Tumor nasal de músculo liso de comportamiento maligno incierto: informe de un caso / Nasal smooth muscle tumor of uncertain malignant potential: A case report

Los tumores de músculo liso que no pueden ser clasificados según su histología como leiomiomas o leiomiosarcomas se denominan tumores de músculo liso de comportamiento maligno incierto. La localización nasal de estos tumores es muy infrecuente y la extensión adecuada de la cirugía para tratar estas neoplasias no está bien definida. Se describe el caso clínico de una adolescente de 16 años, que consultó por padecer un tumor de aspecto vascular en la cavidad nasal derecha y que fue tratada con éxito mediante cirugía intranasal. El diagnóstico histológico fue tumor de músculo liso de comportamiento maligno incierto. Por la rareza de estas neoplasias, su infrecuente localización nasal y la falta de evidencia que soporte cuál debe ser la extensión de la cirugía, es relevante la descripción y discusión del caso clínico.

Smooth muscle tumors that cannot be histologically classified as leiomyomas or leiomyosarcomas are defined as smooth muscle tumors of uncertain malignant potential. The location of these tumors in the nose is very rare, and the appropriate surgical extent to manage these neoplasms has not been adequately defined. Here we describe the case of a 16-year-old female adolescent who consulted due to a vascular-like tumor in the right nasal cavity who was successfully treated with intranasal surgery. The histological diagnosis was smooth muscle tumor of uncertain malignant potential. Given that these neoplasms are rare, the uncommon location in the nose, and the lack of evidence indicating the extent of surgery, it is relevant to describe and discuss this clinical case.

Lipoleiomioma uterino

El lipoleiomioma es una neoplasia uterina benigna poco frecuente cuya incidencia varía entre 0,03% y 0,2%. Este tumor es considerado una variante benigna de los leiomiomas uterinos típicos. Está formado por una proporción variable de adipocitos maduros y células musculares lisas. La etiología puede estar relacionada con la deficiencia de estrógenos que se produce después de la transición menopáusica; generalmente aparece en mujeres obesas perimenopáusicas o menopáusicas. La sintomatología es inespecífica y la mayoría es diagnosticada de forma incidental. Se presenta un caso de lipoleiomioma uterino en paciente de 45 años quien consultó por presentar dolor abdominal. La ecografía mostró tumor en pared anterior de un útero homogéneo y bien definido. Durante la laparotomía se encontró tumor amarillento y de textura blanda. Se realizó histerectomía total más ooforosalpingectomía. El diagnóstico anatomopatológico fue de lipoleiomioma uterino.

Lipoleiomyoma is a rare benign uterine neoplasm whose incidence varies between 0.03%-0.2%. This tumor is considered a benign variant of typical uterine leiomyomas. It consists of a variable proportion of mature adipocytes and smooth muscle cells. The etiology may be related to estrogen deficiency occurring after the menopausal transition; it usually appears in obese perimenopausal or menopausal women. The symptomatology is nonspecific, and most are diagnosed incidentally. We present a case of uterine lipoleiomyoma in a 45-year-old patient who consulted for abdominal pain. Ultrasonography showed a tumor in the anterior wall of a homogeneous and well-defined uterus. During laparotomy, a yellowish tumor with a soft texture was found. Total hysterectomy plus oophorosalpingectomy was performed. The anatomopathologic diagnosis was uterine lipoleiomyoma.

Case report of Epstein-Barr virus-associated smooth muscle tumor in children with arthritis as the first manifestation / 中华实用儿科临床杂志

Clinical data of a case of Epstein-Barr virus-associated smooth muscle tumor with non-human immunodeficiency virus/transplantation admitted in the Department of Nephrology and Rheumatology, Hunan Children′s Hospital in May 2020 were retrospectively analyzed.The 6 years and 7 months old girl presented with bilateral hip pain for more than 1 year.Imaging examination revealed multiple nodular lesions in the central nervous system and lungs.Arthritis was the first manifestation in this case, and as a result, the patient was misdiagnosed as juvenile idiopathic arthritis.Finally, the patient was pathologically diagnosed as Epstein-Barr virus-associated smooth muscle tumor, which was a rare tumor associated with immunosuppression.

