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Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is rare in adults. The presence of intratumoral T lymphocytes and primitive rounded cells characterized this neoplasm. We report a 24-year-old Chinese man who developed EBV-SMT in the right adrenal gland with hepatitis B infection and autoimmune hemolytic anemia without a history of HIV infection, primary immune deficiency, organ transplantation, or malignant tumor. This patient had an unknown immunodeficient state. EBV-SMTs are commonly located in the liver, lung, and gastrointestinal tract but rarely in the adrenal gland. We reviewed 10 reported literature on EBV-SMT in the adrenal gland. It is imperative to distinguish EBV-SMT from conventional somatic smooth muscle tumors. The discovery of EBV-SMT forces the clinician to conduct a thorough evaluation of immune function and immune status surveillance, and these patients are vulnerable to subsequent malignant tumors. [ABSTRACT FROM AUTHOR]

Adrenal leiomyomas are rare and often reported as Epstein-Barr virus (EBV)-associated smooth muscle tumor (SMT) in association with EBV infection in immunocompromised patients. We experienced a case of right adrenal leiomyoma that was incidentally found in a man in his 70s. Computed Tomography (CT) showed a well-circumscribed mass of 3.1 cm in diameter in the right adrenal gland, which increased to 4.9 cm in diameter over 1 year. Preoperative diagnosis was difficult due to the lack of specific imaging findings. He had a history of diffuse large B-cell lymphoma (DLBCL) 8 years ago, and EBV had been detected in his blood. EBV-encoded small RNA(EBER) in situ hybridization (EBER-ISH) of the right adrenal leiomyoma was positive, and the final diagnosis was EBV-associated leiomyoma.

Objectives: The present study aimed to analyze of uterine smooth muscle tumors of uncertain malignant potential (STUMP) and the outcomes of patients with STUMP. Material and methods: In this retrospective study, the data of patients diagnosed with STUMP in a single tertiary center between January 2005--January 2020 were reviewed. We assessed the demographic variables, treatment outcomes, time until recurrence, disease-free and overall survival of the patients. Results: Twenty-five patients diagnosed with STUMP were included in the study. The mean age of the patients was 43.2 ± 10.3 years. Thirteen of the 25 patients (52%) were treated by myomectomy, others received diagnoses following hysterectomy. The median follow-up time was 45.2 months. Recurrence was observed in three cases (12%), two of which were followed up without hysterectomy, and the third patient died by peritonitis carcinomatosa 60 months after diagnosis although she received cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) treatment. Conclusions: This study evaluates the data of patients with STUMP. Our results reveal a STUMP recurrence of 12%, like those previously reported in the literature. Despite the possibility of malignant recurrence, fertility-preserving treatment with close follow-up should be tried, because of the relatively early age at diagnosis. [ABSTRACT FROM AUTHOR]

This case report presents a rare case regarding the contrast-enhanced ultrasound (CEUS) features and minimally invasive treatment of Epstein–Barr virus associated smooth muscle tumors (EBV-SMT) in a patient with human immunodeficiency virus infection (HIV). Currently, there are few studies involving hepatic EBV-SMT. According to WHO guidelines, the malignant potential of the disease is uncertain. The features of CEUS suggest that these hepatic lesions tend to be malignant and are often misdiagnosed as other malignant neoplasms. Presently, hepatic resection is the first choice for treating hepatic EBV-SMT. However, immunocompromised patients may benefit more from minimally invasive microwave ablation therapy (MWA). Since there is no standard diagnosis and therapy are available at present, our findings in this case may contribute to promoting standardized diagnosis and treatment of EBV-SMT.

Abstract Background The greater omentum comprises peritoneal, adipose, vascular, and lymphoid tissues. Most omental malignancies are metastatic tumors, and the incidence of primary tumors is rare. We report on a prior omental smooth muscle tumor case in an adult male patient. Case presentation A 54-year-old Japanese male patient with no relevant medical history was diagnosed with an abdominal mass during a routine medical checkup. Subsequent contrast-enhanced computed tomography revealed a mass of approximately 3 cm in size in the greater omentum, and a laparotomy was performed. A 27 × 25 × 20 mm raised lesion was found in the omentum. Microscopically, spindle cells were observed and arranged in whorls and fascicles. Individual tumor cells had short spindle-shaped nuclei with slightly increased chromatin and were characterized by a slightly eosinophilic, spindle-shaped cytoplasm. The mitotic count was less than 1 per 50 high-power fields. The tumor cells showed positive immunoreactivity for α smooth muscle actin, HHF35, and desmin on immunohistochemical examination. The Ki-67 labeling index using the average method was 1.76% (261/14806). No immunoreactivity was observed for any of the other tested markers. We considered leiomyoma owing to a lack of malignant findings. However, primary omental leiomyoma has rarely been reported, and it can be difficult to completely rule out the malignant potential of smooth muscle tumors in soft tissues. Our patient was decisively diagnosed with a primary omental smooth muscle tumor considering leiomyoma. Consequently, the patient did not undergo additional adjuvant therapy and was followed up. The patient was satisfied with treatment and showed neither recurrence nor metastasis at the 13-month postoperative follow-up. Discussion and conclusion We encountered a primary smooth muscle tumor of the greater omentum with no histological findings suggestive of malignancy in an adult male patient. However, omental smooth muscle tumors are extremely difficult to define as benign, requiring careful diagnosis. Further case reports with long-term follow-up and case series are required to determine whether a true omental benign smooth muscle tumor (leiomyoma) exists. In addition, proper interpretation of the Ki-67 labeling index should be established. This case study is a foundation for future research.

