Dermatofibrosarcoma of the vulva
Introduction
Introduction to vulvar cutaneous sarcoma of vulva Vulvar dermatofibrosarcoma of the vulva is a low-to-moderate malignant fibrous histiocytoma with long course of disease, slow growth, limited lesions, easy recurrence after local resection, and rare metastasis. The incidence of the vulva is less, and it occurs in the trunk of the adult and the proximal end of the limb. basic knowledge The proportion of illness: 0.005% Susceptible people: good for adults Mode of infection: non-infectious Complications: skin cancer
Cause
Causes of vulvar dermatofibrosarcoma
(1) Causes of the disease
Vulvar progeny cutaneous fibrosarcoma originates from mesenchymal cells with multi-directional differentiation potential in the dermis, and some of the onset are related to trauma caused by local vulva, such as vaginal delivery.
(two) pathogenesis
The tumor is single or multiple skin and subcutaneous nodules, and the size can range from 1cm × 1cm to 8cm × 10cm. The recurrence is often multiple, the surface is covered with skin tension or ulceration, the boundary is clear, no capsule, recurrence is common. Mucus degeneration and soft texture, rare bleeding, necrosis.
Microscopic examination is mainly divided into three types: common type, mucus type and fibrosarcoma. The latter two are common in recurrence cases. The tumor cells are fusiform, like fibroblasts. The morphology is relatively uniform, often surrounding a center (collagen or small blood vessels). It is a typical car-like arrangement, with a low profile, and can be found in a few nuclear divisions, no necrosis, deep infiltration of the tumor in the dermis and subcutaneous, extending along the connective tissue between the subcutaneous fat lobules, wrapping into the subcutaneous fat, forming a lace-like or Honeycomb-like images, or images arranged in multiple layers in parallel, the car-like structure of the recurrent cases is not obvious, the mitotic figures are increased, the fibrosarcoma changes the cells more densely, the growth is bundled, and the abnormal shape is more and the nuclear division is more (average 11/ 10HPF), if mucus changes, the cells are loose and the blood vessels are exposed.
Prevention
Vulvar prostatic skin fibrosarcoma prevention
Regular physical examination, early detection, early reasonable surgical treatment, and good follow-up.
Complication
Vulvar dermatofibrosarcoma complications Complications skin cancer
The lesion site is infected.
Symptom
Vulvar dermatofibrosarcoma symptoms common symptoms radioactive pain nodules
Generally no symptoms, some have mild or moderate pain, can be broken after mild trauma, the common part is the labia majora, more on the left side, some may involve the clitoris or haze, mainly for progressive invasiveness It grows and invades the subcutaneous tissue. It is usually a painless plaque-like solid nodule. It can grow rapidly after several years, and it is a large irregular mass. The mass of the mass can be accompanied by local growth. Pain, tenderness and ulceration, the pain can be radiated to the inside of the thigh, and most patients come to see this doctor.
Physical examination revealed that the tumor usually has a single hair, which is characterized by a bulging hard solid mass. It can also be represented by a few small nodules around the central piece, fixed on the surface, covered with skin, deep and muscle, fascia is not sticky, nodules Red or light blue-purple, sometimes bony-like, easy to relapse when the resection is not clean.
Examine
Examination of vulvar dermatofibrosarcoma
Secretion examination, detection of COL1A1-PDGFB fusion protein, fresh tissue can be stained by dopa (DOPA) reaction or S-100 protein immunohistochemistry.
Histopathological examination.
Diagnosis
Diagnosis and differentiation of vulvar cutaneous sarcoma of vulva
According to the clinical manifestations of bulging, strong fibrous damage, slow growth, surface skin atrophy can make a diagnosis, pathological examination found dense fibroblasts arranged in a wheel-like structure to make a diagnosis.
It mainly needs to be differentiated from the invasive skin fibrotic tissue cell tumor. Usually the tissue section can be identified, but special staining is needed. The ligament-like tumor can originate and adhere to the deep fascia, the surface has normal skin, and the prominence skin fibrosarcoma The cell component is single, lacking secondary components such as giant cells, xanthomas, inflammatory cells, etc. seen in general skin fibrous histiocytoma, and the car-like arrangement is obvious. In addition, most skin fibroids do not express CD34, and 95% The cutaneous fibroma expresses FXIIIa, but the specificity of both is not high. Some pigment-free malignant melanomas need to be identified, but there are not many fusiform cells in a wheel-like structure, and the junctional activity is visible, and Producing reticular fibers and collagen, recently, Cribier et al (2002) found that ST3 protein is 100% expressed in cutaneous fibroids including giant cutaneous fibroids, and no protuberance cutaneous fibrosarcoma is expressed as an indicator of the difference between the two. The cutaneous fibroblastoma has no COL1A1-PDGFB fusion gene expression, so detection of the COL1A1-PDGFB fusion protein also helps to identify both.
