Epstein-Barr Virus-Associated Smooth-Muscle Tumor of the Brain.

Epstein-Barr virus, a herpesvirus, has been associated with a variety of cancers, including Burkitt, Hodgkin, and non-Hodgkin lymphomas; posttransplant lymphoproliferative disorders; gastric carcinoma; and nasopharyngeal carcinoma, in both immunocompetent and immunocompromised individuals. Previous studies have established a connection between Epstein-Barr virus and the development of smooth-muscle tumors. Smooth-muscle tumors of the brain are very rare and are often misdiagnosed as meningiomas on imaging. To our knowledge, advanced imaging findings such as MR perfusion of smooth-muscle tumors of the brain have never been reported. We describe the radiologic and pathologic features of the Epstein-Barr virus-associated smooth-muscle tumors of the brain in a person with newly diagnosed advanced HIV.

PET/CT in a 65-Year-Old Woman With Epstein-Barr Virus-Associated Smooth Muscle Tumor After Chemotherapy for Follicular Lymphoma.

ABSTRACT: The Epstein-Barr virus-associated smooth muscle tumor (SMT) is an uncommon neoplasm. It arises mainly in 3 immunosuppression settings: HIV-associated SMT; drug-related immunosuppression in transplant recipients; and congenital immunodeficiency disorder-associated SMT. We present 18 F-FDG PET/CT findings of an adrenal Epstein-Barr virus-associated SMT in a 65-year-old woman with a history of follicular lymphoma after chemotherapy.

Smooth muscle tumours of the uterus: MR imaging malignant predictive features-a 12-year analysis in a referral hospital in Portugal.

PURPOSE: To evaluate the magnetic resonance imaging (MRI) features that may help distinguish leiomyosarcomas from atypical leiomyomas (those presenting hyperintensity on T2-W images equal or superior to 50% compared to the myometrium). MATERIALS AND METHODS: The authors conducted a retrospective single-centre study that included a total of 57 women diagnosed with smooth muscle tumour of the uterus, who were evaluated with pelvic MRI, between January 2009 and March 2020. All cases had a histologically proven diagnosis (31 Atypical Leiomyomas-ALM; 26 Leiomyosarcomas-LMS). The MRI features evaluated in this study included: age at presentation, dimension, contours, intra-tumoral haemorrhagic areas, T2-WI heterogeneity, T2-WI dark areas, flow voids, cyst areas, necrosis, restriction on diffusion-weighted imaging (DWI), apparent diffusion coefficient (ADC) values, signal intensity and heterogeneity after contrast administration in T1-WI, presence and location of unenhanced areas. The association between the MRI characteristics and the histological subtype was evaluated using Chi-Square and ANOVA tests. RESULTS: The MRI parameters that showed a statistically significance correlation with malignant histology and thus most strongly associated with LMS were found to be: irregular contours (p < 0.001), intra-tumoral haemorrhagic areas (p = 0.028), T2-WI dark areas (p = 0.016), high signal intensity after contrast administration (p = 0.005), necrosis (p = 0.001), central location for unenhanced areas (p = 0.026), and ADC value lower than 0.88 × 10-3 mm2/s (p = 0.002). CONCLUSION: With our work, we demonstrate the presence of seven MRI features that are statistically significant in differentiating between LMS and ALM.

Quantitative MR texture analysis for the differentiation of uterine smooth muscle tumors with high signal intensity on T2-weighted imaging.

The purpose of this study was to distinguish leiomyosarcomas/smooth muscle tumors of uncertain malignant potential (STUMP) from leiomyomas with high signal intensity (SI) on T2-weighted imaging (T2WI) using quantitative MR texture analysis combined with patient characteristics and visual assessment. Thirty-one leiomyomas, 2 STUMPs, and 6 leiomyosarcomas showing high SI on T2WI were included. First, we searched for differences in patient characteristics and visual assessment between leiomyomas and leiomyosarcomas/STUMPs. We also compared the MR texture on T2WI and the apparent diffusion coefficient (ADC) to identify differences between leiomyomas and leiomyosarcomas/STUMPs. In the univariate analysis, significant differences between leiomyomas and leiomyosarcomas/STUMPs were observed in age, menopausal status, margin, hemorrhage, long diameter, T2-variance, T2-volume, ADC-variance, ADC-entropy, ADC-uniformity, ADC-90th and 95th percentile values, and ADC-volume (P < .05, respectively). There were significantly more postmenopausal patients with leiomyosarcomas/STUMPs than with leiomyomas, and leiomyosarcomas/STUMPs had more irregular margins, more frequent presence of hemorrhage and exhibited larger tumor diameters, T2-volume, T2-variance, ADC-volume, ADC-variance, ADC-entropy, and higher ADC-90th and 95th percentile values but lower ADC-uniformity. Multivariate analyses revealed that the independent differentiators were menopausal status, hemorrhage and ADC-entropy (P < .05, respectively). The area under the curve obtained by combining the 3 items was 0.980. The best cutoff value for ADC-entropy was 9.625 (sensitivity: 100%, specificity: 58%). The combination of menopausal status, hemorrhage, and ADC-entropy can help accurately distinguish leiomyosarcomas/STUMPs from leiomyomas with high SI on T2WI; however, external validation in a larger population is required because of the small sample size of our study.

