and adjunctive aids for reaching the correct diagnosis of cutaneous BL, especially in case of small or inadequate specimens.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_197.

No MeSH data available.


Related in: MedlinePlus

Immunohistochemical examination of the first biopsy specimen. (A) These modestly atypical endothelial cells were positive for CD31, but strongly positive for LYVE-1. (B) The tumor cells showed a much lower MIB-1 labeling index, less than 1%. Bars =50 μm.
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Fig3: Immunohistochemical examination of the first biopsy specimen. (A) These modestly atypical endothelial cells were positive for CD31, but strongly positive for LYVE-1. (B) The tumor cells showed a much lower MIB-1 labeling index, less than 1%. Bars =50 μm.

Mentions: The first small biopsy specimen (Figure 2A) showed an ill-defined lesion predominantly composed of a significant proliferation of irregular and sometimes anastomosing vascular structures containing no red blood cells mostly in the middle to lower layer of dermis (tumor: 24.5 ± 2.9 per 1 high power fields vs. non-tumor: 2.7 ± 0.4 per 1 high power fields; P <0.0001) (Figure 2A). Few lesions involve the superficial dermis, and extension into the subcutaneous fat was absent (Figure 2A). These vascular channels were lined by modestly atypical endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no apparent mitotic figures (Figure 2B). As the lesion grew within deeper dermis, these proliferating vascular channels dissected dermal collagenous bundles, occasionally arranged in low-papillary projections and/or characteristic hobnail or multi-layered cytomorphology (Figure 2B). Surrounding lymphocytic infiltrate was not evident. On the other hand, the covering epidermis exhibited mild acanthosis and modestly elongated thickened rete ridge without any evidence of atypical changes (Figure 2A). Based on these features, we first diagnosed it as low-grade angiosarcoma following chronic lymphedema due to the radical mastectomy, i.e., the so-called Stewart-Treves syndrome. However, the follow-up biopsy specimens from the affected left arm demonstrated no evidence of any remarkable changes on histopathological findings, very similar to those of the above first biopsy sample. Immunohistochemically, these proliferating endothelial cells were positive for CD31 (DAKO, diluted 1:20) (Figure 3A), factor VIII-related antigen (DAKO, diluted 1:30), CD34 (IMMUNOTECH, Marseille, France, diluted 1:50), and Podoplanin (D2-40; Nichirei Bioscience Co., Tokyo, Japan, diluted 1:1) on the first biopsy specimen. Furthermore, the tumor cells of both the first and follow-up biopsy specimens showed strong immunohistochemical expression of lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 (LYVE-1; R&D Systems, Inc., Minneapolis, MN, USA, diluted 1:320) (Figure 3A) and lower MIB-1 (Ki67; DAKO, diluted 1:50) labeling index, less than 1% (0.3%) (Figure 3B). There was no immunohistochemical expression of human herpes virus (HHV)-8 (HHV-8; Santa Cruz Biotechnology, Santa Cruz, CA, USA, diluted 1:100). Therefore, we confirmed that these mildly proliferating endothelial cells were derived from lymphatic vessels, and finally made a diagnosis of post-mastectomy BL of the skin following chronic lymphedema, based on the clinicopathological findings including its unusually benign clinical course.Figure 2


Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome.

Yamada S, Yamada Y, Kobayashi M, Hino R, Nawata A, Noguchi H, Nakamura M, Nakayama T - Diagn Pathol (2014)

Immunohistochemical examination of the first biopsy specimen. (A) These modestly atypical endothelial cells were positive for CD31, but strongly positive for LYVE-1. (B) The tumor cells showed a much lower MIB-1 labeling index, less than 1%. Bars =50 μm.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4215009&req=5

