Scrotal nerve sheath tumor is an extremely rare and special type of scrotal tumor, which has rarely been reported clinically and has no symptoms other than the presence of masses or nodules. In China, it was first reported by Deng Shaojiang in 1998 [2], and since then there have been several cases in China. The data of a patient with scrotal nerve sheath tumor in our hospital are reported as follows, and discussed with the relevant literature to improve the clinical understanding of scrotal nerve sheath tumor. Case report Patient, male, 36 years old. He was admitted to the hospital with the main reason of “scrotal swelling found for more than 10 years”. The patient unintentionally discovered a soy-sized subcutaneous scrotal mass without tenderness 10 years ago and was not treated. In the past 3 years, the swelling gradually increased in size. On examination: a round swelling of about 2 cm in diameter was found under the skin at the bottom of the scrotum on the left side, with a tough texture, smooth surface, negative tenderness and no obvious adhesion to the surrounding tissues. Ultrasonography: subcutaneous scrotal mass with clear border, regular outline, uniform echogenicity, size 2.0cm×2.5cm×2.5cm, rich blood supply. Ultrasound examination of the urinary tract, abdomen and pelvis showed no abnormality, chest X-ray showed no abnormality, and other laboratory tests showed no significant abnormality. Preoperative diagnosis: solid scrotal occupancy, nature to be investigated. Scrotal mass excision was performed under local anesthesia. The scrotal mass was located under the scrotal skin, with intact envelope, hard texture, rich vascularity on the surface of the tumor, clear boundary with the surrounding tissues, and no association with the testes and epididymis. Pathological findings: a grayish white nodular mass with a diameter of about 2 cm, grayish white and grayish red on the cut surface, medium texture; microscopically, the mass was seen to be spindle-shaped cells, arranged in a fenestrated pattern, with rod-shaped nucleus and translucent cytoplasm. Pathological diagnosis: scrotal subcutaneous nerve sheath tumor. The incision healed well after surgery, and there was no local recurrence at the 3-month follow-up, and there was no abnormality in the urinary tract and abdominal ultrasonography. After searching Wanfang and CNKI, we obtained 10 related papers (including 3 reports of scrotum-related nerve sheath tumors and 7 reports of scrotal nerve sheath tumors), and a total of 8 cases of scrotal nerve sheath tumors were reported in China. Among them, 7 cases with more complete information [3-11] were summarized and analyzed with the clinical data of the total 8 cases in this case. General data: all were male, age 34-78 years, duration of disease 2 months-10 years; tumor size: longest diameter 1.0-30 cm, shortest diameter 0.5-15 cm. Pathological diagnosis was benign nerve sheath tumor of scrotum in 5 cases, including 1 case combined with penile cancer, patient age 78 years, the average age of the rest of benign tumor patients 38.4 years; malignant nerve sheath tumor in 3 cases, average age 75 years, including 1 Among the 8 patients, the average diameter of benign nerve sheath tumor was 9 cm, and the average duration of disease was 3.4 years; the average diameter of malignant nerve sheath tumor was 12.6 cm, and the average duration of disease was 2.2 years. Another case of malignant nerve sheath tumor in the perineum with a diameter of about 5 cm was reported by Kai-Rong Lei [7], and one case of intra-scrotal nerve sheath tumor was reported by Zheng-Yan Tang [10], which was pathologically confirmed as malignant nerve sheath tumor of the testis. Wang Xianzhong [11] reported a case of inguinal nerve sheath tumor, which was confirmed intraoperatively to be connected to the spermatic cord, and the pathological findings were benign nerve sheath tumor. Discussion The scrotum is a skin pouch, and the scrotal wall is a continuation of the skin of the lower abdominal wall. The occurrence of tumors in this area is similar to that of other parts of the skin tissue, except that the types of occurrence, incidence and manifestations differ, and they are also divided into two categories of benign and malignant. Most benign tumors of the scrotal wall originate from the tissue adnexa and have a low overall incidence. The common ones are: scrotal cyst, hemangioma, lymphangioleioma, and lipoma, smooth muscle tumor, fibroma, nerve sheath tumor, etc., which have been reported in the literature in small numbers. Malignant tumors of the scrotal wall include squamous cell carcinoma, basal