A girl, aged 7 years, was admitted due to pain in both lower limbs for more than one year. Lumbar MRI showed soft tissue masses in the paravertebral region. Cerebral MRI showed nodular masses in the cavernous sinus at both sides. Chest CT showed high-density nodules in the outer basal segment of the right inferior lobe and the anterior segment of the left upper lobe of the lung. Biopsy of lumbar lesions showed Epstein-Barr (EB) virus-related smooth muscle tumor. Genetic testing showed a
Epstein-Barr Virus Infections ; Female ; Herpesvirus 4, Human/genetics* ; Humans ; Magnetic Resonance Imaging ; Smooth Muscle Tumor/diagnosis* ; Tomography, X-Ray Computed

OBJECTIVE: In this study, we aimed to evaluate the clinicopathological features, obstetric, and oncological outcomes of patients diagnosed with a uterine smooth muscle tumors of uncertain malignant potential (STUMP). METHODS: A dual-institutional, database review was carried out to screen patients with STUMP who were treated with upfront surgery between January 2006 and December 2017. Data including age at the time of diagnosis, recurrence rate, disease-free survival, overall survival, and fertility outcomes were retrospectively analyzed. RESULTS: Fifty-seven patients with STUMPs were included in the study. The median age at the time of diagnosis was 42 (range, 16 to 75) years. The median follow-up was 57 (range, 16 to 125) months. Eight patients (14%) had recurrence during follow-up. Recurrent STUMPs were seen in seven patients and leiomyosarcoma after 14 months in one patient. Seven patients with a recurrent STUMP survived, while the remaining patient died. Recurrence rates were similar for women who underwent myomectomy and those who underwent hysterectomy. The presence of uterine localization of tumor (subserosal vs intramural-submucosal) statistically significantly affected recurrence rates (odds ratio=5.72; 95% confidence interval=1.349–24.290; p=0.018). Ten of 27 patients who underwent myomectomy for uterine myoma had fertility desire. Seven pregnancies were recorded. CONCLUSIONS: Our study results suggest that fertility-sparing approaches are feasible in patients with STUMP, although recurrence may be seen.
Diagnosis ; Disease-Free Survival ; Female ; Fertility ; Follow-Up Studies ; Humans ; Hysterectomy ; Leiomyoma ; Leiomyosarcoma ; Muscle, Smooth ; Pregnancy ; Recurrence ; Retrospective Studies ; Smooth Muscle Tumor

OBJECTIVE: Intravenous leiomyomatosis (IVL) and benign metastasizing leiomyoma (BML) are uncommon variants of benign uterine leiomyomas with extrauterine manifestations. Categorizing the extent of disease allows clinicians to delineate the clinical spectrum and the level of sophistication for complete surgical resection. METHODS: Twelve patients with IVL and BML were reviewed. They were divided into early versus late stage disease groups, and initial manifestation, clinical characteristics, laboratory values, surgical pathology, and follow up data were summarized. RESULTS: Patients were mostly pre- or peri-menopausal and parous. Patients with late stage disease were more likely to present with cardiac symptoms or abnormal findings on chest X-ray, whereas those with early stage disease presented with classical leiomyoma symptoms including heavy menstrual bleeding, increased myoma size, or lower abdominal discomfort. Tumor marker levels were within normal ranges. A trend of higher neutrophil to leukocyte ratio was observed in the late versus the early stage group (10.4 vs. 1.51, P=0.07); the platelet leukocyte ratio was statistically higher in patients with late stage IVL (0.23 vs. 0.13, P=0.04). The overall recurrence rate was 25%. No recurrence was observed in stage I or stage III IVL groups, while 50% of the stage II IVL group showed recurrence in the pelvic cavity. CONCLUSION: IVL and BML are benign myoma variants with paradoxically metastatic clinical presentation. Careful inquiry of systemic symptoms, the presence of underlying systemic inflammation, and a high index of suspicion are required for preoperative diagnosis. Furthermore, a multidisciplinary approach is necessary to improve outcomes of surgical resection.
Blood Platelets ; Diagnosis ; Follow-Up Studies ; Hemorrhage ; Humans ; Inflammation ; Leiomyoma* ; Leiomyomatosis* ; Leukocytes ; Myoma ; Neoplasm Metastasis ; Neutrophils ; Pathology, Surgical ; Recurrence ; Reference Values ; Smooth Muscle Tumor ; Thorax

