Epstein-Barr virus associated smooth muscle tumors in solid organ transplant recipients: Incidence over 31 years at a single institution and review of the literature.

INTRODUCTION: Epstein-Barr virus (EBV) associated smooth muscle tumors (EBV-SMT) are a rare complication of solid organ transplantation (SOT). Incidence data related to this EBV-SMT are limited. EBV DNA is universally present in these tumors. How these cells get infected with EBV, whether this is a result of primary EBV infection vs reactivation, and how persistent active EBV infection post-transplant influences EBV-SMT pathogenesis remains unknown. METHODS: Among 5006 SOT recipients (474 pediatric, 4532 adult) receiving SOT at our center between Jan 1984 and Dec 2015, three cases of post-transplant EBV-SMT were identified. RESULTS: All cases were pediatric heart transplants who were EBV seronegative prior to transplant, and experienced primary EBV infection with persistently elevated EBV viral loads, despite antiviral therapy. Two are deceased at 3.2 and 0.9 years post-diagnosis, while one remains alive 6.2 years post diagnosis. The overall local incidence of post-transplant EBV-SMT at our institution was 0.7 (95% CI, 0.2-1.7) per 1000 patient years, and 2.6 (95% CI, 0.6-6.7) per 1000 patient years in pediatric heart transplants. A literature review identified 36 pediatric and 51 adult cases of post-transplant EBV-SMT. CONCLUSIONS: We hypothesize that pre-transplant EBV seronegativity, followed by primary EBV infection and persistently high EBV viral loads, represents a unique risk factor for post-transplant EBV-SMT. Pediatric heart transplant recipients were found to be disproportionately affected by post-transplant EBV-SMT at our institution.

Safety of Manual Morcellation After Vaginal or Laparoscopic-assisted Vaginal Hysterectomy.

UNLABELLED: STUDY OBJECTIVE: To determine the safety of manual vaginal morcellation by evaluating the rates of incidental uterine malignancy and manual vaginal morcellation after vaginal or laparoscopic-assisted vaginal hysterectomy. DESIGN: Retrospective analysis (Canadian Task Force classification II-2). SETTING: University of Texas Southwestern Medical Center, Dallas, TX. PATIENTS: Women (n = 1,629) undergoing vaginal or laparoscopic-assisted vaginal hysterectomy. INTERVENTIONS: Vaginal hysterectomy (n = 1,091) or laparoscopic-assisted vaginal hysterectomy (n = 538) with and without scalpel morcellation. MEASUREMENTS AND MAIN RESULTS: The number of uterine malignancies, rate of vaginal morcellation, surgical indications, pathology diagnoses, and uterine weights were evaluated. Chi-square analysis was used to compare categoric data, and analysis of variance was used to compare uterine weights. There were no cases of leiomyosarcomas. There were 2 other sarcomas, 4 smooth muscle tumors of uncertain malignant potential, and 8 endometrial adenocarcinomas. The vaginal morcellation rate was 19.4%, but no malignancy was morcellated. Myomas were more common preoperatively and histologically in morcellated specimens. Mean (± standard deviation) uterine weights for morcellated versus nonmorcellated laparoscopic-assisted vaginal hysterectomy specimens were 285.5 ± 159.3 versus 140.1 ± 83.6 g (p < .001), respectively, and 199.9 ± 92.8 versus 111.9 ± 61.4 (p < .001), respectively, for vaginal hysterectomy. CONCLUSION: Vaginal manual morcellation is safe with a low risk of incidental malignancy. Variables that influence the decision for the vaginal approach may also affect malignancy risk and morcellation decisions. Thus, all patients undergoing vaginal or laparoscopic-assisted vaginal hysterectomy should be counseled regarding incidental malignancy, risk of morcellation, and alternatives for intact specimen removal.

Epstein-Barr virus-associated smooth muscle tumor-report of 3 tumors including 1 intracerebral case with a prominent intratumoral B-lymphocytic component and plasma cells.

