Dermatofibrosarcoma protuberans

Introduction to humeral cutaneous fibrosarcoma Dermatofibrosarcoma protuberans are derived from fibroblasts or histiocytic cells. They originate from tumors that grow slowly in the dermis. Later, most nodules occur on the plaque. The disease is locally invaded and occasionally spreads widely, but Rare transfer. The patient is usually a middle-aged person. The tumor can occur in any part of the body, but it is often found in the trunk and limbs, especially in the proximal part. It is distributed on the ventral side more than the dorsal side, and the proximal end is more than the distal end. Head and face and neck area and face. basic knowledge The proportion of illness: 0.0003%-0.0004% Susceptible people: no special people Mode of infection: non-infectious Complications: malignant melanoma

Causes of dermatofibrosarcoma

Cause (30%):

Recent studies have shown that tumor cells express Vim and CD34, and electron microscopic observation of tumor cells has the characteristics of both fibroblasts, as well as the characteristics of some nerve sheath cells. In some cases, dendritic cells containing melanin bodies are found, suggesting tumors. It is still possible to originate from nerve sheath cells.

Pathogenesis (10%):

The tumor is located in the dermis and subcutaneous fat, and is separated from the epidermis by a normal narrow zone. The tumor cells are fusiform, with uniform size and shape, less nuclear division, and arranged in a wheel shape. The tumor invades the subcutaneous fat to form a honeycomb. A special arrangement of mosaic or horizontal layering, which is the key to distinguishing CD41 positive tumors from other fibrous tissue tumors. If there are melanin-containing dendritic cells in the tumor area, they are Bednar tumors. Mucus changes, and special forms can occur. If the wheel-like arrangement disappears, the bundle-like arrangement is accompanied by an increase in nuclear division. When the proportion is more than 10%, it can be called fibrosarcoma from the dermatofibrosarcoma of the dermatological skin; Focal regional tumor cells may also be morphologically resembling smooth muscle cells and immunohistochemically positive for SMA and MSA, showing muscle-like and myofibroblastic differentiation; recently, there is also a region of giant cell fibroblastoma in the tumor, suggesting that giant cells Fibroblastoma may be a subtype of this tumor.

Protuberance cutaneous fibrosarcoma prevention

(1) The prognosis of fibrosarcoma depends on histological grade and age. The prognosis of children under 10 years of age is significantly better. The recurrence rate of children and adults is about the same, but the transfer is less, generally less than 10%.

(2) Maintain a good mentality and a healthy life rhythm, overcome bad eating habits and hobbies, and work regularly. Life is an effective way to prevent the occurrence of breast diseases.

(3) Supply protein foods that are easily digested and absorbed, such as milk, eggs, fish, soy products, etc., can improve the body's ability to fight cancer. Among them, milk and eggs can improve the disorder of protein after radiotherapy.

(4) Eat more foods with anti-cancer effects, such as turtle, mushroom, black fungus, garlic, seaweed, mustard and royal jelly.

Carinal cutaneous fibrosarcoma complications Complications malignant melanoma

Can be complicated by non-pigmented malignant melanoma.

Symptoms of dermatofibrosarcoma sarcoma Common symptoms Nodules, chronic illness, loss of severe pain

The patient is usually a middle-aged person. The tumor can occur in any part of the body, but it is often found in the trunk and limbs, especially in the proximal part. It is distributed on the ventral side more than the dorsal side, and the proximal end is more than the distal end. Head and face and neck area and face, palmar sputum is not tired, 10% to 20% of patients complained of a history of trauma before the onset, the course of the disease is slow and progressive, starting with a hard plaque on the skin, brown or dark red skin, leather concave It looks like atrophy, and the skin around the tumor is light blue-red. Later, reddish, dark red or purple-blue single nodules or adjacent multiple nodules of different sizes grow, showing a bulging appearance, ranging from 0.5 to 2 cm. And can suddenly accelerate growth and surface rupture, a few tumors see a bit of pigment, known as pigmented dermatoglycosmic sarcoma or Bednar tumor.

As the tumor grows, the pain is obvious. Without treatment, due to severe pain, contracture and long-term disease loss, the patient may suffer from general health failure. The disease is locally invaded, occasionally widely disseminated, but rarely metastasized.

Examination of dermatofibrosarcoma

Currently there are no related content description.

Histopathological examination, the tumor tissue is a well-differentiated fibrosarcoma, the tumor consists of large fusiform nucleus cells embedded in unequal amounts of collagen, usually arranged in irregular interweaving bands, swirling or wheel Arranged, a small number of vascular cavities, a small number of giant cells and tissue cells, damage can occur in the pigment-bearing cells, this damage mainly invaded the colored population, called Bednar tumor, some damage vimentin staining (vimentin) positive, CD34 is positive for this tumor and can be differentiated from cutaneous fibroids. S-100 protein is negative and can be differentiated from melanin.

Diagnosis and diagnosis of dermatofibrosarcoma

diagnosis

According to the clinical manifestations, the characteristics of skin lesions and the characteristics of histopathology can be diagnosed.

Differential diagnosis

It is distinguished from cutaneous fibroma and melanoma.

1. Skin fibroids: a benign tumor in the dermis caused by fibroblast or tissue cell focal hyperplasia. The disease can occur at any age, more common in young and middle-aged, more women than men. May occur naturally or after trauma. A brown or reddish intradermal papule or nodule is a clinical feature of the disease. The lesion grows slowly, persists for a long time, and rarely resolves itself.

Second, melanoma: is a malignant tumor with a very high degree of malignancy, also known as malignant melanoma, mostly originated in the skin, but also originated in the eyes, nasal cavity, etc., early metastasis can occur, metastatic sites are more common in the lungs, brain . Once diagnosed, extensive tumor resection should be performed as soon as possible, and comprehensive measures such as immunotherapy should be assisted.

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