Smooth Muscle Tumors of the Uterus at MRI: Focus on Leiomyomas and FIGO Classification.

Leiomyomas are smooth muscle tumors of the uterus and are the most common uterine neoplasm. Although leiomyomas are usually asymptomatic, they can manifest with symptoms such as pain or uterine bleeding. Leiomyomas are classified on the basis of their anatomic location and morphology. Localization of leiomyomas relative to the endometrium, myometrium, and uterine serosa with use of the International Federation of Gynecology and Obstetrics (FIGO) classification system is helpful for guiding management in symptomatic patients. The FIGO system is a practical and universally accepted approach for classifying leiomyomas to guide radiologists and clinicians in deciding management. The MRI appearance of conventional leiomyomas is related to their tissue contents of smooth muscle and fibrous tissue and is well established. The MRI features of some leiomyoma subtypes and forms of degeneration also have been described. Other smooth muscle tumors of the uterus recognized in the 2020 World Health Organization classification system include intravenous leiomyomatosis, smooth muscle tumors of uncertain malignant potential, and metastasizing leiomyoma. At the far end of the spectrum are leiomyosarcomas, which are frankly malignant and therefore must be managed accordingly. Although MRI features that suggest a diagnosis of leiomyosarcoma have been proposed, these features overlap with those of some leiomyoma subtypes and degeneration. © RSNA, 2023 See the invited commentary by Fennessy and Gargiulo in this issue. Online supplemental material and the slide presentation from the RSNA Annual Meeting are available for this article. Quiz questions for this article are available through the Online Learning Center.

A Transanal Endoscopic Ultrasound-guided Fine-needle Aspiration Biopsy for an Intrapelvic Tumor Diagnosed as Recurrence of a Smooth Muscle Tumor of Uncertain Malignant Potential Following Uterine Morcellation.

A transoral endoscopic ultrasound-guided fine-needle aspiration biopsy (EUS-FNAB) is a well-established tissue-sampling method. However, performing a transanal EUS-FNAB remains challenging. Uterine morcellation has emerged as a minimally invasive approach for benign tumor treatment. However, uterine myomas are heterogeneous and include malignant and indeterminate malignant cells. We herein report a rare case of intrapelvic tumor diagnosed by a transanal EUS-FNAB as a recurrence of smooth muscle tumors of uncertain malignant potential following uterine morcellation. Physicians should be aware that a previous uterine myoma resected under morcellation has the possibility of intra-abdominal recurrence. A transanal EUS-FNAB is a practical option for making a pathological diagnosis.

18F-FDG PET/CT imaging of multiple intrahepatic Epstein-Barr virus-associated smooth muscle tumors in a pediatric patient after heart transplantation.

Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is an exceedingly rare neoplastic disease with a predisposition in immune-compromised individuals, especially in patients with prior transplantation, human immunodeficiency virus infection, or congenital immunodeficiency. Here, we present imaging findings of EBV-SMT in multiphasic contrast-enhanced computed tomography (CT) and fluorine-18-fluorodeoxyglucose (18F-FDG) positron emission tomography (PET)/CT in a two-and-a-half-year-old boy with prior heart transplantation.

Sonographic characterization and surveillance of paravaginal smooth muscle tumor of uncertain malignant potential.

