Puncture biopsy of the facial swelling was performed. The pathological results showed the areatus spindle cell proliferation with vascular fissure formation in the dermis, red blood cell overflow, and hemosiderin deposition, with infiltration of a few inflammatory cells, which accorded with KS. Immunohistochemical results were Platelet endothelial cell adhesion molecule-1 (+), endothelial cell marker (+), human herpesvirus 8 (HHV-8) (+), nuclcar-associated antigen (about 1%+) and Factor VIII (+) (Fig. 4).
And then, the patient began chemotherapy with the intravenous drip infusion of Doxorubicin Hydrochloride Liposome. The facial KS slightly decreased. One month later, the patient was hospitalized again for chemotherapy, without adverse reaction. The KS decreased significantly, the swelling was relieved, and the left eyelid could be opened (Fig. 5). The left eye movement was normal, the corneal reflex existed, and the left pupil was sensitive to light reflex.
KS is a multiple idiopathic hemorrhagic sarcoma.[10–11] The etiology of the disease is unknown and linked to genetic susceptibility, viral infections such as HHV-8,[13–15] angiogenic cell differentiation, immune deficiency such as AIDS, immune status, racial inheritance,[17–19] geographical location, and so on. KS is divided into 4 types: classical type, African local type, immunosuppressive type, and AIDS-related type. The classical KS progresses slowly, with a long survival period and relatively benign. Otherwise, the AIDS-related KS progresses rapidly, and has a high mortality. The lesions of classical KS are often composed of patches, plaques, and nodules, mainly at the end of the extremities (dorsum of hand and dorsum of foot and arch). The most common damage manifestations of AIDS-related KS are plaques and mild infiltrating plaques, mainly in a patient's trunk and head and face.[10,20] Children and young people are the groups at particular risk of the African-type KS, which often involved the internal organs with skin damage not commonly seen and is mainly manifested as the involvement of the lymph nodes.[10,20]
Skin is an important organ involved in KS, with early lesions manifested as small papules and gradually formation of patches or nodules of different sizes, with light red color, which gradually evolves to purplish red and purple black. The patches, plaques, and nodules of KS mainly affect the lower extremities, the face, the trunk, the genital, and the mucous membrane of the oropharynx. Compared with HIV-negative patients, dermal outer layers or visceral KS lesions are more common in HIV-infected people. KS has been reported in almost all anatomical sites, and with the emergence of HIV. AIDS-related KS in the head and neck has become one of the most common manifestations of AIDS. However, reports on the giant facial KS are rare.
The pathological basis of KS is very complex, but it is generally believed that the tumor originates from endothelial cells, most probably from blood vessels. In different pathological stages of the disease, the manifestations are also different. The early manifestations are chronic inflammation or granulomatous inflammation with formation of new vessels and lymphatic vessels, and expansion, edema and bleeding with the lymphocytes, plasma cells and mast cells infiltrating around the blood vessels.[24–25] The late manifestations are presented with the significant proliferation of endothelial cells, dermal spindle cells diffuse hyperplasia, extensive vasodilation congestion. And the sieve-like structures are formed, red blood cells overflow, and hemosiderin deposition. The nuclear mitotic figures can be seen. Inflammatory cells infiltrate around them, with necrosis and fibrosis seen.[24–25]
The imaging findings of facial KS in literatures are relatively rare. However, it has been reported in literatures that the KS distributes along the vessels of the infiltrated organs in the digestive system, with involved local lymph node swelling and uniformly increasing in density. The intestinal tract KS generally manifests as periodic and diffuse intestinal wall thickening with nodular enhancement, mesenteric lymph nodes enlargement and enhancement, suggesting that possible dissemination of KS.[26–27] MRI of liver KS shows high signal lesions infiltrate around the portal vein on T2WI, which, in the shape of grape clusters, are relatively characteristic. Liver and spleen enlarge, and the bigger lymph nodes are seen around the hepatic hilum and the celiac trunk. Pulmonary KS involves bronchovascular bundles, interlobular septa, pleura, and other interstitial tissues. Infiltration of adjacent lung parenchyma results in the formation of consolidation area of nodules or with indistinct boundary. CT shows the distribution along the bronchovascular bundles, most of which appear in bilateral symmetry, irregular flame-like nodule edges, and thickened ant leg-like interlobular septa. It may be accompanied with the masses enlarging and consolidation of the lungs, whereas the consolidation can be caused by tumor invasion, bleeding, or obstructive pneumonia.[29,30]
In this case, the pathological results showed that the areatus spindle cell proliferation with vascular fissure formation in the dermis, red blood cell overflow, and hemosiderin deposition, which presented as low signal intensity with a small amount of high signals in T1WI, T2WI, DWI, and SWI of MRI. Moreover, the MRI could provide the images of multiplane, and the invasion scope of the KS. In this case, the scope of the KS showed by MRI was larger than appearance seen by eyes. Therefore, the MRI could provide the accurate scope of the KS for the better diagnosis. It was regrettable that the follow-up MRI had not been done. But we observed that the MRI was sensitive to the facial KS, so we inferred that MRI may be helpful to the KS for follow-ups.
In conclusion, the case of the giant facial KS being simultaneously carried out by MRI is rare although skin is the common organ involved in KS. Moreover, for the better therapy and follow-up, MRI can not only make a diagnosis of KS, but also can accurately show the invasion range of tumor.
Data curation: Chun-Shuang Guan, Shuo Yan.
Formal analysis: Zhi-Bin Lv, Shuo Yan.
Investigation: Lei Sun.
Methodology: Ru-Ming Xie.
Resources: Chun-Shuang Guan, Xue-Mei Wang, Zhi-Bin Lv, Shuo Yan, Lei Sun.
Writing – original draft: Chun-Shuang Guan, Xue-Mei Wang.
Writing – review & editing: Chun-Shuang Guan, Ru-Ming Xie.
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Keywords:Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
HIV; Kaposi's sarcoma; magnetic resonance