First admission
The patient, 37 years old, married, male, was admitted to the hospital for the first time on May 10, 2015 because of "a subcutaneous mass on the top of the left forehead was found in January". Main complaint: The subcutaneous mass gradually increased in January. Physical examination: The left frontal subcutaneous mass is about 4 cm×5 cm, flexible in texture, no tenderness to the touch, clear border, no redness, no skin ulceration, no obvious sense of movement, 0.7 cm higher than the normal skin margin, and no vascular murmur in the mass is heard. CT prompts: The local skull on the left forehead showed worm-eaten changes, and low-density shadows such as fusiform were seen inside and outside the skull. Part of the brain tissue is slightly compressed and moved inward, its internal density is not uniform, the CT value is about 38 HU, and the uneven edges are enhanced. MRI prompts: On the left frontal parietal bone, there is a fusiform T1 long T2 signal shadow, about 2.2 cm×3.5 cm×4 cm, growing around the skull, the corresponding skull absorption becomes thinner, and the bone destruction is seen in it, showing uneven low signal. After the enhancement, the position is abnormally and unevenly enhanced, and the delayed scan shows obvious enhancement, and the adjacent brain parenchyma is slightly compressed and moved inward (Fig.1).
Preoperative CT showed that the local skull was worm-eaten, and the outer side of the skull showed low-density shadows such as fusiform, and its internal density was uneven(a); Preoperative MRI (T1WI) signal uneven density shadow(b); Preoperative MRI (T2WI) is contour signal(c); Preoperative MRI enhancement showed ring enhancement(d); A re-examination of the brain CT showed that the tumor was completely removed one week after the operation(e).
Operation Procedure: During the operation, the left forehead mass was seen to be fish-like, tough and without obvious adhesion to the scalp. It was frozen during the operation. After completely exposing the tumor outside the skull, drill a hole in the skull, bite the skull along the tumor, and cut the dura mater in a circular shape. There is no adhesion between the tumor and the brain, and the tumor is completely excised (Fig.2); After intraoperative freezing indicated a malignant tumor, the surrounding normal meninges and skull tissue were visible to the naked eye about 2 cm, and the artificial dura mater and titanium mesh were repaired.
Pathological diagnosis: leiomyosarcoma; Irregular cells such as round shape can be seen in the tumor, the nucleus is abnormally enlarged, the nucleus is deeply stained and markedly atypia, a few vacuolar cells are seen, the cytoplasm is rich in eosinophilia, nuclear divisions are seen, diffuse and sheet-like, some interstitial mucinous degeneration(Fig.3); Silver staining (+), B lymphoma-2 (Bcl-2) (+), CD68 (scattered +), CD163 (scattered +), smooth muscle actin (SMA) (+), FLI-1 (+), FN (+), Ki-67 (about 30%+); The edge of the specimen is normal tissue.
Immunohistochemistry SMA(+)(a); Immunohistochemistry Ki-67(+)(b)
Postoperative follow-up: The patient was discharged from the hospital 9 days after extensive tumor resection. After the operation, the tumor was completely removed and the tumor was not sensitive to radiotherapy and chemotherapy, so radiotherapy and chemotherapy were not performed. Routine follow-up visits for 2 years and 3 months, no recurrence was found until August 2017, and no recurrence or distant metastasis was found during PET-CT examination. Two years and four months after surgery, a subcutaneous mass appeared on the left frontotemporal roof, and leiomyosarcoma recurrence was considered.
Second admission
The patient was re-admitted to the hospital on September 27, 2017 because of "the left frontotemporal parietal mass was found for 20 days". Physical examination: The original surgical scar can be seen at the top of the left frontotemporal area, and a subcutaneous mass about 3 cm in diameter can be palpable at the top of the left temporal top. The texture is flexible, no tenderness to the touch, clear borders, no redness, swelling and skin ulceration, and no obvious sense of movement, 0.5 cm higher than the normal skin margin, no blood vessel murmur in the mass was heard. Multiple enlarged lymph nodes can be palpable on both sides of the neck, with a maximum diameter of about 3 cm. CT prompts: The left frontal-temporal parietal bone is congenitally absent, and a wide basal segment with low-density shadows can be seen on the left top. The boundary is about 3.2 cm×1.3 cm, and the CT value is about 39 HU. MRI prompts: The left frontal mass and the frontal bone changed postoperatively. An irregular abnormal lesion was seen in the inner plate of the left parietal bone, showing a slightly longer T2 signal with a length of 3.5 cm×1.6 cm×3.5 cm. After the enhancement, the enhancement is uneven and the space-occupying sign is obvious (Fig.4).
Preoperative CT wide basal segment low-density shadow, clear boundary(a); Preoperative MRI (T1WI) soft tissue showed low density shadow(b); Preoperative MRI (T2WI) is contour signal(c); Preoperative MRI enhancement showed uneven enhancement(d); A re-examination of the brain CT showed that the tumor was completely removed 1 week after the operation(e)
Operation process: During the operation, there was a 3 cm×4 cm mass attached to the three-dimensional titanium mesh on the top of the left forehead, which was not separated from the surrounding tissues, partial resection was performed. After removing the titanium mesh, the space occupied was seen at the top of the left temporal area, with a diameter of 4 cm, and a fish-like appearance with clear boundaries. Remove the artificial dura mater, tumor tissue and the affected skull at this site, and it was found that the tumor originated from the top meninges. 3 cm of skull, dura mater, periosteum and subcutaneous tissue were resected toward the top, and proliferative tissue, artificial dura mater, brain tissue, and skull were resected to the frontotemporal area 4 cm from the tumor. After the pathological biopsy of the resection margin showed negative, the resection range continued to be expanded by about 3-4 cm. At the same time, a 2 cm×5 cm skin flap at the site of the tumor was removed, and one-stage artificial dura mater and titanium mesh were performed.
Pathological diagnosis: leiomyosarcoma (WHO grade Ⅲ); In the tumor, irregular cells such as fusiform and round-like are seen, the nuclei are deeply stained and huge, and are significantly atypia, accompanied by a few vacuolar cells, mitoses are obvious, flaky and diffuse, and some interstitial mucinous degeneration (Fig.5); Silver immersion staining (+), SMA (+); no tumors were found in the surrounding tissues; no tumor cells were found in the neck lymph node needle aspiration cytology biopsy.
Postoperative follow-up: The patient was discharged 10 days after another extended resection. Leiomyosarcoma recurred without metastasis. No radiotherapy or chemotherapy was performed before or after surgery. Regular review after discharge. As of June 1, 2020, the patient has been reviewed for more than 2 years and 8 months without any obvious complications. Follow-up is continuing.