Sonographic characterization and surveillance of paravaginal smooth muscle tumor of uncertain malignant potential. (A1) Transvaginal ultrasound with probe placed over the right vaginal wall, showing a well-defined round mass with regular contours, a mostly hypoechoic and heterogeneous echotexture, and edge shadowing, deep to the right distal third of the right vagina. (A2) Multifrequency linear probe (9-14 MHz) placed over the right labium majus revealing hyperechoic striations (arrows on A1-A2) and central flow (arrowheads on A2). (B1) Resected solid white-tan mass of bland consistency. (B2) Hematoxylin-eosin microscopy (40X) showing fusiform cells, with mild to moderate atypia. (C1) Repeat transvaginal ultrasound six-years later showing a recurrent solid oval-shaped mass with regular contour, a mostly hypoechoic heterogeneous echotexture, and an anechoic area inside the solid mass (asterisk on C2) that could represent a focus of necrosis.

Mixed Endometrial Stromal and Smooth Muscle Tumor of the Uterus with Unusual Morphologic Features in a 35-Year-Old Nulliparous Woman: A Case Report.

BACKGROUND Mixed endometrial stromal and smooth muscle tumors (MESSMT) of the uterus are rare disease entities. The histogenesis is unclear, but its clinical manifestations are similar to those of other mesenchymal tumors. This unique uterine tumor was originally reported as having ultrastructural characteristics of both endometrial stromal and smooth muscle cells. Subsequently, MESSMT was defined as having at least 30% of each component present. Here we present an MESSMT with unusual features in a nulliparous woman and describe its morphological and immunohistochemical characteristics. CASE REPORT A 35-year-old nulliparous woman presented with menorrhagia for 2 months. Transvaginal ultrasonography and magnetic resonance imaging revealed an enlarged uterus with a 6.0×5.5×4.5 cm mass and cystic degeneration. The patient underwent abdominal mass excision. Microscopically, the tumor consisted of 3 distinct components. The outermost area consisted of smooth muscle cells. Well-differentiated endometrial stromal cells that were centrally located showed irregular borders and were merged with smooth muscle cells. In addition, the endometrial stromal component showed focal sex-cord-like differentiation. Morphological and immunohistochemical evaluations were performed, and a MESSMT with focal sex-cord-like differentiation was diagnosed. The patient's postoperative course was uneventful for 29 months. CONCLUSIONS The diagnosis of MESSMT is challenging due to its many overlapping features with other mesenchymal uterine tumors. Although this rare tumor was histologically and clinically consistent with low-grade endometrial stromal sarcoma, it can cause recurrence and metastasis. Therefore, regular follow-up with radiologic examination is essential for the timely detection of local recurrence and distant metastasis.

Ultrasound features of highly vascularized uterine myomas (uterine smooth muscle tumors) and correlation with histopathology.

OBJECTIVE: To correlate the ultrasound appearance of highly vascularized uterine myomas with their histopathological diagnosis. METHODS: This was a prospective observational study of patients with a preoperative ultrasound diagnosis of a highly vascularized uterine myoma (color score of 3 or 4, according to the Morphological Uterus Sonographic Assessment (MUSA) criteria), characterized by circumferential and intralesional vascular pattern, who underwent myomectomy or hysterectomy. For each patient, ultrasound characteristics were recorded at baseline, including the number of lesions, the size, echogenicity and border regularity of the lesion, presence of cystic areas and shadowing within the myoma, and visualization of the endometrium. Ultrasound features were correlated with the definitive histological diagnosis. Ultrasound features were then compared between malignant and benign lesions. RESULTS: We included 70 patients with highly vascularized uterine myomas on power/color Doppler. Their mean age was 46.5 ± 11.4 years and 13 (18.6%) were postmenopausal. At histological examination, 65 (92.9%) uterine myomas were benign lesions, comprising 32 typical leiomyomas, 29 leiomyoma variants and four adenomyomas. The remaining five (7.1%) uterine myomas were malignant masses, comprising two uterine sarcomas, one leiomyosarcoma, one neuroendocrine tumor and one uterine smooth muscle tumor of uncertain malignant potential (STUMP). The mean age of patients with a malignant lesion was significantly higher than the age of those with a benign lesion (64.8 ± 16.0 vs 42.4 ± 5.1; P < 0.001). Four out of five patients with a malignant lesion were over 45 years old. Ultrasound demonstrated cystic areas within the lesion in 10/32 (31.3%) typical leiomyomas, 16/29 (55.2%) leiomyoma variants, all four adenomyomas and in the cases of STUMP and leiomyosarcoma. Lesion borders were regular in 64/65 (98.5%) benign lesions and 2/5 (40%) malignant lesions (P < 0.05). No significant differences were observed between benign and malignant lesions with respect to echogenicity, presence of shadowing and size. The endometrium was visible in 55/65 women with benign lesions and in 2/5 with malignant lesions (P = 0.03). CONCLUSIONS: Our results showed that ultrasound features of uterine myomas, such as circumferential and intralesional vascularity, cystic areas and lesion borders, are important parameters for differential diagnosis, especially when combined with the patient's age. Such features could be useful to differentiate typical myomas from benign variants and malignant lesions in a preoperative setting and to select patients that may benefit from conservative management rather than surgery. © 2022 International Society of Ultrasound in Obstetrics and Gynecology.