Multicenter retrospective study of 38 cases with fumarate hydratase deficiency uterine leiomyoma / 中华妇产科杂志

Objective:To investigate the clinicopathological features of fumarate hydratase (FH) deficiency uterine leiomyoma.Methods:The data of 38 patients with FH deficiency uterine leiomyoma were screened and analyzed. The expressions of FH, S-(2-succino)-cysteine (2SC), desmin, p16, p53, CD 10 and cell proliferation associated nuclear antigen (Ki-67) proteins were detected by immunohistochemistry, and their clinicopathological features were analyzed retrospectively. Results:(1) Clinical features: the median age of the patients was (42.5±7.4) years old. Twenty-one cases (55%) of them were myomas found in physical examination, and the median maximum diameter of the tumor was 6.0 cm (range: 5.0-7.5 cm); myomectomy was performed in 23 cases (61%), total hysterectomy with or without bilateral appendages in 15 cases (39%); laparoscopic surgery in 27 cases (71%), open surgery in 11 cases (29%); none of the patients had renal cell carcinoma. (2) Histological features: atypical nuclear cells were distributed locally or diffusely, eosinophilic nucleoli and intranuclear inclusion bodies could be seen, glass like globules could be seen in the cytoplasm, nuclear division was 0-4/10 high power field (HPF), and antler like blood vessels and pulmonary edema-like changes could be seen in the stroma. Among 38 patients with FH deficiency uterine leiomyoma, FH was negative in 37 cases (97%), and positive in 1 case (3%); 2SC, desmin, p16, p53, CD 10 and Ki-67 showed focal positive expression in 38 cases (100%), including 35 cases (92%) with Ki-67 index<10% and 3 cases (8%) with Ki-67 index ≥10%. (3) Follow-up: 4 cases (11%) recurred, and there was no death. There were significant differences in age, family history, distribution of atypical nuclei and mitosis number between recurrent group and non-recurrent group (all P<0.05). Conclusions:FH deficiency uterine leiomyoma is a rare tumor, which needs pathological examination,immunohistochemical examination and clinical history. Patients younger than 43 years old, with family history, histologically atypical diffuse nuclear distribution and mitotic number ≥3/10 HPF should be alert to the risk of recurrence.

Cutaneous smooth muscle tumors associated with Epstein-Barr virus in an adult patient with HIV

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.

Uterine smooth muscle tumor of uncertain malignant potential: a case diagnosis, management and follow-up in the department of gynecology-obstetrics II of the Hassan II teaching hospital of Fes in Morocco

Rarely diagnosed in authors department, uterine smooth muscle tumor of uncertain malignant potential (STUMP) is one of the histologic types of uterine sarcoma. Among women undergoing hysterectomy or myomectomy for a presumed diagnosis of leiomyoma, 0.01% receive a diagnosis of STUMP. Authors report a case occurring to a patient aged of 55 which was diagnosed, managed successfully and followed-up in authors department.

Leiomiomatosis peritoneal diseminada en paciente histerectomizada / Disseminated Peritoneal Leiomyomatosis in a Hysterectomized Patient

La leiomiomatosis peritoneal diseminada es una entidad rara de etiología incierta, caracterizada por la proliferación de múltiples nódulos benignos en la cavidad peritoneal, formados por células musculares lisas, que pueden simular una carcinomatosis peritoneal. Es más frecuente en mujeres en edad fértil, durante el embarazo o por el uso de anticonceptivos orales. Se presenta el caso de una mujer de 40 años diagnosticada de leiomiomatosis peritoneal diseminada, sometida a una histerectomía simple por miomatosis uterina. Después de dos años del diagnóstico presenta una exacerbación de la enfermedad y se sospecha de malignización, por lo que se realiza una laparotomía exploratoria donde se observan múltiples nódulos miomatosos extendidos por epiplón, peritoneo y apéndice. Se le practica la exéresis de todos los nódulos (omentectomía, apendicectomía y anexectomía bilateral(. La biopsia confirma el diagnóstico de leiomiomatosis peritoneal diseminada. Actualmente, la paciente permanece asintomática tras 6 años de seguimiento(AU)

Disseminated peritoneal leiomyomatosis is a rare entity of uncertain etiology, characterized by the proliferation of multiple benign nodules in the peritoneal cavity. These nodules are formed by smooth muscle cells, which can simulate peritoneal carcinomatosis. It manifests predominantly in women of childbearing age, especially during pregnancy or with the use of oral contraceptives. We report here the case of a 40-year-old woman diagnosed with disseminated peritoneal leiomyomatosis, who underwent simple hysterectomy due to uterine myomatosis. Two years after the diagnosis, she presented with an exacerbation of the disease and malignancy was suspected. So exploratory laparotomy was performed. Multiple myomatous nodules were observed, extended by omentum, peritoneum and appendix. All nodules were removed -omentectomy, appendectomy and bilateral adnexectomy. Biopsy confirms the diagnosis of disseminated peritoneal leiomyomatosis. As of today, the patient remains asymptomatic after 6 years of follow-up(AU)