Background: The greater omentum comprises peritoneal, adipose, vascular, and lymphoid tissues. Most omental malignancies are metastatic tumors, and the incidence of primary tumors is rare. We report on a prior omental smooth muscle tumor case in an adult male patient. Case presentation: A 54-year-old Japanese male patient with no relevant medical history was diagnosed with an abdominal mass during a routine medical checkup. Subsequent contrast-enhanced computed tomography revealed a mass of approximately 3 cm in size in the greater omentum, and a laparotomy was performed. A 27 × 25 × 20 mm raised lesion was found in the omentum. Microscopically, spindle cells were observed and arranged in whorls and fascicles. Individual tumor cells had short spindle-shaped nuclei with slightly increased chromatin and were characterized by a slightly eosinophilic, spindle-shaped cytoplasm. The mitotic count was less than 1 per 50 high-power fields. The tumor cells showed positive immunoreactivity for α smooth muscle actin, HHF35, and desmin on immunohistochemical examination. The Ki-67 labeling index using the average method was 1.76% (261/14806). No immunoreactivity was observed for any of the other tested markers. We considered leiomyoma owing to a lack of malignant findings. However, primary omental leiomyoma has rarely been reported, and it can be difficult to completely rule out the malignant potential of smooth muscle tumors in soft tissues. Our patient was decisively diagnosed with a primary omental smooth muscle tumor considering leiomyoma. Consequently, the patient did not undergo additional adjuvant therapy and was followed up. The patient was satisfied with treatment and showed neither recurrence nor metastasis at the 13-month postoperative follow-up. Discussion and conclusion: We encountered a primary smooth muscle tumor of the greater omentum with no histological findings suggestive of malignancy in an adult male patient. However, omental smooth muscle tumors are extremely difficult to define as benign, requiring careful diagnosis. Further case reports with long-term follow-up and case series are required to determine whether a true omental benign smooth muscle tumor (leiomyoma) exists. In addition, proper interpretation of the Ki-67 labeling index should be established. This case study is a foundation for future research. [ABSTRACT FROM AUTHOR]

Objective: Epstein-Barr Virus-Associated Smooth Muscle Tumor (EBV-SMT) is a rare tumor with a higher rate of occurrence in unusual locations in the setting of immunodeficiency. In this study, we evaluated a cohort of ordinary leiomyosarcomas (LMS) for the presence of EBV and described the clinicopathological features deviating from routinely diagnosed cases of EBV-SMT. Material and Method: The sections of tissue microarrays including 93 classical LMS occurring in various locations were hybridized with EBER and stained for LMP1 antibody using the Leica Bond Autostainer. EBV real-time PCR assay was performed in 2 EBER-positive cases. Results: Among the 93 LMS cases, 2 non-uterine cases (2.2%) were positive for EBER and negative for LMP1, and were referred to as `EBV-positive LMS`. Both were females in their 6th decade without immunosuppression. EBV real-time PCR assay revealed the presence of EBV in one of the cases. Tumors were located in the pancreas and chest wall. Morphologically, tumors were rather myxoid, multinodular, and composed of long fascicles of spindle cells with intermediate- to high-grade features. High mitotic activity and focal necrosis were present, whereas no accompanying lymphocytes were detected. One of the patients developed metastatic disease after 3 years. Conclusion: EBV-positive LMS occurring in immunocompetent patients has features distinct from classical EBV-SMT seen in immunosuppressed patients.

Objective: Epstein-Barr Virus-Associated Smooth Muscle Tumor (EBV-SMT) is a rare tumor with a higher rate of occurrence in unusual locations in the setting of immunodeficiency. In this study, we evaluated a cohort of ordinary leiomyosarcomas (LMS) for the presence of EBV and described the clinicopathological features deviating from routinely diagnosed cases of EBV-SMT. Material and Methods: The sections of tissue microarrays including 93 classical LMS occurring in various locations were hybridized with EBER and stained for LMP1 antibody using the Leica Bond Autostainer. EBV real-time PCR assay was performed in 2 EBER-positive cases. Results: Among the 93 LMS cases, 2 non-uterine cases (2.2%) were positive for EBER and negative for LMP1, and were referred to as "EBV-positive LMS". Both were females in their 6th decade without immunosuppression. EBV real-time PCR assay revealed the presence of EBV in one of the cases. Tumors were located in the pancreas and chest wall. Morphologically, tumors were rather myxoid, multinodular, and composed of long fascicles of spindle cells with intermediate-to high-grade features. High mitotic activity and focal necrosis were present, whereas no accompanying lymphocytes were detected. One of the patients developed metastatic disease after 3 years. Conclusion: EBV-positive LMS occurring in immunocompetent patients has features distinct from classical EBV-SMT seen in immunosuppressed patients. [ABSTRACT FROM AUTHOR]

Epstein-Barr virus (EBV) can rarely induce smooth muscle tumors (SMTs). A 20-year-old female patient underwent kidney transplantation for renal failure. Since then, she has been treated with immunosuppressants, including a calcineurin inhibitor, tacrolimus, and prednisolone, owing to the immunological rejection. Three years later, she developed large liver tumors (diameter >5 cm) and multiple small lung tumors that were identified as EBV-SMTs based on the results of liver biopsy/histopathology. No intervention was performed except for the addition of a mammalian target of the rapamycin inhibitor, everolimus, which inhibits both immune reaction and SMT growth. Finally, after 8 years, the transplanted kidney became nonfunctional, and immunosuppressant administration became unnecessary as urinary dialysis was started. Under these circumstances, SMT growth was observed despite the absence of immunosuppressant administration. Three months after the cessation of the immunosuppressants, EBV-SMTs in the liver and lungs shrank slightly. To the best of our knowledge, this is the first report on the genomic profile of this rare tumor. The clinical course of our patient indicates that EBV can induce SMTs, and immunological suppression of EBV may inhibit the activity of these tumors.

Colonic leiomyomatous lesions are smooth muscle tumors including both benign leiomyoma and malignant leiomyosarcoma. They usually occur in elderly and middle-aged groups. They are rare, representing 3% of all gastrointestinal leiomyomas. Descending and sigmoid colon are the most commonly affected segments of colon. Patients are usually asymptomatic but occasionally they may present with abdominal pain, hemorrhage, and intestinal obstruction. Radiological findings for these lesions are variable and overlapping with other diagnoses but usually manifest as large lesions with lobulated margins, extra-colic growth, and heterogeneous enhancement. Final diagnosis is achieved by tissue diagnosis in which immunohistochemistry is used to differentiate them from other types of mesenchymal tumors like gastrointestinal stromal tumor. A complete surgical/endoscopic resection is usually curative with an excellent prognosis in cases of benign leiomyoma. In cases of leiomyosarcoma, post-surgical chemotherapy and radiotherapy are usually needed with a five-year survival of about 50%. We report the radiological findings with pathological correlation and literature review of two cases of colonic leiomyomatous lesions that presented with nonspecific abdominal pain. The lesions were resected surgically and confirmed histopathologically as leiomyoma and leiomyosarcoma of the colon, respectively. To the best of our knowledge, these cases are the first of colonic leiomyomatous lesions to be reported in Oman.