Three Epstein-Barr virus-associated smooth muscle tumors (intracranial, endobronchial, and paraspinal) in 2 patients are presented. The patient with the intracranial tumor had no concurrent or previous manifestation of this neoplasm and was immunosuppressed because of a renal transplant. The other patient had AIDS and radiologic evidence of involvement of multiple other sites. Although mitotic activity was present, none of the tumors displayed any high-grade malignant histologic features. All tumors were strongly positive for smooth muscle actin and variably expressed h-caldesmon. Desmin was negative in all 3 tumors. The intracranial tumor was associated with prominent intracerebral edema and displayed, in addition to a prominent intratumoral T-cell component, a hitherto unreported component of prominent intratumoral B cells and plasma cells.

Unexpected uterine smooth muscle tumor of uncertain malignant potential and sarcoma: A single center cohort study in South Korea.

OBJECTIVE: To evaluate the risk of encountering unexpected uterine smooth muscle tumors of uncertain malignant potential (STUMPs) or sarcomas during surgical treatment of mesenchymal tumors of the uterus using morcellation. MATERIAL AND METHODS: Data were collected retrospectively from subjects who were pathologically diagnosed with uterine leiomyoma or its variants, STUMP or other premalignant mesenchymal tumors of uterus, or sarcoma during surgical treatment between July 2014 and June 2017. RESULTS: A total of 3785 women were investigated; 2824 laparoscopic procedures (74.6%) were performed, and an electronic power morcellator was used in 1636 patients (43.2%). Sixteen women (0.42%) were diagnosed with STUMP and 14 (0.37%) were diagnosed with uterine sarcoma. The incidence rate of unexpected STUMP or uterine sarcoma was 0.61% (23 of 3785 women); unexpected STUMP in 13 (0.34%), and unexpected sarcoma was in 10 (0.26%). Moreover, the unexpected leiomyosarcoma rate was 0.08% (3 in 3785). The rate of unintended morcellation of STUMPs was relatively high at 0.26% (10 in 3785), however, that for uterine sarcomas was 0.05% (2 in 3785). CONCLUSION: The risks of unintended morcellation were very low for sarcomas and STUMPs, although the risk of the latter was approximately 5-fold that of the former. To reduce the unintended dissemination of tumors, patients suspected of having malignancies should be provided adequate information regarding their treatment options as well as their associated risks. Meanwhile, improved preoperative screening methods for STUMP and sarcoma should be established.

Revision of gastrointestinal mesenchymal tumors.

BACKGROUND: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumor of the gastrointestinal (GI) tract. The advent of target therapy (imatinib mesylate) for GISTs increases the importance of pathologic diagnosis. The previous diagnosis with smooth muscle tumor (leiomyoma or leiomyosarcoma) and nerve sheath tumor (schwannoma) become GISTs after the study with CD117 immunohistochemistry accompanying conventional histologic study in many series. OBJECTIVE: To identify the incidence of GISTs in the patients who were previously diagnosed with smooth muscle or nerve sheath tumors. The histology and immunoreactivity of both newly found and previously diagnosed with GISTs are also studied MATERIAL AND METHOD: A retrospective database identified all patients seen from 1998 to 2006. Patients with mesenchymal tumors of the GI tract and intraabdominal extragastrointestinal tract were selected, 53 cases in total. Clinical and pathological data, treatment, and outcome were analyzed RESULTS: After revision, the total number of GISTs is 42 cases. There were 33 cases previously diagnosed with leiomyosarcoma that became the diagnosis with GISTs (31 cases or 93.9%), due to CD117 positivity. Most of GISTs cases had spindle cell type (26 cases, 61.9%) and only the colon and omentum had predominant mixed cell type. CONCLUSION: GISTs are the most common mesenchymal neoplasm of the stomach and small intestine and are relatively less frequent at other gastrointestinal sites. An increasing awareness of their histologic, immunophenotypic, and molecular features coupled with an evolving understanding of their histogenesis is facilitating our ability to identify these tumors.

Clinicopathologic evaluation of uterine smooth muscle tumors of uncertain malignant potential (STUMP): A single center experience.