Primary Leiomyoma of the Chest Wall in a Pediatric Patient / 임상소아혈액종양

Primary leiomyoma of the chest wall is extremely rare. A 13-month-old boy presented to outpatient clinic of the department of pediatrics with a soft, movable, and palpable mass in the right supraclavicular area. Neck computed tomography revealed a 2.5-cm-sized soft tissue mass in the right anterior supraclavicular area. The mass was completely resected, and histopathological examination showed a localized primary leiomyoma with cystic change. The patient was followed up and has been disease-free for more than 11 months since surgery. To the best of our knowledge, 13 cases of leiomyoma of the chest wall have been reported to date, but this is the second case of primary leiomyoma of the chest wall in a pediatric patient. This report describes the clinical course of this case and presents a review of relevant literature.

Endobronchial Smooth Muscle Tumors: A Series of Five Cases Highlighting Pitfalls in Diagnosis

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.

Aberrant uterine leiomyomas with extrauterine manifestation: intravenous leiomyomatosis and benign metastasizing leiomyomas

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.

The evaluation of the caveolin‑1 and AT‑rich interactive domain 1 alpha expressions in uterine smooth muscle tumors.

Objectives: This retrospective study was designed to evaluate the importance of tissue expressions of caveolin‑1 (Cav‑1) and AT‑rich interactive domain 1 alpha (ARID‑1A) which are known as signal regulator and tumor suppressor in differential diagnosis of uterine smooth muscle tumors (SMTs). Materials and Methods: Thirty patients recently diagnosed as uterine SMTs at the Tepecik Training and Research Hospital were identified using pathology databases. Immunohistochemical stains for Cav‑1 and ARID‑1A were performed. Results: In this series, there were 10 leiomyosarcomas (LMSs), 10 uterine smooth muscle tumors of uncertain malignant potentials (STUMPs), and 10 leiomyomas (LMs). Cav‑1 expression located cytoplasmic or perivascular area. Cytoplasmic Cav‑1 expression was determined in 5 LMSs and 2 STUMPs while perivascular Cav‑1 expression was determined in 9 LMSs and 2 STUMPs. Statistically, it was determined that if the tumor becomes malignant and more invasive, it gains the perivascular Cav‑1 expression (P = 0.029). On the other hand, the mean nuclear staining rate for ARID‑1A in LMSs (63 ± 23.4%) was higher than both STUMPs (60 ± 18.5%) and LMs (34.5 ± 16.5%). Statistically, it was determined that the expression of ARID‑1A was significantly downregulated in LMs when compared with STUMPs and LMSs (P = 0.004). Conclusions: Our findings were demonstrated that perivascular Cav‑1 expression was seen to be a marker for malignancy of uterine SMTs. Similarly, we found to link of ARID‑1A expression and the aggressiveness of SMTs. Therefore, it may be suggested that Cav‑1 and ARID‑1A may act as predictive biomarkers in uterine SMTs.

Vaginal leiomyosarcoma in a cow from Uberlândia, Minas Gerais, Brazil / Leiomiossarcoma Vaginal em uma vaca de Uberlândia, Minas Gerais, Brasil

The clinical, pathological, and immunohistochemical findings associated with a vaginal leiomyosarcoma in a 12-year-old, Senepol breed of cow from Uberlândia, Minas Gerais are described. The animal had a large ulcerative neoplastic growth that originated between the base and the left-lateral vaginal wall. Histopathology revealed a tumor formed by muscle-like cells that demonstrated cellular pleomorphism, anisokaryosis, prominent and multiple nucleoli, with rare tumor giant cells. The neoplastic growth invaded adjacent adipose tissue, and contained areas of hemorrhage with discrete accumulations of inflammatory cells. By immunohistochemistry, most neoplastic cells expressed actin, while immunoreactivity to desmin was weakly expressed. These findings support a diagnosis of leiomyosarcoma, and represent the first description of a bovine vaginal leiomyosarcoma from Brazil.