A 48‐year‐old woman underwent total abdominal hysterectomy and right salpingo‐oophorectomy and was initially diagnosed with a uterine leiomyoma and right ovarian cystadenoma. After 4 years, multiple pulmonary metastases were identified, and treatment with gonadotropin‐releasing hormone agonists was started, but stopped later due to disease progression. The patient developed dyspnea and underwent right upper lobectomy. The histopathological findings were consistent with those of pulmonary metastases secondary to a uterine smooth muscle tumor of uncertain malignant potential. Slow disease progression after a poor response to adriamycin and hormone receptor positivity led to the start of letrozole. Letrozole induced spontaneous regression of the pulmonary metastases, and about 2 years into the treatment, sustained response was achieved with minimal side effects. This may be the first case supporting the long‐term efficacy and safety of letrozole in the management of adriamycin‐resistant lung metastases of uterine smooth muscle tumors of uncertain malignant potential. [ABSTRACT FROM AUTHOR]

We report an Epstein-Barr virus–associated smooth muscle tumor in an adult male with AIDS. The patient had multiple lung nodules seen on computed tomography of the chest and an endobronchial lung tumor identified on bronchoscopy. Initiation of antiretroviral therapy slowed the progression of the tumors. [ABSTRACT FROM AUTHOR]

Smooth muscle tumors represent the second most common mural mesenchymal neoplasm in the gastrointestinal tract, but established criteria for prognostic assessment of these tumors are lacking. A large cohort of surgically resected intramural gastrointestinal smooth muscle tumors from 31 institutions was analyzed to identify potential prognostic features. Pathologic features were assessed by expert gastrointestinal and/or soft tissue pathologists at each center. Immunohistochemical confirmation was required. A total of 407 cases from the esophagus (n = 97, 24%), stomach (n = 180, 44%), small bowel (n = 74, 18%), and colorectum (n = 56, 14%) were identified. Patients ranged in age from 19 to 92 years (mean 55 years), with a slight female predominance (57%). Mean tumor size was 5.4 cm, with the largest tumor measuring 29 cm. Disease progression following surgery, defined as local recurrence, metastasis, or disease-related death, occurred in 56 patients (14%). Colorectal tumors were most likely to progress, followed by small bowel and gastric tumors. None of the esophageal tumors in this series progressed. Receiver operator characteristic analysis identified optimal cutoffs of 9.8 cm and 3 mitoses/5 mm2 for discriminating between progressive and non-progressive tumors. Histologic features strongly associated with progression by univariate analysis included moderate-to-severe atypia, high cellularity, abnormal differentiation (defined as differentiation not closely resembling that of normal smooth muscle), tumor necrosis, mucosal ulceration, lamina propria involvement, and serosal involvement (P 10 cm and/or showing ≥3 mitoses/5 mm2 may behave aggressively, and therefore close clinical follow-up is recommended in these cases.

Uterine smooth muscle tumor of uncertain malignant potential is a subtype of uterine smooth muscle neoplasms. It is characterized by distinct pathologic findings with morphologic features intermediate between those of benign leiomyoma and malignant leiomyosarcoma. Clinically, STUMP is rare and its clinical picture is comparable to that of leiomyoma, with diagnosis typically being made postoperatively. Most patients with STUMP are uneventful after tumor resection. However, a small portion of patients may experience recurrence that may even lead to mortality. Given the uncommon occurrence of STUMP and the low frequency of malignant potential, currently there is still no standard guideline in treating patients with this disease and this can be challenging for physicians. Moreover, because cases are rarely available for study, investigating this tumor is difficult. Thus, matters such as the pathologic diagnostic criteria, strategy of clinical management, identification of prognostic factors, and the pathogenesis of this disease remain to be clarified. We collected and analyzed recently published case series studies of STUMP to obtain up-to-date clinical information. The current status of research in various basic and clinical aspects of this tumor was also reviewed.

We aimed to develop an artificial intelligence (AI) diagnosis system for uterine smooth muscle tumors (UMTs) by using deep learning. We analyzed the morphological features of UMTs on whole-slide images (233, 108, and 30 digital slides of leiomyosarcomas, leiomyomas, and smooth muscle tumors of uncertain malignant potential stained with hematoxylin and eosin, respectively). Aperio ImageScope software randomly selected ≥10 areas of the total field of view. Pathologists randomly selected a marked region in each section that was no smaller than the total area of 10 high-power fields in which necrotic, vascular, collagenous, and mitotic areas were labeled. We constructed an automatic identification algorithm for cytological atypia and necrosis by using ResNet and constructed an automatic detection algorithm for mitosis by using YOLOv5. A logical evaluation algorithm was then designed to obtain an automatic UMT diagnostic aid that can "study and synthesize" a pathologist's experience. The precision, recall, and F1 index reached more than 0.920. The detection network could accurately detect the mitoses (0.913 precision, 0.893 recall). For the prediction ability, the AI system had a precision of 0.90. An AI-assisted system for diagnosing UMTs in routine practice scenarios is feasible and can improve the accuracy and efficiency of diagnosis. [ABSTRACT FROM AUTHOR]