OBJECTIVE: To investigate the clinical outcomes and histopathological features of uterine smooth muscle tumors of uncertain malignant potential (STUMP). METHODS: The study analysed cases diagnosed with uterine STUMP in a tertiary center, between January 2003 and September 2018. We investigated the clinical, operative and histopatholologic data of the cases. Follow-up information and clinical outcomes were also examined. RESULTS: 28 cases with uterine STUMP were studied. The mean age of the patients was 44.5 ± 9.0 years and the median parity was 2 (0-6). The mean tumor diameter was 6.3 cm (range 2-27 cm) and most (78.6%) of the tumors were located intramurally. In 25% of the cases diagnosis was after myomectomy, while in the others diagnosis was after hysterectomy. Of the patients who wanted to preserve the uterus and their fertility and who did not therefore undergo a subsequent hysterectomy, one patient became pregnant without any complication. One case with a history of myomectomy, presented as STUMP. The median follow-up time was 45.4 months (range 5-180). Recurrence occured in one case (3.7%) 33 months after diagnosis. Distant metastasis occurred in the lungs and the pathology of the biopsy was liposarcoma, and the patient died of the disease 62 months after diagnosis. CONCLUSION: Uterine STUMP is a rare condition, and diagnosis can be difficult, often with unusual combinations of findings. Prognosis for the patient is unclear and their is a risk of recurrence with the tumors. To reduce mortality, regular follow-up and a centralised approach are recommended.

A clinicopathologic review and obstetric outcome of uterine smooth muscle tumor of uncertain malignant potential (STUMP) in a single institution.

OBJECTIVES: The present study aimed to analyze the clinicopathologic features and treatment of uSTUMP in a single institution. In addition, we described the obstetric outcomes after uterine-preserving surgery for uSTUMP. METHODS AND MATERIALS: A retrospective chart review was performed of patients diagnosed with uSTUMP between January 2000 and February 2018 at the Comprehensive Gynecologic Cancer Center, CHA Bundang Medical Center. We obtained data on the patients' demographics, treatment, therapeutic results, time to recurrence, disease-free and overall survival, and subsequent obstetric outcomes. The central pathology reviews were carried out by two pathologists specializing in gynecologic oncology. RESULT: A total of 19 patients diagnosed with uSTUMP were identified and included in the study. The mean age at diagnosis was 41 years (range 28-49 years). Frozen sections were performed in 11 patients including five patients during surgery. Nine of the 19 patients (47.4%) were treated by hysterectomy and 10 patients were initially treated by myomectomy. The mean follow-up period was 47 months (range 6-209 months). Two patients (10.5%; 2/19) experienced recurrence. Although not defined as recurrence in the present study criteria, one patient had a secondary diagnosis of atypical leiomyoma one year after the initial diagnosis of uSTUMP. Seven patients requested uterine-preserving treatment, five of whom wanted to become pregnant. Three of them (3/5; 60%) successfully delivered live birth to full term by Cesarean section without complications such as abortion, preterm delivery or uterine rupture, and tumor recurrence. CONCLUSIONS: The present study describes the clinicopathologic data of uSTUMP patients. Our results suggest a uSTUMP recurrence of 10.5%, comparable to previous reports. Although there is a possibility of malignant recurrence, fertility-preserving management is worth attempting because of relatively low affected age with careful close follow-up.

Gastro-intestinal stromal tumours (GISTs): a six year Zimbabwean experience. Cellular differentiation with immunocytochemistry.

