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Low-grade myofibroblastic sarcoma (LGMS) characterized by the increased proliferation of myofibroblasts is a rare type of malignant myofibroblastic tumor that frequently occurs in the head and neck region. Presently, there is no consensus regarding the treatment of LGMS. Here, we report a rare case of LGMS of the pharynx in a 40-year-old male admitted to our hospital. The patient underwent resection for a right metastatic lesion and parapharyngeal mass. However, he had recurrence and multiple metastases without a surgical indication. Then the patient received the treatment of anlotinib plus pembrolizumab for 4 cycles, and there was a partial response (PR) to the treatment. Due to the adverse reaction of anlotinib, the patient subsequently received monotherapy of pembrolizumab for 22 cycles and achieved a complete response (CR). As the first case report of the immunotherapy for LGMS, our study highlights that this strategy may be of great significance to the treatment of LGMS.

Fibroadenoma and phyllodes tumor are the prototypical mammary fibroepithelial lesions (FELs). Recently, a subset of FELs, identified as stromal-epithelial lesion (SEL) with myofibroblastic stroma have been labelled as myofibroepithelial nodule (MFN). The MFN stromal cells are diffusely positive for SMA immunostaining and frequently show unusual histological features including irregular borders. There is limited literature on FELs with myofibroblastic or smooth muscle stroma. The etiology of the variation in the FEL stromal histology and its clinical significance is unknown. In this short report we describe clinicopathologic features of six FELs with myofibroblastic and/or smooth muscle stroma. We also report immunohistochemical overexpression of HMGA2 in 2 FELs that contained stromal smooth muscle differentiation suggesting a link to mammary myoid hamartoma. On limited follow up all the 6 FELs with myofibroblastic or smooth muscle stroma had benign outcome. The HMGA2 overexpressing FEL with smooth muscle stroma and myoid hamartoma of the breast show overlapping etiology, and histological features.

Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of inflammatory myofibroblastic tumors located mostly in the intra-abdominal region. Herein, we present a case of a 32-year-old male with a lobulated growth in the right maxilla. Radiology revealed a solitary osteolytic lesion with an irregular margin, causing buccal and palatal cortex erosion. Histopathology revealed a tumor composed of spindle-shaped fascicles that blend into sheets of round to ovoid-shaped epithelioid cells, areas of myxoid changes, and necrosis. Tumor cells showed a moderate amount of eosinophilic cytoplasm, large vesicular nuclei with coarse chromatin, nuclear pleomorphism, and increased mitoses. Tumor cells were immunopositive for ALK-1, focal positivity for smooth muscle actin, panCK, epithelial membrane antigen, and immunonegativity for CD 30, desmin, CD34, and STAT6. P53 showed a wild-type staining pattern, and INI-1 expression was retained. The Ki-67 proliferative index was 22%. To the best of our knowledge, this is the first case of EIMS occurring in the maxilla.

Inflammatory myofibroblastic tumour (IMT) is a rare tumour with an intermediate biological behaviour. It usually occurs in children and adolescents, primarily in the abdomen or lungs. Histopathologically, IMT consists of spindle cells, i.e., myofibroblasts, and a variable inflammatory component. Localization in the urinary bladder is rare. We are presenting a rare case of IMT in the bladder in a middle-aged man treated by partial cystectomy. A 62-year-old man consulted a urologist because of haematuria and dysuric disturbances. A tumorous mass was detected by an ultrasound in the urinary bladder. CT urography described the tumorous mass at the dome of the urinary bladder measuring 2 × 5 cm. A smooth tumorous mass was cystoscopically observed at the dome of the urinary bladder. Transurethral resection of the bladder tumour was performed. Histopathological analysis of the specimen identified spindle cells with a mixed inflammatory infiltrate; immunohistochemical findings showed positivity for anaplastic lymphoma kinase (ALK), smooth muscle actin (SMA) and vimentin. A histopathological diagnosis of IMT was established. It was decided that the patient would undergo a partial cystectomy. A complete excision of the tumour from the dome of the urinary bladder with surrounding healthy tissue was performed. Histopathological and immunohistochemical findings of the sample confirmed the diagnosis of IMT, without the presence of the tumour at the surgical margins. The postoperative course went smoothly. IMT is a rare tumour in adults, especially localised in the urinary bladder. IMT of the urinary bladder is difficult to distinguish from urinary bladder malignancy both clinically and radiologically, as well as histopathologically. If the location and size of the tumour allow it, bladder-preserving surgeries such as partial cystectomy represent a reasonable modality of operative treatment.