No presente trabalho, foram descritos os achados clínicos, patológicos e imunohistoquímicos de uma vaca da raça Senepol, 12 anos, com leiomiossarcoma vaginal, de Uberlândia, Minas Gerais. O animal apresentou massa ulcerada localizada entre a base e parede lateral-esquerda da vagina. A histopatologia revelou uma neoplasia formada por células fusiformes a arredondadas que demonstraram pleomorfismo celular, anisocariose, nucléolos proeminentes e múltiplos, com raras células gigantes tumorais. O crescimento neoplásico invadiu o tecido adiposo adjacente e continha áreas de hemorragia com discreto acúmulo de células inflamatórias. A maioria das células neoplásicas expressou actina na avaliação imunohistoquímica, enquanto a imunoreatividade para desmina foi fracamente expressa. Os achados suportam o diagnóstico de leiomiossarcoma e representa a primeira descrição de leiomiossarcoma vaginal em bovinos no Brasil.

Leiomioma vascular bucal: reporte de un caso / Vascular leiomioma: case report

Leiomiomas son neoplasias benignas de músculo liso, poço frecuentes en cavidad bucal, que presentan crecimiento lento y se asemejan a otras lesiones de tejido conectivo. Clinicamente de tamaño entre 0,5 y 3 cm de diámetro, los leiomiomas vasculares generalmente tienen base pedunculada y coloración diversa debido a su ubicación y vascularización. El propósito de este artículo es presentar un reporte de caso de leiomioma vascular en lábio inferior y discutir su etiología, diagnóstico diferencial y tratamiento

Leiomyomas are benign smooth muscle neoplasms, rarely seen in oral cavity, that presents slow growth and are similar to others lesions of conjuntive tissue. Clinically with a size between 0,5 and 3 cm in diameter, vascular leiomyomas generally have pedunculated basis and diverse coloration due to location and vascularity. The purpose of this article is to present a case report of oral vascular leiomyoma in the lower lip and discuss their etiology, differential diagnosis and treatment

Case for diagnosis / Caso para diagnóstico

Vascular leiomyoma are uncommon benign smooth muscle tumors which generally present as a single painful nodule in the lower limbs. We report a case of vascular leiomyoma on the second finger of the left hand, an unusual location for this tumor.

Angioleiomiomas são tumores benignos raros derivados da musculatura lisa vascular, que geralmente se apresentam como nódulo doloroso solitário nos membros inferiores. Relata-se um caso de angioleiomioma no segundo quirodátilo esquerdo, localização incomum deste tumor.

Late presentation of metastatic smooth muscle neoplasm of the uterus with low malignant potential / 부인종양

A 48-year-old woman underwent total abdominal hysterectomy with conservation of the ovaries and tubes. Histology showed a well-circumscribed smooth muscle tumor with foci of degeneration (including infarct-type necrosis) but no coagulative tumor cell necrosis and only mild focal cytological atypia. She presented, 24 years later with shortness of breath and abdominal distension and underwent bilateral salpingo-oophorectomy, appendectomy, omental biopsy and para-aortic lymph node sampling. Histology showed bilateral ovarian smooth muscle tumors with no coagulative tumor cell necrosis or significant cellular atypia. The cells were mitotically active. The tumors in both ovaries were most likely secondary to the previous uterine smooth muscle neoplasm. To our knowledge, this case is the first in the literature to describe a benign cellular leiomyoma that subsequently behaved as a smooth muscle tumor of uncertain malignant potential, which recurred 24 years after the initial diagnosis.

Uterine smooth muscle tumors of uncertain malignant potential and atypical leiomyoma: A morphological study of these grey zones with clinical correlation.

Objectives: To study the histological features in uterine STUMP, and atypical leiomyomas (AL), and to correlate with clinical outcome. Materials and Methods: From January 2004 to August 2010, a total of 21 cases were retrieved from records, labeled as STUMP(7), AL (5), AL with low risk of recurrence (2), smooth muscle tumor of low malignant potential (STLMP) (2), and symplastic leiomyoma (5). The slides were reviewed for coagulative tumor cell necrosis (CTCN), hyaline necrosis/ infarction type necrosis, presence and degree of cytological atypia, mitotic activity, epithelioid morphology and myxoid features. The other characteristics (such as size, circumscription, individual tumor cell necrosis), were noted, wherever available. Results: The mean age was 45 years (median 46; range 24-67 yrs). CTCN was seen in 2 cases on examination of additional material; wherein a revised diagnosis of leiomyosarcoma had been given. Infarction type necrosis and individual cell necrosis was seen in 2 and 3 cases, respectively. Mitoses were less than 5/10hpf in all the cases. One of the tumours labeled as STUMP also had concurrent endometrial adenocarcinoma. Follow up: Follow-up was available in 11 cases (52.3%). One patient had died. (cause not known). In 10 patients, the follow-up ranged from 4 to 56 months (mean 20.9 months; median 15 months) nine patients were alive and well. One patient (labeled STLMP) had metastatic liver disease 3 yrs after the primary surgery, at the last follow-up. Conclusions: 1) There is an overlap in using the terminologies as STUMP, AL, AL with low risk of recurrence, AL with low malignant potential. A designation of STUMP does convey a category of borderline malignancy to the gynecological surgeons. Most behave in a benign fashion and follow-up without adjuvant therapy is currently recommended. Critical evaluation of coagulative tumor necrosis is essential. Follow-up remains a challenge in our setting.