Fig3: Immunohistochemical examination of the first biopsy specimen. (A) These modestly atypical endothelial cells were positive for CD31, but strongly positive for LYVE-1. (B) The tumor cells showed a much lower MIB-1 labeling index, less than 1%. Bars =50 μm.
Mentions: The first small biopsy specimen (Figure 2A) showed an ill-defined lesion predominantly composed of a significant proliferation of irregular and sometimes anastomosing vascular structures containing no red blood cells mostly in the middle to lower layer of dermis (tumor: 24.5 ± 2.9 per 1 high power fields vs. non-tumor: 2.7 ± 0.4 per 1 high power fields; P <0.0001) (Figure 2A). Few lesions involve the superficial dermis, and extension into the subcutaneous fat was absent (Figure 2A). These vascular channels were lined by modestly atypical endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no apparent mitotic figures (Figure 2B). As the lesion grew within deeper dermis, these proliferating vascular channels dissected dermal collagenous bundles, occasionally arranged in low-papillary projections and/or characteristic hobnail or multi-layered cytomorphology (Figure 2B). Surrounding lymphocytic infiltrate was not evident. On the other hand, the covering epidermis exhibited mild acanthosis and modestly elongated thickened rete ridge without any evidence of atypical changes (Figure 2A). Based on these features, we first diagnosed it as low-grade angiosarcoma following chronic lymphedema due to the radical mastectomy, i.e., the so-called Stewart-Treves syndrome. However, the follow-up biopsy specimens from the affected left arm demonstrated no evidence of any remarkable changes on histopathological findings, very similar to those of the above first biopsy sample. Immunohistochemically, these proliferating endothelial cells were positive for CD31 (DAKO, diluted 1:20) (Figure 3A), factor VIII-related antigen (DAKO, diluted 1:30), CD34 (IMMUNOTECH, Marseille, France, diluted 1:50), and Podoplanin (D2-40; Nichirei Bioscience Co., Tokyo, Japan, diluted 1:1) on the first biopsy specimen. Furthermore, the tumor cells of both the first and follow-up biopsy specimens showed strong immunohistochemical expression of lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 (LYVE-1; R&D Systems, Inc., Minneapolis, MN, USA, diluted 1:320) (Figure 3A) and lower MIB-1 (Ki67; DAKO, diluted 1:50) labeling index, less than 1% (0.3%) (Figure 3B). There was no immunohistochemical expression of human herpes virus (HHV)-8 (HHV-8; Santa Cruz Biotechnology, Santa Cruz, CA, USA, diluted 1:100). Therefore, we confirmed that these mildly proliferating endothelial cells were derived from lymphatic vessels, and finally made a diagnosis of post-mastectomy BL of the skin following chronic lymphedema, based on the clinicopathological findings including its unusually benign clinical course.Figure 2

Bottom Line: Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well.Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%.Therefore, we finally made a diagnosis of BL of the skin.

View Article: PubMed Central - PubMed

Affiliation: Departments of Pathology and Cell Biology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu 807-8555, Japan. sousuke@med.uoeh-u.ac.jp.

ABSTRACT
Benign lymphangioendothelioma (BL) represents a very rare lymphatic vascular proliferation. Our aim is to be aware that owing to its characteristic features, pathologists can easily misinterpret it as cutaneous low-grade angiosarcoma when examining only small specimens. In the present case, multiple small and yellowish to reddish soft nodules were noticed in the edematous left arm of a 54-year-old Japanese female 4 years after the radical mastectomy with axillary lymph nodes dissection and following radiotherapy to the chest for the left breast carcinoma. The biopsy specimen showed an ill-defined lesion composed of a proliferation of irregular and sometimes anastomosing vascular structures in the dermis, lined by endothelial cells having mildly hyperchromatic and pleomorphic nuclei, but no mitotic figures. As the lesion grew within deeper dermis, these proliferating vessels dissected dermal collagenous bands, occasionally arranged in low-papillary projections and/or characteristic hobnail cytomorphology. We first interpreted it as low-grade angiosarcoma following chronic lymphedema due to the operation, i.e., the so-called Stewart-Treves syndrome. Although additional treatments were performed for 7 years, she had neither local invasion nor metastases of these tumors, respectively, and was alive and well. Retrospective immunohistochemical findings demonstrated that these mildly atypical endothelial cells were strongly positive for lymphatic vessel endothelial hyaluronan receptor (LYVE)-1 as well, and MIB-1 labeling index was less than 1%. Therefore, we finally made a diagnosis of BL of the skin. MIB-1 labeling index might be useful and adjunctive aids for reaching the correct diagnosis of cutaneous BL, especially in case of small or inadequate specimens.Virtual Slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/13000_2014_197.

No MeSH data available.


Related in: MedlinePlus