Epstein-Barr virus (EBV) can rarely induce smooth muscle tumors (SMTs). A 20-year-old female patient underwent kidney transplantation for renal failure. Since then, she has been treated with immunosuppressants, including a calcineurin inhibitor, tacrolimus, and prednisolone, owing to the immunological rejection. Three years later, she developed large liver tumors (diameter >5 cm) and multiple small lung tumors that were identified as EBV-SMTs based on the results of liver biopsy/histopathology. No intervention was performed except for the addition of a mammalian target of the rapamycin inhibitor, everolimus, which inhibits both immune reaction and SMT growth. Finally, after 8 years, the transplanted kidney became nonfunctional, and immunosuppressant administration became unnecessary as urinary dialysis was started. Under these circumstances, SMT growth was observed despite the absence of immunosuppressant administration. Three months after the cessation of the immunosuppressants, EBV-SMTs in the liver and lungs shrank slightly. To the best of our knowledge, this is the first report on the genomic profile of this rare tumor. The clinical course of our patient indicates that EBV can induce SMTs, and immunological suppression of EBV may inhibit the activity of these tumors. [ABSTRACT FROM AUTHOR]

Introduction: Primary small intestinal malignancies are rare with an incidence of less than 5% of all gastrointestinal malignancies and are more common in the middle-aged and elderly population. They are comprised either an adenocarcinoma, neuroendocrine tumor, gastrointestinal stromal tumor (GIST), lymphoma, and/or sarcoma. Case Presentation: Here we exhibit the case of a 23-year-old who presented with progressive nausea, weight loss, abdominal pain, and iron deficiency anemia and was diagnosed with leiomyosarcoma of the jejunum. Conclusion: We distinguish a GIST from leiomyosarcoma based on radiographic, histologic, and epidemiologic evidence and review the significance of prompt, accurate diagnosis as related to treatment. [ABSTRACT FROM AUTHOR]

Case summary: Leiomyoma of the urinary bladder in cats is infrequently documented in the literature, and a description of the ultrasonographic appearance has never been reported. A 9-year-old female spayed cat presented with a 6-month history of intermittent hematuria and progressive pollakiuria. On abdominal ultrasound, a large ovoid mass of mixed echogenicity was seen originating from the ventral apical bladder wall. The mass had faint peripheral hyperechoic radiating striations and was mildly vascularized, especially at its 1.5 cm point of origin from the wall. A partial cystectomy was performed to remove the mass that filled the bladder lumen. Microscopically, the well-demarcated mass extended from the inner muscular layer of the wall into the submucosal layer and was diagnosed as a leiomyoma. Surgical excision was curative. Relevance and novel information: This is the first ultrasonographic description of a bladder leiomyoma in a feline patient. Smooth muscle tumors should be included on the differential diagnosis list in future cases with similar ultrasonographic features and chronic lower urinary clinical signs. [ABSTRACT FROM AUTHOR]

Background: Primary immunodeficiency (PID) having defects related to lymphocyte cytotoxic pathway or T-cell dysfunction are well known for developing opportunistic infections and Epstein-Barr virus (EBV)-associated diseases. CARMIL2 deficiency is a recently described combined immunodeficiency (CID) disorder characterized by defective CD28-mediated T cell co-stimulation, altered cytoskeletal dynamics, susceptibility to various infections and Epstein Barr Virus smooth muscle tumor (EBV-SMT). Case report: We report a homozygous CARMIL2 pathogenic variant presenting with recurrent infections and EBV associated smooth muscle tumor (SMT) in a child. Conclusion: The present study reports that EBV SMT may occur in a child with CARMIL2 deficiency. [ABSTRACT FROM AUTHOR]

Fat‐poor leiomyomatous angiomyolipoma, which is similar to smooth muscle tumors, is positive for smooth muscle markers and melanocytic marker human melanin black 45 (HMB‐45). We report a case of fat‐poor leiomyomatous angiomyolipoma arising from renal parenchyma negative for HMB‐45 diagnosed by combined staining with melanocytic markers HMB‐45 and Melan‐A. [ABSTRACT FROM AUTHOR]

Abstract Introduction Immunodeficient patients, including the recipients of solid organs, exhibit an increase in the incidence of neoplasms. Post-transplant smooth muscle tumor (PTSMT) is a distinct and infrequent entity of these groups of neoplasms. Epstein–Barr virus (EBV) is considered to be involved in the etiology of this neoplasm. Case report A 28-year-old man who underwent liver transplantation presented with abdominal pain and diarrhea for several months. He had a history of resistant systemic cytomegalovirus (CMV) infection after transplantation. Radiologic evaluation and colonoscopy revealed multiple liver, spleen, lung, and colon lesions. Microscopic assessment of colon and liver lesions using IHC study were in favor of spindle cell proliferation with mild atypia and a mild increase in mitotic rate without any necrosis, with features of smooth muscle tumor. Considering the transplantation history, EBER chromogenic in situ hybridization (CISH) study on paraffin blocks was requested, which demonstrated EBV RNA in tumor cell nuclei, suggesting EBV-associated smooth muscle tumor. In addition, PCR for CMV on paraffin blocks was positive. PCR for EBV and CMV viremia were negative. The dosage of immunosuppressive agents was reduced, and currently, he is being followed, with slow expansion in the size of the lesions. Conclusion Although the incidence of post-transplant smooth muscle tumors (PTSMTs) is low, it should be remained in the differential diagnosis in post-transplantation patients, especially dealing with multifocal tumors. As strong stimulant for smooth muscle tumors, close follow-up and screening for EBV and CMV infection and early treatment at the time of diagnosis are recommended to avoid these virus-induced tumors.

Abstract Fat‐poor leiomyomatous angiomyolipoma, which is similar to smooth muscle tumors, is positive for smooth muscle markers and melanocytic marker human melanin black 45 (HMB‐45). We report a case of fat‐poor leiomyomatous angiomyolipoma arising from renal parenchyma negative for HMB‐45 diagnosed by combined staining with melanocytic markers HMB‐45 and Melan‐A.

Leiomyosarcomas are rare aggressive smooth muscle tumors that represent 0.1% of all colorectal malignancies. The lack of literature available concerning leiomyosarcomas presents a challenge when diagnosing and treating these tumors, thus it is crucial that we differentiate them from gastrointestinal stromal tumors (GIST), the most common type of mesenchymal neoplasms of the gastrointestinal (GI) tract, especially when considering leiomyosarcoma's high prevalence of recurrence and malignancy. In this article, we present a case of a 74-year-old male with a diagnosis of colonic leiomyosarcoma. We analyze the tumor's CT imaging findings as well as correlation with the patient's pathological findings including immunostains, size, and mitotic activity, as well as the patient's risk for recurrence.

