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.

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

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.

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.

Long-term effect of letrozole on metastatic uterine smooth muscle tumors of uncertain malignant potential: A case report.

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.

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.

Uterine smooth muscle tumor of uncertain malignant potential: A review of current knowledge.

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.

Clinical and Histopathological Predictors of Recurrence in Uterine Smooth Muscle Tumor of Uncertain Malignant Potential (STUMP): A Multicenter Retrospective Cohort Study of Tertiary Centers.

BACKGROUND: The term uterine smooth muscle tumor of uncertain malignant potential (STUMP) indicates a rare, equivocal entity between benign leiomyomas and leiomyosarcomas. In the present study, we evaluated a comprehensive range of clinical, surgical, and pathological features in a large multicenter series of patients with STUMP to identify risk factors for recurrence. METHODS: This is a retrospective study performed by collecting consecutive cases diagnosed between January 2000 and December 2020 in five tertiary centers. Associations between STUMP recurrence and clinicopathological characteristics as well as surgical treatment modality were investigated. RESULTS: Eighty-seven patients affected by STUMP were considered. Of them, 18 cases (20.7%) recurred: 11 as leiomyosarcoma (LMS) and 7 as STUMP. The mean time to recurrence was 79 months. We found that fragmentation/morcellation, epithelioid features, high mitotic count, Ki-67 value > 20%, progesterone receptor (PR) < 83%, and p16 diffuse expression were associated with higher risk of recurrence and shorter recurrence-free survival (RFS). Furthermore, morcellation/fragmentation and mitotic count remained independent risk factors for recurrence and shorter RFS after multivariate analysis, while the presence of epithelioid features was an independent risk factor for recurrence only. CONCLUSIONS: Our results suggest that morcellation is associated with risk of recurrence and shorter RFS, thus it should be avoided if a STUMP is suspected preoperatively. Epithelioid features, high proliferation activity, low PR expression, and diffuse p16 expression are also unfavorable prognostic factors, so patients presenting these features should be closely followed up.

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.

A 39-Year-Old Woman With Synchronous Endobronchial and Adrenal Tumors.

CASE PRESENTATION: A 39-year-old woman with systemic lupus erythematosus that was complicated by end-stage renal disease that had required a deceased donor renal transplant 16 years ago was referred for evaluation of chronic, nonproductive cough for 2 years. She was a lifetime nonsmoker whose condition was maintained on prednisone 5 mg daily, tacrolimus 3 mg twice day, mycophenolate mofetil 500 mg twice a day for her immunosuppression regimen, valacyclovir 500 mg twice a day for prophylaxis, and clonidine 0.1 mg daily and metoprolol succinate 100 mg twice daily for hypertension.

Leiomyosarcoma in adrenal gland.

Not required for Clinical Vignette.

Uterine smooth muscle tumors of uncertain malignant potential (STUMP): A retrospective study in a single center.

OBJECTIVE: Uterine smooth muscle tumors of uncertain malignant potential (STUMP) is a heterogeneous group of tumors with histological and biological diversity that cannot be defined as a benign leiomyoma or malignant leiomyosarcoma. The study aims to investigate the diagnostic methods, treatment management and prognosis of STUMP patients in a 13-year period. STUDY DESIGN: We retrospectively reviewed the clinicopathologic information of 31 STUMP patients in Peking University People's Hospital. Statistical analyses were conducted to compare the difference of clinical characteristics between the women in myomectomy group and those in hysterectomy group. RESULTS: The most common clinical presentation was menstrual disorder. The tumors were mainly manifested as hypoechoic, non-cystic nodules with low blood flow signal by pelvic doppler ultrasonography. Most tumors carried Ki-67 index ranging from 10% to 30%. Immunohistochemical markers such as ER, PR, p16 and Desmin was positively expressed in tumors. At the first operation, 21 cases underwent myomectomy and 10 cases underwent hysterectomy. The patients in myomectomy group were younger than those in hysterectomy group. In the follow-up period, two cases experienced a relapse in the form of STUMP within 36 months. One case died of cardiovascular accident while the other cases were alive. Six of 21 women in myomectomy group desired pregnancy and two healthy live births were recorded. CONCLUSION: The diagnosis of STUMP primarily depends on histopathologic features. Fertility-sparing surgery may be a treatment selection for patients with fertility desire. Patients with STUMP, especially in the case of myomectomy, should be informed of recurrence risk and monitored closely.

