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Infraorbital cutaneous angiosarcoma: a diagnostic and therapeutic dilemma.

Ettl T, Kleinheinz J, Mehrotra R, Schwarz S, Reichert TE, Driemel O - Head Face Med (2008)

Bottom Line: This case presents a 64-year-old man with a six-month-history of a recurrent diffuse and erythematous painless swelling below the left eye.Neither, finally achieved negative margins on permanent sections, nor a following chemotherapy could prevent the recurrence of the disease after five months and the patient's dead 21 months after the first diagnosis.The case elucidates the current diagnostic and therapeutic dilemma of this entity, which shows an unfavourable clinical course in spite of multimodal therapy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Oral and Maxillofacial Surgery, Muenster University, Germany. Johannes.Kleinheinz@ukmuenster.de

ABSTRACT

Background: A cutaneous angiosarcoma is a rare malignant tumour of vascular endothelial cells with aggressive clinical behaviour and poor prognosis. Diagnosis is often delayed due to its variable and often benign clinical appearance.

Case presentation: This case presents a 64-year-old man with a six-month-history of a recurrent diffuse and erythematous painless swelling below the left eye. Several resections with intraoperatively negative resection margins followed, but positive margins were repeatedly detected later on permanent sections. Histopathologic examination of the specimen diagnosed a cutaneous angiosarcoma. Neither, finally achieved negative margins on permanent sections, nor a following chemotherapy could prevent the recurrence of the disease after five months and the patient's dead 21 months after the first diagnosis.

Conclusion: The case elucidates the current diagnostic and therapeutic dilemma of this entity, which shows an unfavourable clinical course in spite of multimodal therapy.

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Clinical appearance after first incisional biopsy: Discreet skin erythema below the left eye.
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Figure 1: Clinical appearance after first incisional biopsy: Discreet skin erythema below the left eye.

Mentions: A 64-year-old man presented with a six month history of a recurrent diffuse and erythematous painless swelling (3 × 2 cm2) below the left eye to the Department of Dermatology, Regensburg University, Germany. Cervical lymphadenopathy was clinically not detectable. Routine laboratory results showed no abnormality. Presuming an allergic dermatitis, topical treatment with steroids was initiated. Because of the persistence of the lesion, an incisional biopsy was performed three weeks later (Figure 1). Histopathology of the specimen showed an invasively growing tumour of the dermis, composed of atypical vascular endothelia in a disordered manner, forming bizarre vascular lumina. The tumor cells were characterized by an elevated proliferated activity with a proliferation fraction (MIB-1) of 5%–10%. The vascular endothelial proliferation showed a papillary architecture accompanied by small lymphocytes. The majority of endothelial cells presented a hyperchromatic nucleus and a swollen cytoplasm. (Figure 2a, 2b, 2c). Immunohistochemical studies demonstrated positivity for CD 31 (Figure 2d) and factor VIII-related antigen. Based on these findings the diagnosis of a cutaneous angiosarcoma was made.


Infraorbital cutaneous angiosarcoma: a diagnostic and therapeutic dilemma.

Ettl T, Kleinheinz J, Mehrotra R, Schwarz S, Reichert TE, Driemel O - Head Face Med (2008)

Clinical appearance after first incisional biopsy: Discreet skin erythema below the left eye.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2533304&req=5

Figure 1: Clinical appearance after first incisional biopsy: Discreet skin erythema below the left eye.
Mentions: A 64-year-old man presented with a six month history of a recurrent diffuse and erythematous painless swelling (3 × 2 cm2) below the left eye to the Department of Dermatology, Regensburg University, Germany. Cervical lymphadenopathy was clinically not detectable. Routine laboratory results showed no abnormality. Presuming an allergic dermatitis, topical treatment with steroids was initiated. Because of the persistence of the lesion, an incisional biopsy was performed three weeks later (Figure 1). Histopathology of the specimen showed an invasively growing tumour of the dermis, composed of atypical vascular endothelia in a disordered manner, forming bizarre vascular lumina. The tumor cells were characterized by an elevated proliferated activity with a proliferation fraction (MIB-1) of 5%–10%. The vascular endothelial proliferation showed a papillary architecture accompanied by small lymphocytes. The majority of endothelial cells presented a hyperchromatic nucleus and a swollen cytoplasm. (Figure 2a, 2b, 2c). Immunohistochemical studies demonstrated positivity for CD 31 (Figure 2d) and factor VIII-related antigen. Based on these findings the diagnosis of a cutaneous angiosarcoma was made.

Bottom Line: This case presents a 64-year-old man with a six-month-history of a recurrent diffuse and erythematous painless swelling below the left eye.Neither, finally achieved negative margins on permanent sections, nor a following chemotherapy could prevent the recurrence of the disease after five months and the patient's dead 21 months after the first diagnosis.The case elucidates the current diagnostic and therapeutic dilemma of this entity, which shows an unfavourable clinical course in spite of multimodal therapy.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Oral and Maxillofacial Surgery, Muenster University, Germany. Johannes.Kleinheinz@ukmuenster.de

ABSTRACT

Background: A cutaneous angiosarcoma is a rare malignant tumour of vascular endothelial cells with aggressive clinical behaviour and poor prognosis. Diagnosis is often delayed due to its variable and often benign clinical appearance.

Case presentation: This case presents a 64-year-old man with a six-month-history of a recurrent diffuse and erythematous painless swelling below the left eye. Several resections with intraoperatively negative resection margins followed, but positive margins were repeatedly detected later on permanent sections. Histopathologic examination of the specimen diagnosed a cutaneous angiosarcoma. Neither, finally achieved negative margins on permanent sections, nor a following chemotherapy could prevent the recurrence of the disease after five months and the patient's dead 21 months after the first diagnosis.

Conclusion: The case elucidates the current diagnostic and therapeutic dilemma of this entity, which shows an unfavourable clinical course in spite of multimodal therapy.

Show MeSH
Related in: MedlinePlus