Transcervical Transtracheal Resection of Cervical Esophageal Smooth Muscle Tumor.

Cervical esophageal smooth muscle tumors are traditionally resected via lateral transcervical with or without video-assisted thoracoscopic approaches. Exposure is frequently limited, however, with risks of recurrent laryngeal nerve and posterior tracheal wall injury and jeopardization of cervical tracheal and cervical esophageal blood supply. We herein describe an anterior transcervical transtracheal approach to counter some of the aforementioned problems and avoid morbidities associated with thoracoscopic surgery when resecting smooth muscle tumors arising from the cervical esophagus.

Two cases of nodular smooth muscle proliferation suspected of primary lung cancer from preoperative images: a case report.

BACKGROUND: It is difficult to obtain a definitive diagnosis for nodular smooth muscle proliferation (NSMP) before surgery, and a pathological diagnosis is necessary to differentiate it from primary lung cancer. We report two cases of NSMP that were suspected to be primary lung cancer on preoperative images. CASE PRESENTATION: Case 1: An 81-year-old man who had undergone right upper lobectomy for lung cancer 2 years earlier was point out a nodular shadow with ground glass opacity (GGO) in the lower right lobe, suggesting a second primary lung cancer by chest computed tomography (CT). A thoracoscopic partial resection of the right lower lobe was performed, and pathological diagnosis was NSMP. The patient was discharged without any problems at 3 days postoperatively. Case 2: A 72-year-old woman was pointed out a nodular shadow suspected primary lung cancer in the left lower lobe by chest CT. Therefore, thoracoscopic partial resection of the left lower lobe was performed, and pathological diagnosis was NSMP. The patient was discharged without any problems at 5 days postoperatively. CONCLUSION: This report demonstrates that NSMP can be distinguished from leiomyoma and hamartoma by imaging features and pathological findings.

Mesenchymal neoplasms of the tubular gut and adjacent structures: experience with EUS-guided fine-needle aspiration cytopathology.

INTRODUCTION: Unlike epithelial malignancies, mesenchymal neoplasms arising within the tubular gut are less often encountered in endoscopic ultrasound-guided (EUS) fine-needle aspiration biopsies (FNABs). Nonetheless, preoperative diagnosis of such neoplasms has important therapeutic and prognostic value. We report our experience with this category of neoplasms from the past decade. MATERIALS AND METHODS: We performed a 10-year retrospective search at our respective institutions to identify EUS-guided FNAB cases of mesenchymal neoplasms arising from the tubular gut wall and closely adjacent structures. Cytopathologic diagnoses were compared to corresponding surgical pathology (SP) when available. Cases with either no confirmatory cell block (CB) immunohistochemical (IHC) staining, or no SP were excluded. RESULTS: Two-hundred eighty-two cases (M:F = 1:1; age range: 25-94 years, mean age = 60 years) of EUS-guided FNAB from the tubular gut met our criteria. Onsite adequacy was performed on nearly all cases. Case numbers: 209 gastrointestinal stromal tumors (GIST), 58 smooth muscle neoplasms, and 15 miscellaneous neoplasms. Of these, 188 (67%) had SP follow-up. We found that 258 (91%) aspirates had a correct specific diagnosis, 3 (1%) were nondiagnostic, 18 (6%) had indeterminate diagnoses, and 3 (1%) had incorrect diagnoses (2 leiomyosarcomas mistaken as leiomyoma, and 1 fibrosclerotic lesion mistaken as inflammatory pseudotumor). Of 94 cases with no SP, all had a specific cytologic diagnosis based on confirmatory IHC staining from the CB including 61 GISTs, 29 smooth muscle neoplasms, and 4 miscellaneous tumors. CONCLUSION: This study endorses the clinical utility of EUS-guided FNAB in the diagnosis of tubular gut mesenchymal neoplasms. A definitive and accurate diagnosis is possible in over 90% of cases, chiefly when cytomorphology is coupled with optimal cellularity and IHC from a concurrent CB. EUS-guided FNAB diagnosis of mesenchymal tubular gut neoplasms may play an important role in determining neoadjuvant therapy as targeted therapy evolves.