"Epstein-Barr virus associated smooth muscle tumour as an unusual cause of ureteric graft obstruction in a child".

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.

Clinical and reproductive outcomes of uterine smooth muscle tumor of uncertain malignant potential: a single-center retrospective study.

OBJECTIVE: To evaluate the clinical outcomes, histopathological features, and obstetric and oncological outcomes of uterine smooth muscle tumor of uncertain malignant potential (STUMP). METHODS: We conducted a single-center, database review of patients with STUMP between January 2001 and December 2015. We investigated the clinical, operative, histopathologic, recurrence, and fertility outcomes of the included cases. RESULTS: Nineteen patients with STUMP were studied. Three were reclassified as sarcoma after slide review, and 16 patients were finally included in the study. The mean age was 45 years. Ki-67 expression was ≥10% in 25.0% of cases and 30% in the only recurrent case. Recurrence occurred 52 months after a diagnosis of STUMP in a 56-year-old female patient who underwent hysterectomy. Two of six patients who underwent myomectomy had fertility requirements, and both successfully delivered babies without recurrence. Recurrence was not related to mitosis, degree of atypia, or necrosis. There was also no relationship between type of surgery or surgical approach and recurrence. CONCLUSIONS: Patients with STUMP warrant a pathological review process in centers with experience. Fertility-preservation is worth attempting, but young patients must be followed-up closely. Ki-67 might be a valuable marker predicting recurrence.

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.

PHENOTYPIC CHARACTERISTICS OF RELAPSED LEIOMYOMA AND SMOOTH MUSCLE TUMORS OF UNCERTAIN MALIGNANCY POTENTIAL IN REPRODUCTIVE WOMEN.

Uterine leiomyoma represents the most common pelvic tumor in females, including numerous histological subtypes, from which smooth muscle tumors of uncertain malignancy potential (STUMP) represents the diagnostic challenge. On the other hand, the study of the relapse risk markers after laparoscopic myomectomy is of high interest. We investigated the molecular phenotype of different types of leiomyoma after hysterectomy or laparoscopic surgery in reproductive and menopausal women. Standard immunohistochemistry was used to detect proliferation markers Ki67 and cyclin D1, apoptotic markers Bcl2 and Cas3, and ER and PR. The results of our study indicated that ER expression is significantly higher in relapsed leiomyoma, compared to control group. In addition, relapsed leiomyomas are characterised with high proliferation and apoptotic index. With regard to STUMP despite histological homogeneity, this entity is characterised with the presence of three distinct molecular subtypes, based on proliferation and apoptotic marker expression, which should be used as diagnostic aid in these tumors.

Role of surgery in treating epstein-barr virus-associated smooth muscle tumor (EBV-SMT) with central nervous system invasion: A systemic review from 1997 to 2019.

Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is a rare mesenchymal tumor occurred almost exclusively in immunocompromised hosts. This article provides a systematic review of literature under PRISMA guideline on clinical features, treatment modalities, roles of surgical intervention, and outcomes of all 65 reported EBV-SMTs with central nervous system (CNS) invasion. Over 95% of reported cases were immunocompromised, while human immunodeficiency virus infection and post-organ transplantation were the most commonly associated underlying causes (near 90%). Despite a heterogeneous follow-up period, a 1-year survival rate of 76.0% and 5-year survival rate of 59.6% may support the indolent and non-deadly nature of EBV-SMT even with CNS invasion. Immune survey and reconstruction should be conducted for every patient with CNS EBV-SMT. Surgical resection is mostly adopted as primary treatment to obtain diagnosis and relieve compressive effect. A total resection of tumor may be beneficial if tumor was symptomatic and had intracranial invasion.

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.

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.