Uterine leiomyomas usually arise from the uterine body (95%), and rarely from the cervix (0.6%) or other urogenital sites. Lipoleiomyomas are benign, uncommon variants of leiomyomas (0.03–0.2%), histologically composed of smooth muscle cells and mature adipocytes; they usually occur in the uterine body and exceptionally in the cervix. We performed the first systematic literature review of cervical lipoleiomyomas (PRISMA guidelines), presenting five new cases. Including our series, thirty-one detailed cases were reported in the literature (mainly in Asia). The age range was 35–74 years, revealing a higher mean age than conventional cervical leiomyomas (46.5 vs. 39.4 years). Patients were usually multiparous (94%), typically complaining of vaginal bleeding (11/31, 36%), pelvic/abdominal pain (10/31, 32%), and/or urinary disturbances (6/31, 19%) 1 week to 10 months before presentation. Clinical examination revealed a pedunculated tumor (48%), or prolapse of ≥1 pelvic organs (16%). Twenty-four (77%) patients underwent total hysterectomy ± additional surgery; simple myomectomy/excision was performed in five (16%) cases. Only one (3%) of our cases recurred 2 years after partial excision; no evidence of disease was found 13 years after recurrence excision. Adipocytes occupied ≤50% of the tumor volume. Hyaline or myxoid changes and cartilaginous metaplasia were uncommon histological findings. Surgically challenging cases or pregnant patients may require expert gynecologists. Interventional radiology or conservative treatments were rarely proposed. [ABSTRACT FROM AUTHOR]

This case report presents a rare case regarding the contrast-enhanced ultrasound (CEUS) features and minimally invasive treatment of Epstein-Barr virus associated smooth muscle tumors (EBV-SMT) in a patient with human immunodeficiency virus infection (HIV). Currently, there are few studies involving hepatic EBV-SMT. According to WHO guidelines, the malignant potential of the disease is uncertain. The features of CEUS suggest that these hepatic lesions tend to be malignant and are often misdiagnosed as other malignant neoplasms. Presently, hepatic resection is the first choice for treating hepatic EBV-SMT. However, immunocompromised patients may benefit more from minimally invasive microwave ablation therapy (MWA). Since there is no standard diagnosis and therapy are available at present, our findings in this case may contribute to promoting standardized diagnosis and treatment of EBV-SMT., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Yang, Bai, Wu, Yang and He.)

Gastrointestinal (GI) leiomyomas, particularly those located in the rectum, are extremely rare, accounting for approximately 0.1% of rectal tumors. We report the case of a middle-aged female who presented with chronic diarrhea of unknown origin. A colonoscopy was performed, during which a rectal polyp was resected. Histopathology examination of the polyp revealed smooth muscle proliferation and a positive immunohistochemical profile for desmin, consistent with leiomyomatous nature and a diagnosis of rectal leiomyoma. This case underscores the importance of a comprehensive diagnostic approach, including endoscopic, histologic, and immunohistochemical analysis, to differentiate leiomyomas from other GI tumors. Following GI evaluation, the patient underwent a total abdominal hysterectomy, which revealed multiple uterine leiomyomas. The concurrent presence of rectal and uterine leiomyomas in this patient is significant and suggests a potential association between these lesions in females. Further research is needed to explore the potential relationship between rectal and uterine leiomyomas and its implications for clinical practice., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Nieuwland et al.)

Abstract Epstein Barr virus-associated smooth muscle tumors are an uncommon neoplasm that occurs in immunosuppressed patients of any age. Usually, it presents as multifocal tumors mainly in the spinal cord, epidural region, gastrointestinal tract and liver, upper respiratory tract and skin, the latest with few cases reported in the literature and related with human immunodeficiency virus infection and acquired immune deficiency syndrome. The authors present the first case of a Colombian adult patient with human immunodeficiency virus infection and multifocal Epstein Barr virus-associated smooth muscle tumors in the skin and epidural region, confirmed by histopathology, immunohistochemistry and in situ hybridization studies.

Primary thyroid leiomyosarcoma is an extremely rare soft tissue sarcoma, characterized by high malignancy and poor prognosis. Currently, only 13 cases of primary thyroid leiomyosarcoma have been described in the medical literature (limited to English). A 76-year-old female presented with a giant neck mass. Physical examination revealed a large, firm mass in the left thyroid gland. No symptoms such as hoarseness or dysphagia were noted at the time of presentation. The patient underwent unilateral thyroidectomy and cervical lymph node dissection. Pathologic findings revealed a low-grade sarcoma with spindle-shaped tumor cells in an interwoven, sheet-like distribution. Immunohistochemistry showed positivity for desmin, SMMHC, STAT6, CK19, and Galectin3, but negativity for S-100, MyoD1, CD34, CK (AE1/AE3), CD117, and CD56. The findings were consistent with thyroid leiomyosarcoma. The treatment of primary thyroid LMS presents challenges due to its atypical symptoms and high malignance, highlighting the imperative for further exploration of therapeutic strategies to improve the outcomes. • Primary thyroid leiomyosarcoma is rare. Only 13 cases have been described in the medical literature. • Primary thyroid leiomyosarcoma is extremely aggressive, with a high risk of recurrence and metastasis. • The diagnosis of primary thyroid leiomyosarcoma relies on the biopsy and immunohistochemical analysis. • Patients often experience tumor recurrence, distant metastasis, or even mortality shortly following surgery. [ABSTRACT FROM AUTHOR]