cell carcinoma, and inflammatory carcinoma (eczema-like carcinoma, Paget’s disease) [1]. Scrotal nerve sheath tumors are extremely rare, and malignant nerve sheath tumors of the scrotum are even rarer. Nerve sheath tumors originate from the Schwann cells of the peripheral nerve sheath, unlike neurofibromas that originate from the connective tissue of the nerve sheath cells and mesenchymal tissue. Nerve sheath tumors are rare clinically and were first described by Verocay in 1908 and then by Stout [12] in 1918, who suggested that they originated from the Shewan cells and were called nerve sheath tumors. Most nerve sheath tumors are benign and malignant are rare. Malignant nerve sheath tumors are currently considered to be hypodifferentiated spindle cell sarcomas of peripheral nerve origin, which rarely arise from nerve sheath tumors but can arise from nerve fibroid tumors. Malignant nerve sheath tumors are usually well-defined, often with pseudo-envelope, and variable in size, nodular, lobulated, or irregular. The tumors are distributed in the intra- and paramedian parts of the nerve trunk, with infiltrative growth, multiple and highly invasive. The histopathological features are: microscopically, the tumor cells are dense, mainly fat shuttle-shaped, and may be arranged in a wavy, fenestrated, or swirling pattern, with distorted rippled nuclei, rare nucleoli, and common nuclear schizophrenia. Immunohistochemistry, S-100(+), myelin basic protein or ultrastructure show that the tumor cells have the characteristics of nerve sheath cell differentiation. Nerve sheath tumors are less clinically symptomatic and are mostly single nodular masses encased by a fibrous capsule composed of the outer nerve membrane and residual nerve fibers; the nerve bundle does not usually pass through the tumor. In rare cases, the tumor is intramembranous in origin and grows in a plexiform or multinodular fashion, much like a neurofibroma. They can occur throughout the body, mostly on the flexor surfaces of the upper and lower limbs, followed by the head and neck, posterior mediastinum and retroperitoneum, and again on the cranial nerves. Surgery is the only treatment for nerve sheath tumors, which are usually found occasionally. Since nerve sheath tumors usually have an intact envelope, especially in early cases, complete excision is not difficult, and resection of tumor margins is sufficient to prevent tumor recurrence and preserve nerve function as much as possible. Scrotal nerve sheath tumors originate from the peripheral nerve fibers in the scrotum. A review of the relevant literature indicates that the proportion of malignant nerve sheath tumors in the scrotum is relatively high compared with those in other areas, and is characterized by high age of onset, short disease duration, and large tumor size. Preoperative diagnosis of this disease is difficult, and the clinical presentation is not specific. Generally speaking, imaging examination can only localize the tumor, and it is difficult to provide sufficient basis for preoperative diagnosis. The course of the disease is generally long, and pathological examination is required to confirm the diagnosis. The main treatment for this disease is surgical removal of the tumor. The prognosis of nerve sheath tumor is good, but there is a possibility of local recurrence. Zhang Jiandong reported a case of malignant nerve sheath tumor in the scrotum: 2 pieces of gray-white irregular tissue in the left scrotum with gray-white cut surface and fine texture. Microscopically, tumor cells were seen in long spindle, short spindle, ovoid or polygonal shape, with deep-stained nuclei and frequent nuclear divisions, most areas were dense spindle cell areas, some areas were areas of mucus-like degeneration, tumor cells were short spindle or polygonal, cells were loosely arranged with different directions, and hemorrhage and necrosis were seen. Immunohistochemistry: S-100(+),MBP(+),CD34(-),CR(-),CK(-). Pathological diagnosis: (left) malignant peripheral nerve sheath tumor of the scrotum. Nerve sheath tumors are mostly benign lesions. Whether the malignant nerve sheath tumor of the scrotum is a benign tumor malignancy cannot be determined at present, so long-term follow-up is necessary. In view of the small number of reported cases of scrotal nerve sheath tumors, the presence of a large number of cases will allow a more accurate determination of the benign-to-malignant ratio and further enhance the understanding of scrotal nerve sheath tumors. It is hoped that this article will provide insight into the research related to scrotal nerve sheath tumors.