Overview of mesenteric neurofibrosarcoma
Mesenteric neurofibrosarcoma is a neurofibrosarcoma distributed in the mesentery, which is more common in young and middle-aged people, with no gender difference. The tumor may appear as a single nodule, oval, lobulated, sometimes multiple tumors are fused to form a large mass with clear borders, and a few can be seen with pseudo-envelope or incomplete encapsulation. The tumor is grayish-white in color, with many fibers in a woven structure, rich in cells, and the interstitial blood vessels may be fish-like, brittle, and may be accompanied by hemorrhage and necrosis. 40% of the cases are accompanied by neurofibromatosis.
Etiology
The etiology and pathogenesis of mesenteric neurofibrosarcoma are unknown.
Symptoms
Patients with mesenteric neurofibrosarcoma may present with abdominal pain, wasting, abdominal mass and gastrointestinal bleeding, which may be accompanied by systemic symptoms, such as hypoplasia, mental retardation and other malformations. When the tumor infiltrates adjacent bones, periosteal reaction may occur, even local bone destruction. Tumor infiltration to the surface can be ulcerated and infected, and hemorrhage can occur after ulceration. Lymph node metastasis and hematogenous metastasis may occur in advanced stage.
Examination
Ultrasonography and computed tomography (CT) can show the morphology of the mass and its liquefied necrotic area.
Diagnosis
Combined with medical history and clinical manifestations, imaging studies are helpful in determining the nature of the lesion. Barium contrast can show the relationship between the lesion and the intestinal canal and the destruction of the mucosa, but it cannot show the specific morphology of the mass. Ultrasound and CT can show the morphology of the mass and its liquefied necrotic area. The final diagnosis still depends on surgical and pathologic confirmation.
Differential diagnosis
1. Small bowel cancer
Symmetrical circular narrowing of intestinal canal can be seen on imaging, with dilatation of intestinal canal at the proximal end of the narrowing, and filling defects can be seen in the lumen of the canal.
2. Lymphosarcoma of small intestine
Single or multiple polypoid filling defects in the intestinal lumen, extensive destruction of mucosa and widening of the lumen can be seen on imaging, and CT shows thickening of the intestinal wall, extra-luminal soft tissue mass and extensive lymph node enlargement in the abdominal cavity.
3.Smooth muscle sarcoma
On contrast, there is a smooth filling defect in the intestinal lumen, with ulcers and fistulae in the mass, but the mucosa is relatively intact.CT shows a huge mass outside the lumen, with low-density necrotic areas and scattered displacement of the intestinal tubes.
4. Fibrosarcoma
The cells are spindle-shaped, intertwined and arranged, with obvious heterogeneity, but without nuclei, fenestrated arrangement, the tumor has no connection with the nerve trunk.
Treatment
Wide excision is the main treatment method. Some of them are closely related to the main blood vessels of the tethered membrane and cannot be cured radically. For those occurring in the pelvis and paravertebral area which cannot be widely resected or incompletely resected can be supplemented with radiation therapy, but the effect is not good, and it is prone to skin ulceration, compression of blood vessels, and metastasis. Chemotherapy is effective for tumors with metastases, and commonly used drugs include adriamycin, vincristine, cyclophosphamide, and so on.