Simple Summary: The preoperative diagnosis of the uterine smooth muscle tumor of uncertain malignant potential (STUMP) is a challenge, as it does not have specific preoperative features; diagnosis is often incidental to the postoperative specimen in patients with suspected fibroids. Electrical morcellation during laparoscopic surgery can lead to cellular diffusion in the peritoneal cavity. Therefore, in 2014, the U.S Food and Drug Administration published an alert that focused the entire world's attention on the associated risks. Although STUMPs are thought to have low malignant potential, their capacity for recurrence or metastasis is described. Nevertheless, there are no available guidelines, and data about the risk factors for recurrence are scarce and of low quality. Thus, we felt the need for an updated literature review on this topic to provide any information about characteristics and outcomes of patients with STUMP over a period of 60 years, focusing on the risk factors for recurrence. Background: This study aimed to systematically review the existing literature on uterine smooth muscle tumor of uncertain malignant potential (STUMP) to provide information about characteristics and outcomes of patients and the risk factors for recurrence over a period of 60 years (1960–2021). Methods: According to PRISMA guidelines, we searched for "uterine smooth muscle tumor of uncertain malignant potential" in PubMed (all fields) and Scopus (Title/Abstract/Keywords) databases (accessed on 1 January 2022). Relevant articles were obtained in full-text format and screened for additional references. The only filter used was the English language. Studies including full case description of patients with histopathological diagnosis of STUMP in accordance with Stanford criteria were included. Results: Thirty-four studies, including 189 cases, were included. The median age was 43 years, and in 21.5% of cases there was a recurrence of the disease. Bivariate analysis showed a significant association between use of morcellation without bag and risk of recurrence (p = 0.001). Unprotected morcellation during demolitive or conservative surgery was independently associated with a higher risk of disease recurrence with a relative risk of 2.94 (p < 0.001). A significant progressive decrease in the recurrence rate was observed over time (r = −0.671, p = 0.008). The percentage of patients who underwent surgery followed by in-bag protected morcellation significantly increased after the publication of the U.S. Food and Drug Administration alert about the risk linked to this procedure (p = 0.01). Conclusions: Unprotected morcellation of the lesion is associated with the relapse of the disease. However, this clinical condition showed a drastic decrease over time. This could likely be due to the increased awareness by surgeons of the importance of customizing surgical treatment. [ABSTRACT FROM AUTHOR]

Smooth muscle neoplasms represent an important group of lesions which is rare in the oral cavity. Leiomyoma (LM) is benign smooth muscle/pericytic tumor usually presenting as non-aggressive neoplasm, while leiomyosarcoma (LMS) represents its malignant counterpart. The rarity of these lesions, together with its unspecific clinical presentation and a variable histopathological appearance, lead to a broad list of differential diagnoses, hampering their diagnoses. Therefore, in this study we describe the clinical and microscopic features of a series of oral and maxillofacial LMs and LMSs. A retrospective search from 2000 to 2019 was performed and all cases diagnosed as LM and LMS affecting the oral cavity and gnathic bones were retrieved. Clinical and demographic data were obtained from the patients' pathology records, while microscopic features and immunohistochemistry were reviewed and completed when necessary to confirm the diagnoses. Twenty-two LMs and five LMSs were obtained. In the LM group, males predominated, with a mean age of 45.7 years. The upper lip was the most affected site, and 18 cases were classified as angioleiomyomas and four as solid LM. In the LMS group, females predominated, with a mean age of 47.6 years. The mandible was the most affected site. Diffuse proliferation of spindle cells, with necrosis and mitotic figures, were frequent microscopic findings. LMs and LMSs were positive for α-smooth muscle actin, HHF-35 and h-caldesmon. In conclusion, oral LM/LMS are uncommon neoplasms with the latter usually presenting as metastatic disease. H&E evaluation may be very suggestive of oral LMs, but h-caldesmon staining is strongly recommended to confirm LMS diagnosis. [ABSTRACT FROM AUTHOR]

Primary superficial leiomyosarcoma is a very uncommon malignant tumor which occurs most commonly the lower limbs. We report one case of unusual topography of this tumor. An 81-year-old female patient presented with a 2 year history of a periungual tumor of the left index finger. The histopathological and immunohistochemical examination of a biopsy specimen was compatible with the diagnosis of leiomyosarcoma. There was no evidence of metastatic disease. An amputation of the index was performed.

Introduction: Immunodeficient patients, including the recipients of solid organs, exhibit an increase in the incidence of neoplasms. Post-transplant smooth muscle tumor (PTSMT) is a distinct and infrequent entity of these groups of neoplasms. Epstein–Barr virus (EBV) is considered to be involved in the etiology of this neoplasm. Case report: A 28-year-old man who underwent liver transplantation presented with abdominal pain and diarrhea for several months. He had a history of resistant systemic cytomegalovirus (CMV) infection after transplantation. Radiologic evaluation and colonoscopy revealed multiple liver, spleen, lung, and colon lesions. Microscopic assessment of colon and liver lesions using IHC study were in favor of spindle cell proliferation with mild atypia and a mild increase in mitotic rate without any necrosis, with features of smooth muscle tumor. Considering the transplantation history, EBER chromogenic in situ hybridization (CISH) study on paraffin blocks was requested, which demonstrated EBV RNA in tumor cell nuclei, suggesting EBV-associated smooth muscle tumor. In addition, PCR for CMV on paraffin blocks was positive. PCR for EBV and CMV viremia were negative. The dosage of immunosuppressive agents was reduced, and currently, he is being followed, with slow expansion in the size of the lesions. Conclusion: Although the incidence of post-transplant smooth muscle tumors (PTSMTs) is low, it should be remained in the differential diagnosis in post-transplantation patients, especially dealing with multifocal tumors. As strong stimulant for smooth muscle tumors, close follow-up and screening for EBV and CMV infection and early treatment at the time of diagnosis are recommended to avoid these virus-induced tumors. [ABSTRACT FROM AUTHOR]