Ultrasound and clinical characteristics of uterine smooth muscle tumors of uncertain malignant potential (STUMPs).

OBJECTIVE(S): Smooth muscle tumors of uncertain malignant potential are rare uterine neoplasms. Their identification through imaging is still limited due to the few available descriptions in the scientific literature. The objective of this paper is to provide clinical and ultrasound features that could support an early identification of these neoplasms. STUDY DESIGN: We retrospectively evaluated preoperative sonographic data of patients receiving a histopathological diagnosis of smooth muscle tumors of uncertain malignant potential between 2014 and 2019 at the S. Anna Hospital (Turin, Italy), a tertiary gynecological center. Tumors were characterized on the basis of ultrasound images using terms and definitions according to the morphological uterus sonographic assessment group. RESULTS: A total of fourteen patients with smooth muscle tumors of uncertain malignant potential (20 lesions, including 18 pure and 2 with associated leiomyosarcoma) were identified. The median age was 47 years (range 28-77) and nine (64%) patients were of reproductive age. Six patients (43%) were asymptomatic, two (14%) presented with abdominal pain, two (14 %) with menorrhagia and four (29%) with both symptoms. Two (14%) patients developed local recurrences as uterine smooth muscle tumor of uncertain malignant potential and leiomyosarcoma, respectively. At ultrasound imaging, nine (69%) smooth muscle tumors of uncertain malignant potential were poorly or moderately vascularized and nine (82%) showed both circumferential and intra-lesional flows. Only three (15%) showed shadowing. The outlines were well-defined in seventeen cases (85%) and most (90%) showed isoechoic or mixed echogenicity with microcystic anechoic areas in fourteen (70%) of cases. CONCLUSION(S): Sonographic features of smooth muscle tumors of uncertain malignant potential may vary and a pathognomonic description has not been recognized. However, the identification of single or multiple lesions with specific ultrasound features should raise the suspicion of tumors of uncertain malignant potential. These features include isoechogenicity or mixed echogenicity, regular borders, presence of internal microcystic and anechoic areas, circumferential and intralesional vascularization ranging from minimal to high and absence of shadowing.

Rare Epstein-Barr Virus-Associated Smooth Muscle Tumor in a Patient with AIDS: A Case Report.

CASE: A 34-year-old man with poorly controlled acquired immune deficiency syndrome underwent excision of a left arm mass. The histopathologic workup identified the features of an Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT). The patient was readmitted 5 months later for vomiting and found to have liver metastases that were confirmed to be EBV-SMT. Six months after discharge, there was no recurrence of the arm mass or increase in the size of the liver metastases. CONCLUSION: Most commonly found in immunocompromised patients, EBV-SMTs are rare tumors that can be mistaken for a leiomyosarcoma.

Epstein-Barr Virus-Associated Smooth Muscle Tumor of the Spine After Bone Marrow Transplant: Case Report and Review of Literature.

BACKGROUND: Epstein-Barr virus-associated smooth muscle tumors (SMTs) are rare neoplasms that have been found to develop in immunocompromised patients. Three distinct groups of affected patients have been described: (1) human immunodeficiency virus-infected patients, (2) post-transplant patients, and (3) patients with congenital immunodeficiency. The tumors can develop anywhere in the body, with 17 reported cases occurring in the spinal canal, all in patients with human immunodeficiency virus infection. CASE DESCRIPTION: We report the first case of Epstein-Barr virus-associated SMT affecting the spinal canal in a post-bone marrow transplant adult patient. Interestingly, unlike other reported cases, the patient described here had not been receiving immunosuppressive therapy in the 2 years prior to diagnosis of the tumor. CONCLUSIONS: Despite the growing number of case reports, this diagnosis presents a challenge, as the pathophysiology and optimal treatment regimens are not well understood. Results of a literature review of Epstein-Barr virus-associated SMT of the spine as well as a discussion of the presentation, management, and prognosis of this condition is presented here.