BACKGROUND: Primary endobronchial smooth muscle tumors (SMTs), which are extremely rare, include endobronchial leiomyomas and leiomyosarcomas. Clinically, SMTs present with signs and symptoms of bronchial obstruction, and lack specific radiological findings. Thus, histopathological examination is required for accurate diagnosis as well as for tumor grading. We examined the histomorphological and immunohistochemical features of endobronchial SMTs and highlighted pitfalls in diagnosis, particularly when using small biopsies. METHODS: Cases of primary endobronchial SMTs diagnosed at our Institute over the last 6 years (2012–2017) were retrieved from the departmental archives. Histopathological features and immunohistochemistry performed for establishing the diagnosis were reviewed. RESULTS: Five cases of SMTs occurring in endobronchial locations were identified. These included three cases of leiomyoma, and two cases of leiomyosarcoma. The age distribution of patients ranged from 13 to 65 years. Leiomyomas showed more consistent staining with smooth muscle markers (smooth muscle actin, desmin, and smooth muscle myosin heavy chain), while tumors of higher grade showed variable, focal staining, leading to erroneous diagnosis, especially on small biopsies. CONCLUSIONS: The diagnosis of endobronchial SMTs relies on histopathological examination, for both confirmation of smooth muscle lineage and determination of the malignant potential of the lesion. Appropriate immunohistochemical panels including more than one marker of smooth muscle differentiation are extremely valuable for differential diagnosis from morphological mimics, which is necessary for instituting appropriate management.
Actins ; Age Distribution ; Biopsy ; Desmin ; Diagnosis ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Leiomyoma ; Leiomyosarcoma ; Muscle, Smooth ; Myosins ; Neoplasm Grading ; Smooth Muscle Tumor

BACKGROUND: Cutaneous angioleiomyoma is a rare and benign smooth muscle tumor derived from the walls of blood vessels. No studies on this condition have been conducted in Korea since a study of 59 cases of angioleiomyoma was reported in 2000. OBJECTIVE: We sought to analyze the clinical and histopathological types of angioleiomyoma in Korea. METHODS: Data were collected from 27 cases of angioleiomyoma in the dermatology department at Hallym University Medical Center. A clinical analysis was conducted through a chart and photographic review. All cases were histologically classified into four types: solid, cavernous, venous, and mixed. RESULTS: The number of males and females was 12 and 15, respectively. The mean age at diagnosis was 41.1 years, and the mean duration of disease was 4.3 years. Lower extremity (63.0%) was the most common site in both, males and females, and the size of tumors did not exceed 2 cm. About half of the patients (51.9%) had pain or tenderness associated with the tumor. The tumors histopathologically were of the solid (16 cases), mixed (6 cases), cavernous (3 cases), and venous (2 cases) types. CONCLUSION: This study will help to improve our understanding of the characteristics of angioleiomyoma in Korea.
Academic Medical Centers ; Angiomyoma* ; Blood Vessels ; Dermatology ; Diagnosis ; Female ; Humans ; Korea ; Lower Extremity ; Male ; Smooth Muscle Tumor

OBJECTIVETo study the clinicopathologic features, immunophenotype, histological diagnosis and prognosis of hepatic epithelioid angiomyolipoma.

METHODSClinical data of 25 cases of hepatic epithelioid angiomyolipoma were collected along with follow-up study of the patients. The pathological features were documented and immunohistochemical study of various markers was performed with an emphasis on diagnosis and differential diagnosis.

RESULTSHepatic epithelioid angiomyolipoma was more commonly found in young women without characteristic clinical symptoms. Its morphological features were characterized by marked cytological atypia, relatively rare mitotic figures; radial distribution of tumor cells around the thin-walled blood vessels or muscular vessels; and the presence of common multinucleated giant cells and large ganglion-like tumor cells. The tumor cells expressed both melanoma cell markers (HMB45, MART-1) and smooth muscle cell markers (SMA). Tumor cells expressed various other markers including ER 16% (4/25), PR 32% (8/25), TFE3 24% (6/25) and p53 60% (15/25).