Posttransplant smooth muscle tumors (PTSMTs) are rare Epstein–Barr virus (EBV)-associated neoplasms, mostly occurring after solid organ transplantation. Current therapeutic strategies include surgery and reduction of immunosuppressive medication. We describe for the first time a novel treatment approach for PTSMT by adoptive cell transfer (ACT) of EBV-specific T cells to a 20-year-old patient with a medical history of cardiac transplantation, posttransplant lymphoproliferative disease, and multilocular PTSMT. During ACT, mild cytokine release syndrome occurred, while no unexpected safety signals were recorded. We observed in vivo expansion of EBV-specific T cells and reduction of EBV viremia. Best response was stable disease after 4 months with reduction of EBV viremia and normalization of lactate dehydrogenase levels. ACT with EBV-specific T cells may be a safe and efficacious therapeutic option for PTSMT that warrants further exploration. [ABSTRACT FROM AUTHOR]

Primary intracranial smooth muscle tumors are rare. Most cases are related to Epstein-Barr virus proliferation in immunocompromised patients such as organ solid recipients. Only a few cases have been reported in pediatric patients. The clinical features are very variable depending mainly on the location and size of the smooth muscle tumor (SMT) and the pathogenesis is poorly understood. We describe two cases of intracranial SMT localized in the temporal lobe and associated with EBV in immunosuppressed children. A review of the literature associated with intracranial leiomyomas was also done. [ABSTRACT FROM AUTHOR]

Background: Epstein‐Barr virus (EBV) is a DNA virus with oncogenic potential, especially in immunocompromised patients. EBV can promote smooth muscle proliferation, resulting in EBV‐associated smooth muscle tumors (EBV‐SMT). Methods: We report a case of a 10‐year‐old child with end‐stage renal disease secondary to hypoplastic crossed and fused kidneys who underwent kidney transplantation. EBV serology was unknown for the donor and negative for the recipient; three months after he had a primary EBV infection. Two years after the transplantation, percutaneous nephrostomy was performed because of a drop in the estimated glomerular filtration rate and severe dilatation of the graft. Nephrography showed contrast enhancement of the pelvis of the graft kidney and proximal ureter, with a clear blockage at the level of the mid ureter and no passage towards the bladder. A 1.5‐cm tumor was found causing intraluminal compression of the mid ureter. Results: Complete resection of the tumor and distal ureter was performed leaving a short proximal ureter. A tension‐free uretero‐ureteroanastomoses was achieved using the native ureter. There were no surgical complications. Histologic evaluation showed spindle‐shaped muscle cells, moderate pleomorphism, and inflammatory infiltration. Immunohistochemical staining was positive for muscle‐specific actin. Epstein‐Barr encoding region (EBER) in situ hybridization was positive, confirming the diagnosis of EBV‐associated SMT. Conclusions: EBV–SMT is an exceedingly rare oncological entity that may develop in either the graft or any other organ. The clinical findings are location related. EBV seroconversion following transplantation might be a risk factor for the development of SMT in solid organ recipients. [ABSTRACT FROM AUTHOR]

Laryngeal mesenchymal neoplasms are rare, with smooth muscle tumors comprising a small subset. Specifically, Epstein-Barr virus (EBV)-associated smooth muscle tumors are exceptionally rare, lacking a comprehensive evaluation of their clinical and histologic features. Two patients (a 59 year old male and 51 year old female) had received renal transplants 156 and 240 months, respectively prior to onset of laryngeal symptoms. Supraglottic polypoid masses were identified and removed conservatively. Histologically, the tumors were hypercellular, showing alternating light and dark areas, the latter composed of primitive appearing round cells, while a more characteristic spindled tumor cell population was noted in the remaining areas. Cytoplasmic vacuoles were noted adjacent to the nucleus. There was no tumor necrosis or pleomorphism, but increased mitotic figures (11–12/2 mm2) were seen, without atypical forms. The tumor cells were strongly immunoreactive with smooth muscle actin and smooth muscle myosin heavy chain and with Epstein-Barr virus encoded RNA (EBER) by in situ hybridization. These patients were reviewed in the context of a thorough English literature review, which demonstrates a wide age range at presentation without a sex predilection, but with most patients from specific ethnic groups (Chinese, Thai, Pilipino). Three-quarters of patients are part of multifocal disease and the majority are post-renal transplantation patients. Conservative management seems to yield the best overall outcome for these indolent tumors. In conclusion, EBV-associated smooth muscle tumors should be considered in any immunocompromised patient with a head and neck smooth muscle tumor, especially when EBER is documented by in situ hybridization. Conservative management may be employed, even when multifocal tumors are documented. [ABSTRACT FROM AUTHOR]

Objective: Cutaneous smooth muscle tumors represent a rare group of cutaneous lesions including piloleiomyoma, angioleiomyoma, genital leiomyoma, smooth muscle hamartoma, and leiomyosarcoma. In this study, we aimed to evaluate the clinical and pathological characteristics of CSMTs, focusing on the rare and unspecified histological features. Material and Method: The clinical, demographic and histological findings of the patients with CSMTs were reviewed and evaluated retrospectively. The histopathological sections were re-evaluated for all cases. Results: A total of 32 patients with CSMTs were enrolled. The majority were female (n=20). The most common tumor diagnosed was angioleiomyoma (n=19, 59.4%) followed by piloleiomyoma (n=8, 25%), smooth muscle hamartoma (n=2, 6.3%), leiomyosarcoma (n=2, 6.3%), and genital leiomyoma (n=1, 3%). Five lesions were painful and only 3 specimens were submitted with the preliminary diagnosis of a cutaneous smooth muscle tumor. Conclusion: There are very few studies investigating both clinical and histological characteristics of CSMTs in detail. Along with the classical histological clues, evaluation of the clinical findings and less-defined histological features may enhance the diagnostic accuracy. To the best of our knowledge, this study represents the first original study focusing on the clinical and pathological aspects of CSMTs in our country.

Angioleiomyoma (vascular leiomyoma) is a common benign smooth muscle tumor, often affecting the soft tissue of the extremities, head, and neck regions. However, testicular involvement is an exceedingly rare event. Herein, we present an unusual case of testicular angioleiomyoma. To the best of our knowledge, this is the third reported case in English literature.