A Case of Cutaneous Smooth Muscle Tumor of Uncertain Malignant Potential / 대한피부과학회지

Smooth muscle tumors have been classified into leiomyoma and leiomyosarcoma. But the criteria for malignancy are not well defined. Traditionally, mitoses are the most important differentiating feature between leiomyoma and leiomyosarcoma. However mitotic activity was regarded as an acceptable finding in about 20% of cutaneous leiomyoma. The widely used designation "smooth muscle tumor of uncertain malignant potential" is a reflection of the limitation of available criteria to precisely diagnose tumors with borderline atypical features. We present herein a case of a cutaneous smooth muscle tumor of uncertain malignant potential, which is mitotically active, on the abdomen of a 45-year-old man.

Piloleiomioma simulando morfea en un niño / Piloleiomyoma mimicking morphea in a child

Los piloleiomiomas son tumores cutáneos raros que tienen origen en el músculo erector del pelo. Presentamos el caso de un niño de tres años de edad con una placa indurada en el glúteo izquierdo, asintomática, que estuvo presente desde los tres meses de edad. El examen anatomopatológico mostró un piloleiomioma. Este tumor benigno es raro en niños y suele ser confundido con otras dermatosis. En este artículo revisamos los casos pediátricos publicados y los aspectos genéticos actuales de esta enfermedad. Además, llamamos la atención para el diagnóstico diferencial con otras patologías en la edad pediátrica (AU)

Piloleiomyomas are rare benign smooth muscle tumors arising from the aerector pili muscles in the skin.We describe a three year-old boy presenting with an asymptomatic hard plaque on the left gluteus, which was present since 3 months of age.The anatomopathological examination evidenced a piloleiomyoma.There are few previous reports of piloleiomyomas in children and so they are frequently confounded with other disorders. In this paper, we review the literature on the pediatric cases reported and the current genetics aspects of this disease. Moreover, we make the differential diagnosis with other entities in children (AU)

A case of retroperitoneal huge smooth muscle tumor misleading to ovarian cancer / 부인종양

Smooth muscle tumors are very common tumors in the uterus and related adjacent structures but occur rarely in the retroperitoneum. Traditionally, most retroperitoneal smooth muscle tumor are believed to be malignant. But well-differentiated smooth muscle tumors with lack of atypia, necrosis, and significant mitotic activity appear to have a benign behaviors. Laparotomy revealed a huge solid tumor in the retroperitoneal space, about 50 cm in diameter, and histologically diagnosed as a smooth muscle tumor of uncertain malignant potential (STUMP). We report a case of primary retroperitoneal smooth muscle tumor with a brief review of literatures.

A Case of Pulmonary Leiomyoma in a Renal Transplanted Patient / 대한신장학회잡지

The overall incidence of malignancy in a renal transplanted patient is 3 to 5 times higher compared with general population. We report a very rare case of multiple leiomyoma originated from lung after renal transplantation. 33-year-old male underwent renal transplantation in November, 1989. A 5 mm sized pulmonary nodule was found in the left lower lobe incidentally in March, 2001. The size of pulmonary nodule increased and same lesion was found on the opposite side of the lung on chest roentgenogram after one year, so we proceeded with computed tomography of the chest. Variable sized multiple nodules in the whole lung field were noted, which were thought be metastatic lesions, and for detection of the primary site of malignancy, further study including esophagogastroduodenoscope, colon study, abdominal ultrasound, abdominal CT, bone scan and tumor marker were checked, but there was no evidence of primary cancer. After open lung biopsy low malignant potential smooth muscle tumor was noted, and Epstein-barr virus (EBV) DNA was detected. The patient had been maintained on immunosuppressive therapy with cyclosporine and mycophenolate mofetil (MMF), and after the diagnosis of leiomyoma administration of MMF was stoped, and cyclosporine dosage was reduced. With the reduction of immunosuppressants, intravenous immunoglobulin trial was done for the first cycle, but the efficacy of treatment is not clear.