CONCLUSIONSHepatic epithelioid angiomyolipoma has variable morphological features and characteristic immunohistochemical phenotype. The differential diagnoses include a variety of tumors. The biological behavior of the tumor tends to be benign.

Age Factors ; Angiomyolipoma ; genetics ; immunology ; metabolism ; pathology ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Gastrointestinal Neoplasms ; Giant Cells ; pathology ; Humans ; Immunohistochemistry ; Immunophenotyping ; Liver Neoplasms ; genetics ; immunology ; metabolism ; pathology ; MART-1 Antigen ; metabolism ; Melanoma-Specific Antigens ; metabolism ; Muscle, Smooth ; metabolism ; Prognosis

OBJECTIVETo systematically study the clinical diagnosis and treatment of smooth muscle tumor in the male reproductive system.

METHODSWe analyzed the ultrasonographic features, pathological findings, treatment strategies and postoperative follow-up results of 5 male patients with smooth muscle tumor in the reproductive system, and reviewed other relevant literature.

RESULTSCompared with leiomyoma, leiomyosarcoma exhibited stronger mixed echoes than the testis at ultrasonography, typical mitotic phase (> or = 2/10 HP) of tumor cells at HE staining, and significant expressions of HIF-1alpha and Glut-1 at immunohistochemistry. No relapse was observed in the 2 cases of leiomyoma during the 10-year follow-up after simple tumor resection, nor were recurrence and metastasis in another 3 cases of leiomyosarcoma during the first year after radical surgery without combined radio- and chemo-therapy.

CONCLUSIONPrimary smooth muscle tumor of the male reproductive system is difficult to be diagnosed. Ultrasonography can help to preliminarily screen leiomyosarcoma. For those with possible leiomyosarcoma, preoperative MRI and intraoperative frozen sectioning examinations are recommended for the possibility of lymphatic metastasis. Postoperative radiotherapy and chemotherapy should be chosen cautiously for those confirmed with leiomyosarcoma by pathological examination.

Adult ; Genital Neoplasms, Male ; diagnosis ; therapy ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Smooth Muscle Tumor ; diagnosis ; therapy

Uterine smooth muscle tumor is very rare in laboratory rats and, there has been no report in the wild rodents. Among a total of 400 wild rats captured in Gyeonggi, Gangwon, and Chungbuk provinces of Korea in 2007, 2010, and 2011, we found a uterine spindle cell tumor, diagnosed as smooth muscle cell origin based on differential features of histology and immunohistochemistry. Its incidence was very low, like in the laboratory rats, as under 0.5% for female. Considering generally applied histological and cellular criteria, this case was difficult in differential diagnosis between benign and malignant. Ki-67 labeling index was therefore further investigated, and it ranged from 26.4 to 37.6% in the 10 different areas, representing an average of 32.9+/-0.05%. The Ki-67 labeling index of neoplastic cells near the necrotic area was recorded as 83.5%. According to such high Ki-67 labeling index, it was more likely a malignant leiomyosarcoma, assenting to the previous proposal that Ki-67 labeling index is a significant criterion to differentiate between malignant and benign in the smooth muscle tumors.
Animals ; Diagnosis, Differential ; Female ; Humans ; Immunohistochemistry ; Incidence ; Korea ; Leiomyosarcoma ; Myocytes, Smooth Muscle ; Rats ; Rodentia ; Smooth Muscle Tumor

Actins ; metabolism ; Adult ; Breast Neoplasms ; metabolism ; pathology ; surgery ; Calcium-Binding Proteins ; metabolism ; Cell Differentiation ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Fibroadenoma ; metabolism ; pathology ; surgery ; Hamartoma ; pathology ; Humans ; Hyperplasia ; Leiomyoma ; pathology ; Microfilament Proteins ; metabolism ; Muscle, Smooth ; pathology ; Phyllodes Tumor ; pathology

Actins ; metabolism ; Adenoma ; metabolism ; pathology ; Aged, 80 and over ; Desmin ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Leiomyoma ; metabolism ; pathology ; Smooth Muscle Tumor ; metabolism ; pathology ; surgery ; Thyroglobulin ; metabolism ; Thyroid Neoplasms ; metabolism ; pathology ; surgery ; Thyroidectomy ; Vimentin ; metabolism