BACKGROUND: Low-grade myofibroblastic sarcoma (LGMS) is an atypical type of tumor composed of myofibroblasts. LGMS in the femoral head neck junction is extremely rare and no case treated by hip arthroscopy was reported.
METHODS: We reported a case of LGMS in the femoral head neck junction treated by hip arthroscopy. A 30-year-old female was admitted to our hospital with discomfort and pain after left hip sprained one year prior. Physical examination revealed swelling of the left hip and magnetic resonance images showed a soft tissue mass in the femoral head neck junction.
METHODS: Via microscopy of pathological specimens, spindle cell proliferative lesions, atypia of some cells, and mitotic figures/pathological mitotic figures of some cells were observed. Immunohistochemistry revealed positive for smooth muscle actin, focally positive for CD34 and CD68, while negative for S-100, desmin, and anaplastic lymphoma kinase. The imaging, histomorphological and immunohistochemical features suggested a final diagnosis of LGMS of the proximal femur.
METHODS: This patient underwent hip arthroscopy for excision of the soft tissue mass.
RESULTS: The clinical and imaging follow-up at 6 months postoperatively showed that surgery had achieved good clinical outcomes.
CONCLUSIONS: To the best of our knowledge, this is the first case report of LGMS in the femoral head neck junction treated by hip arthroscopy. Beyond the present case, other 120 cases from 58 literatures (1998-2022) are reviewed and discussed. The age of LGMS patients ranged from 11 months to 77 years and the male-to-female ratio was approximately 1.28:1. The location distribution of previously reported LGMS cases and the present case was as follows: Head&neck (45.90%), trunk (30.33%), and extremity (23.77%). Hip arthroscopic excision of LGMS may achieve relatively good clinical outcomes.

Proliferative fasciitis (PF) is a rare pseudosarcomatous myofibroblastic benign tumor, a subcutaneous counterpart of proliferative myositis. Usually seen in upper extremities, no case has yet been documented in tongue or any other subsites in oral cavity. The present case becomes the first to be reported at this site as well as the first case of synchronous coexistent PF with squamous cell carcinoma (SCC) of tongue. The patient was 50 years male, having a polypoidal swelling at right lateral border of tongue with an ulcer adjacent to it. Histopathologically, the swelling was diagnosed as PF and ulcer as SCC; both the diagnoses were confirmed by immunohistochemistry. The polypoidal lesion was immunopositive for smooth muscle actin and calponin and immunonegative for pan cytokeratin, cytokeratins (5/6), P40 and P63, proving it to be a non-SCC lesion, different from its adjacent ulcerative one.

Low-Grade Myofibroblastic sarcoma (LGMS) is categorized as an extremely rare malignant neoplasia of myofibroblasts, which has only recently become more widely studied. Our patient was referred for evaluation of a nodule involving the palate. The histopathological analysis showed a mesenchymal tumor formed for stellate-shaped cells. Negativity for Laminin, Desmin, Collagen IV, CK pool, CD34, S100, and ALK1, discarded epithelial, endothelial, neural, and inflammatory origin. On the other hand, the positivity for Calponin and SMA demonstrated myofibroblastic and smooth muscle differentiation. The diagnosis of LGMS was endorsed and the patient was submitted for medical treatment. To date, only 18 cases describing patients diagnosed with intrabuccal LGMS have been reported in the scientific literature. Here, we introduce a rare report and for the first time, also provide an update of the literature and a clear review regarding the immunohistochemical panel to diagnose this entity, once the histopathological diagnosis is still challenging.

BACKGROUND: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal tumor that is prevalent among children and adolescents. Surgery is the most important therapeutic approach for IMT and complete resection is recommended. Although 50% of IMTs show anaplastic lymphoma kinase (ALK) rearrangements, crizotinib has proven an effective therapeutic approach. However, the genetic landscape of this tumor is still not fully understood and treatment options are limited, especially in the majority of ALK-negative tumors.
METHODS: We describe the clinical case of a healthy 18-year-old female in whom a pulmonary nodule was incidentally detected.
METHODS: Following a small increase in the size of the nodule, the patient underwent both 18FDG-PET/CT and 68Ga-PET/CT, resulting in a suspicion of bronchial hamartoma.
METHODS: The patient underwent surgery and a salivary gland-like lung tumor was diagnosed.
RESULTS: After surgery, the patient was referred to our cancer center, where a review of the histology slides gave a final diagnosis of ALK-negative lung IMT. Given the histology, it was decided not to administer adjuvant therapy and the patient was placed in a 3-monthly follow-up program. The patient is still disease-free 2 years post-surgery.
CONCLUSIONS: Although there is no standard of care for the treatment of IMT, identifying genomic alterations could help to redefine the management of patients with negative-ALK disease. Our review of the literature on IMT and other kinase fusions revealed, in addition to ALK rearrangements, the potential association of ROS1, NTRK, RET, or PDGFR beta alterations with the tumor.

UNASSIGNED: As of August 23, 2020, the 2019 novel coronavirus disease (COVID-19) has infected more than 23,518,340 people and caused more than 810,492 deaths worldwide including 4,717 deaths in China. We present a case of a 53-year-old woman who was admitted to the hospital because of dry coughs and high fever on January 26, 2020, in Wuhan, China. She was not tested for SARS-CoV-2 RNA until on hospital day 11 (illness day 21) because of a significant shortage of test kits at the local hospital. Then, her test was positive for COVID-19 on hospital day 20. Despite intensive medical treatments, she developed respiratory failure with secondary bacterial infection and expired on hospital day 23 (3 days after she was tested positive for SARS-CoV-2 RNA). A systemic autopsy examination, including immunohistochemistry and ultrastructural studies, demonstrates that SARS-CoV-2 can infect multiple organs with profound adverse effect on the immune system, and the lung pathology is characterized by diffuse alveolar damage. Extrapulmonary SARS-CoV-2 RNA was detected in several organs postmortem. The detailed pathological features are described. In addition, this report highlights the value of forensic autopsy in studying SARS-CoV-2 infection and the importance of clinicopathological correlation in better understanding the pathogenesis of COVID-19.