OBJECTIVES: To determine the prevalence of GISTs in general, the terminology in use, biological behaviour in terms of benign, borderline and malignant variants and assessment of the different lines of differentiation using immunochemical techniques. DESIGN: A retrospective study. SETTING: Department of Histopathology, University of Zimbabwe Medical School at Parirenyatwa and Harare Central teaching Hospitals. SUBJECTS: Patients with a surgical pathology diagnosis of gastro-intestinal related stromal tumours including leiomyosarcomas, leiomyomas and GISTs over a six year period. MAIN OUTCOME MEASURE: Evaluation for different lines of differentiation and malignant potential using immunocytochemical techniques. RESULTS: A total of 24 cases with a primary diagnosis of GISTs over a period of six years were retrieved from the records and of these 22 had material available for evaluation. Four of these were reported as pure GISTs, two of them benign and the other two malignant. Seven cases were reported as leiomyosarcomas. Six of these had evidence of neural differentiation on immunocytochemistry and therefore were strictly not leiomyosarcomas. Of the 11 cases reported as leiomyomas, four (36.4%) had neural differentiation on immunohistochemistry implying a low malignant potential. CONCLUSION: GISTs are not uncommon in this patient population considering that 24 cases were reported over a six year period in a department that averages 11,000 surgical pathology cases per year. Minimal use of immunostains, in addition to general morphological features, can be utilized to determine the line of cellular differentiation and malignant potential of the seemingly benign tumours.

Miscellaneous uterine malignant neoplasms detected during hysteroscopic surgery.

STUDY OBJECTIVES: To estimate the incidence of incidental miscellaneous uterine malignant neoplasms other than endometrioid adenocarcinoma detected during routine resectoscopic surgery in women with abnormal uterine bleeding (AUB) and to examine the effect of hysteroscopic surgery on long-term clinical outcome. DESIGN: Prospective cohort study (Canadian Task Force classification II-3). SETTING: University-affiliated teaching hospital. PATIENTS: Women with AUB. INTERVENTION: From January 1, 1990, through December 31, 2008, one of the authors (G.A.V.) and several fellows performed primary hysteroscopic surgery at St. Joseph's Health Care in 3892 women with AUB. Of the 7 with malignant disease, one underwent hysteroscopic partial (n = 1) or complete (n = 6) rollerball electrocoagulation or endomyometrial resection. After diagnosis of uterine cancer, the women were counseled about the disease and management in accord with established clinical practice guidelines. Follow-up with office visits and telephone interviews ranged from 2 to 8 years (median, 6 years). MEASUREMENTS AND MAIN RESULTS: Of the 3892 women, 4 had undiagnosed and 3 had suspected miscellaneous uterine malignant neoplasms including 1 endometrial stromal sarcoma, 2 carcinosarcomas, 2 atypical polypoid adenomyomas of the endometrium, 1 minimal deviation adenocarcinoma of the cervix, and 1 smooth-muscle tumor of uncertain malignant potential. At 2 to 8 years of follow-up, 1 patient died accidentally after 1 year, 1 died of carcinomatosis of either coexisting breast cancer or a carcinosarcoma (postmortem examination was declined) after 1 year, and 5 were alive and well. CONCLUSION: Resectoscopic surgery in women with miscellaneous uterine malignant lesions not adversely affect 5-year survival and long-term prognosis.

Endoscopic features of smooth muscle tumors in children with AIDS.

BACKGROUND: Smooth muscle tumors are the second most common malignancy encountered in children with acquired immunodeficiency syndrome (AIDS). This study reviewed the incidence and endoscopic appearance of smooth muscle tumors in the gastrointestinal tract of children with AIDS undergoing endoscopy. METHODS: Retrospective review of all endoscopic records (n = 70) of children with AIDS from August 1988 to December 1997 at a tertiary care inner city hospital. RESULTS: Three children with advanced AIDS (4%) were found to have smooth muscle tumors, all of which had the typical appearance of submucosal nodules with central ulceration. Some were hemorrhagic. They measured less than 1 to 4 cm in diameter. Two children had multiple tumors. All lesions except for one were located in the colon. Colonic biopsies revealed a leiomyoma in one patient and a smooth muscle tumor of uncertain malignant potential in another. The forceps biopsy of the single gastric mass was not deep enough to make the diagnosis of a smooth muscle tumor. A nonmalignant smooth muscle tumor grew from less than 5 mm to more than 4 cm in 1 year and was removed surgically. All three children are alive without any evidence of local spread or distant metastases despite no specific therapy for the smooth muscle tumor. CONCLUSIONS: Smooth muscle tumors in the GI tract of children with AIDS are relatively common. Further study of the long-term outcome for children infected with the human immunodeficiency virus who have these lesions are needed to establish prognosis and management guidelines.