A rare case of spindle cell tumor of low histologic grade, smooth muscle origin in mandible of 11 year old girl is reported together with the conventional histologic and immunohistochemical findings. The tumor was in left retromolar region showing rapid increase in size and clinical features suggestive of malignancy. However on histopathologic evaluation it was diagnosed as Benign Spindle Cell tumor of possibly smooth muscle or fibroblastic origin. On Immunohistochemical evaluation the tumor cells were positive for Desmin, Smooth muscle actin but negative for S100. The Ki67 labelling index was 10–15%.The diagnosis was supported by the mitotic rate and Ki67.

Posttransplant smooth muscle tumors (PTSMTs) are rare Epstein–Barr virus (EBV)-associated neoplasms, mostly occurring after solid organ transplantation. Current therapeutic strategies include surgery and reduction of immunosuppressive medication. We describe for the first time a novel treatment approach for PTSMT by adoptive cell transfer (ACT) of EBV-specific T cells to a 20-year-old patient with a medical history of cardiac transplantation, posttransplant lymphoproliferative disease, and multilocular PTSMT. During ACT, mild cytokine release syndrome occurred, while no unexpected safety signals were recorded. We observed in vivo expansion of EBV-specific T cells and reduction of EBV viremia. Best response was stable disease after 4 months with reduction of EBV viremia and normalization of lactate dehydrogenase levels. ACT with EBV-specific T cells may be a safe and efficacious therapeutic option for PTSMT that warrants further exploration.

Leiomyomas are smooth muscle tumors that are commonly present in premenopausal women. These tumors are benign and of monoclonal origin. Peritoneal cavity leiomyomas are commonly reported in females and rarely reported in males. Here, we present a 58-year-old male who presented to the emergency room with abdominal pain. Computed tomography (CT) scan of the abdomen revealed multiple well-circumscribed left lower quadrant mesenteric masses containing heterogeneous attenuation and macroscopic fat. Exploratory laparotomy performed following abdominal CT and subsequent CT-guided biopsy revealed two intra-abdominal masses. Histopathological evaluation was positive for desmin and caldesmon immunohistochemical stains, and negative for C-kit, consistent with benign leiomyomata. This case highlights a benign leiomyoma within the abdominal cavity, which is an extremely rare occurrence and a potentially rare cause of abdominal pain., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Patel et al.)

We aimed to develop an artificial intelligence (AI) diagnosis system for uterine smooth muscle tumors (UMTs) by using deep learning. We analyzed the morphological features of UMTs on whole-slide images (233, 108, and 30 digital slides of leiomyosarcomas, leiomyomas, and smooth muscle tumors of uncertain malignant potential stained with hematoxylin and eosin, respectively). Aperio ImageScope software randomly selected ≥10 areas of the total field of view. Pathologists randomly selected a marked region in each section that was no smaller than the total area of 10 high-power fields in which necrotic, vascular, collagenous, and mitotic areas were labeled. We constructed an automatic identification algorithm for cytological atypia and necrosis by using ResNet and constructed an automatic detection algorithm for mitosis by using YOLOv5. A logical evaluation algorithm was then designed to obtain an automatic UMT diagnostic aid that can “study and synthesize” a pathologist’s experience. The precision, recall, and F1 index reached more than 0.920. The detection network could accurately detect the mitoses (0.913 precision, 0.893 recall). For the prediction ability, the AI system had a precision of 0.90. An AI-assisted system for diagnosing UMTs in routine practice scenarios is feasible and can improve the accuracy and efficiency of diagnosis.

Ki67 labeling index (LI) has been proposed as a prognostic factor in uterine leiomyosarcoma (uLMS), although the evidence in this field is still unclear. We aimed to assess the prognostic value of ki67 LI in uLMS. A systematic review was performed by searching electronic databases from their inception to August 2020 for all studies assessing the prognostic value of ki67 LI in uLMS. Ki67 LI was assessed to the nearest 10% to define the most prognostically accurate threshold. Cox regression survival analysis with calculation of hazard ratio (HR) of death was performed; a p-value < 0.05 was considered significant. Ten studies were included in the qualitative review, out of which 6 were suitable for quantitative review. The absolute risk of death was 0.29 for a ki67 LI < 10%, remained stable at 0.49 in the 10%-39% LI range and increased to 0.65 for a LI ≥ 40%. On univariate analysis, both 10% and 40% thresholds were significantly associated with the hazard of death, with HRs of 3.349 (p = 0.007) and 3.172 (p = 0.001), respectively. On multivariate analysis, only the 10% threshold was significantly associated with the hazard of death (HR = 2.712; p = 0.028). In conclusion, a Ki67 LI ≥ 10% is a significant prognostic factor in uLMS. [ABSTRACT FROM AUTHOR]

Epstein-Barr Virus (EBV) has been linked to various human malignancies. EBV-related smooth muscle cell tumors (EBV-SMT) are rare form of those malignancies that commonly affect patients with immunodeficiency. EBV-SMT usually arises from lungs, livers or soft tissues.We present a case of a 12-year-old immunocompetent female with a mediastinal mass, which was found to be related to EBV-SMT. The mass was managed successfully with a thoracoscopic resection. The patient is tumor and symptoms -free at 2 years follow-up.

Herein, we reported a rare case of Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) combined with juvenile idiopathic arthritis (JIA) in a 6-year old girl without HIV, organ transplantation, or congenital immunodeficiency. The patient suffered from pain in the bilateral hip joints, which drastically affected her physical activity. Consequently, she was diagnosed with JIA (September 2019). She was given methotrexate and methylprednisolone pills via oral route and a subcutaneous injection of Recombinant Human Tumor Necrosis Factor-α Receptor II;lgG Fc Fusion Protein for 4 weeks that successfully relieved the pain. In May 2020, the pain reoccurred and was accompanied by occasional headaches. After extensive pathological examination, the patient was diagnosed with EBV-SMT. The imaging examinations after admission showed multiple lesions in the skull, lungs, and vertebral body. Biopsy of the L2 vertebral body was then performed to clarify the diagnosis. Finally, the in-situ hybridization of the tumor of the lumbar vertebrae suggested a non-HIV/transplantation-related EBV-SMT. Consequently, the patient received surgery without chemotherapy and radiotherapy, after which her conditions improved.