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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

Clinical finding of this BL. (A, B) The patient suffered from chronic lymph edema of the affected left arm supervened shortly after the radical mastectomy with axillary lymph nodes dissection (A) and noticed multiple small and yellowish to reddish soft nodules (A, arrows), measuring up to 6 mm in the edematous left arm (B). The number of those lesions was gradually increasing up. Bar = 10 mm.
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Fig1: Clinical finding of this BL. (A, B) The patient suffered from chronic lymph edema of the affected left arm supervened shortly after the radical mastectomy with axillary lymph nodes dissection (A) and noticed multiple small and yellowish to reddish soft nodules (A, arrows), measuring up to 6 mm in the edematous left arm (B). The number of those lesions was gradually increasing up. Bar = 10 mm.

Mentions: She suffered from chronic lymphedema of the left arm supervened shortly after the operation (Figure 1A) and noticed multiple small and yellowish to reddish soft nodules, measuring up to 6 mm in the edematous left arm (Figure 1B). Since the number of those lesions was gradually increasing, a biopsy was performed. Moreover, interleukin-2 immunotherapy, additional radiotherapy (to the chest) and adjuvant chemotherapy using low-dose docetaxel were done for post-operative 7 years, under the initial diagnosis of low-grade angiosarcoma following longstanding lymphedema due to the radical mastectomy with axillary lymph nodes dissection for the breast carcinoma, i.e., the so-called Stewart-Treves syndrome, based on the initial clinicopathological features. Laboratory data, including blood cell count and chemistry, were within normal limits, except for modestly high level of hemoglobin A1c (HbA1c; 6.1 mg/dL), and there was no evidence of tumor or tumor-like lesions in the CT scannings of chest and abdomen. Anti-human immunodeficiency virus (HIV) antibodies were completely negative. Within the post-operative 7 years with additional treatment of interleukin-2 immunotherapy and chemo-radiotherapy, partial remission and neogenesis of the above small nodules were repeatedly seen. Very surprisingly, the patient had neither local invasion nor metastases of these lesions, respectively, and was alive and well at 5 years after the final chemotherapy. Furthermore, other skin lesions including those at the mastectomy site have never appeared.Figure 1


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)

Clinical finding of this BL. (A, B) The patient suffered from chronic lymph edema of the affected left arm supervened shortly after the radical mastectomy with axillary lymph nodes dissection (A) and noticed multiple small and yellowish to reddish soft nodules (A, arrows), measuring up to 6 mm in the edematous left arm (B). The number of those lesions was gradually increasing up. Bar = 10 mm.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig1: Clinical finding of this BL. (A, B) The patient suffered from chronic lymph edema of the affected left arm supervened shortly after the radical mastectomy with axillary lymph nodes dissection (A) and noticed multiple small and yellowish to reddish soft nodules (A, arrows), measuring up to 6 mm in the edematous left arm (B). The number of those lesions was gradually increasing up. Bar = 10 mm.
Mentions: She suffered from chronic lymphedema of the left arm supervened shortly after the operation (Figure 1A) and noticed multiple small and yellowish to reddish soft nodules, measuring up to 6 mm in the edematous left arm (Figure 1B). Since the number of those lesions was gradually increasing, a biopsy was performed. Moreover, interleukin-2 immunotherapy, additional radiotherapy (to the chest) and adjuvant chemotherapy using low-dose docetaxel were done for post-operative 7 years, under the initial diagnosis of low-grade angiosarcoma following longstanding lymphedema due to the radical mastectomy with axillary lymph nodes dissection for the breast carcinoma, i.e., the so-called Stewart-Treves syndrome, based on the initial clinicopathological features. Laboratory data, including blood cell count and chemistry, were within normal limits, except for modestly high level of hemoglobin A1c (HbA1c; 6.1 mg/dL), and there was no evidence of tumor or tumor-like lesions in the CT scannings of chest and abdomen. Anti-human immunodeficiency virus (HIV) antibodies were completely negative. Within the post-operative 7 years with additional treatment of interleukin-2 immunotherapy and chemo-radiotherapy, partial remission and neogenesis of the above small nodules were repeatedly seen. Very surprisingly, the patient had neither local invasion nor metastases of these lesions, respectively, and was alive and well at 5 years after the final chemotherapy. Furthermore, other skin lesions including those at the mastectomy site have never appeared.Figure 1

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