Open Gastrostomy of a Gastric Leiomyoma Proximal to the Gastroesophageal Junction: A Case Report.

Gastric leiomyomas are benign, submucosal tumors found incidentally on unrelated imaging or during autopsy. The majority of leiomyomas are asymptomatic; however, patients can develop central ulcerations on the lesions leading to upper gastrointestinal (GI) bleeding. A 75-year-old female, with a past medical history of hypertension, hyperlipidemia, and a cerebrovascular accident, presented with complaints of melena, near-syncope events, lightheadedness, weakness, and hematemesis. A computed tomography (CT) of the abdomen with contrast found a heterogeneous low-attenuation mass of 4×4×3 cm3 within the gastric fundus and near the gastroesophageal (GE) junction. After an open gastrostomy and excisional biopsy, the mass was identified as a leiomyoma. This case report reviews the presentation, diagnostic assessments, and treatment of a gastric leiomyoma in a complex location proximal to the gastroesophageal junction. Gastric leiomyomas should be considered as a differential diagnosis for patients presenting with an upper gastrointestinal bleed.

Genomic profile analysis of leiomyomas with bizarre nuclei and fumarate hydratase deficient leiomyomas: Strengths, weaknesses, and limitations of array-CGH interpretation.

A close relationship has been demonstrated between genomic complexity and clinical outcome in uterine smooth muscle tumors. We studied the genomic profiles by array-CGH of 28 fumarate hydratase deficient leiomyomas and 37 leiomyomas with bizarre nuclei (LMBN) from 64 patients. Follow-up was available for 46 patients (from three to 249 months, mean 87.3 months). All patients were alive without evidence of disease. For 51 array-CGH interpretable tumors the mean Genomic Index (GI) was 16.4 (median: 9.8; from 1 to 57.8), significantly lower than the mean GI in LMS (mean GI 51.8, p < 0.001). We described three groups: (1) a group with FH deletion (24/58) with low GI (mean GI: 11 vs. 22,4, p = 0.02), (2) a group with TP53 deletion (17/58) with higher GI (22.4 vs. 11 p = 0.02), and (3) a group without genomic events on FH or TP53 genes (17/58) (mean GI:18.3; from 1 to 57.8). Because none of these tumors recurred and none showed morphological features of LMS we concluded that GI at the cut-off of 10 was not applicable in these subtypes of LM. By integration of all those findings, a GI <10 in LMBN remains a valuable argument for benignity. Conversely, in LMBN a GI >10 or alteration in tumor suppressor genes, should not alone warrant a diagnosis of malignancy. Nine tumors were tested with Nanocind CINSARC® signature and all were classified in low risk of recurrence. We propose, based on our observations, a diagnostic approach of these challenging lesions.

Benign female genital tract smooth muscle tumors with adipocytic differentiation: A morphologic, immunohistochemical and MDM2 fluorescence in situ hybridization study of 44 conventional lipoleiomyomas and lipoleiomyoma variants.

Leiomyomas with adipocytic differentiation typically occur in the uterus although they may arise at several sites in the female genital tract. While these are most commonly spindled leiomyomas with a component of adipocytic tissue ("conventional lipoleiomyomas"), there is a relatively ill-defined assortment of leiomyoma variants with adipocytic differentiation. We performed a morphologic, immunohistochemical and MDM2 gene amplification analysis of a large series of gynecologic leiomyomas with adipocytic differentiation to better define the clinicopathologic spectrum. Forty four tumors from 44 patients were identified and classified as conventional lipoleiomyoma (n = 21), adipocyte-rich lipoleiomyoma (defined as tumor volume >80 % adipocytes, n = 9); cellular lipoleiomyoma (n = 9); hydropic lipoleiomyoma (n = 3); and lipoleiomyoma with bizarre nuclei (n = 2). Patient age ranged from 32 to 83 years (mean 63; median 63). Primary location included uterine corpus (35), uterine cervix (3), uterine corpus/cervix (1), broad ligament (2), parametrium (2), and round ligament (1). Tumor size was 0.6-30 cm (mean 8; median 6). None of the 34 patients with follow up developed further disease (range 1-311 months; mean 65; median 41). Immunohistochemical expression of ER, PR, HMB45, Melan A, Cathepsin K and WT-1 in lipoleiomyomas and variants was similar to patterns in non-adipocytic gynecologic leiomyomas. MDM2 amplification fluorescence in situ hybridization performed on 14 tumors was negative in all. Our findings suggest female genital tract conventional lipoleiomyomas and lipoleiomyoma variants largely parallel their non-adipocytic counterparts in morphology and immunophenotype, and may be categorized using non-adipocytic leiomyoma histologic criteria.

Smooth Muscle Tumor of Uncertain Malignant Potential (STUMP): A Comprehensive Multidisciplinary Update.

Background and Objectives: The uterine smooth muscle tumors of uncertain malignant potential (STUMP) are tumors with pathological characteristics similar to leiomyosarcoma, but that do not satisfy histological criteria for leiomyoma. These are problematic lesions with intermediate morphologic features; thus, diagnosis and treatment are difficult. This narrative review aims to review data in the literature about STUMPs, particularly focusing on management and therapeutic options and strategies for women who desire to preserve fertility. Material and Methods: authors searched for "uterine smooth muscle tumor of uncertain malignant potential" in PubMed and Scopus databases, from 2000 to March 2023. Pertinent articles were obtained in full-text format and screened for additional references. Only articles in English language were included. Studies including full case description of patients with histopathological diagnosis of STUMP in accordance with Stanford criteria were included. Results: The median age was 43 years old. Symptoms are similar to those of leiomyomas, with a mean diameter of 8.0 cm. Total hysterectomy with or without bilateral salpingo-oophorectomy is the standard care for women if fertility desire is satisfied. Myomectomy alone can be considered for young patients. Although these tumors have not a high malignant potential, several studies described recurrence and metastases. Conclusions: STUMPs are complex uterine smooth muscle tumors, with a rare but reasoned clinical-diagnostic management. Considering the high clinical and histological complexity of these tumors, high level of expertise is mandatory.

Top 10 Significant Spindled Head and Neck Lesions to Scrutinze.

BACKGROUND: Spindled lesions are a challenging area in head and neck pathology. This is particularly true in the sinonasal tract, where several uncommon entities with both unique and overlapping morphologic, immunophenotypic, and/or molecular features can occur. METHODS: Review. RESULTS: The clinicopathologic characteristics of biphenotypic sinonasal sarcoma and nine important differential diagnostic considerations with one or more overlapping feature are summarized to establish a practical framework for approaching spindled lesions of the sinonasal tract. CONCLUSION: Morphologic evaluation is central to the work up of sinonasal spindle cell lesions-in particular, cellular morphology, tumor architecture and growth pattern, and the presence of admixed epithelial elements - however, focused immunohistochemical analysis of neural, myogenic, rhabdomyoblastic, epithelial, and/or melanocytic marker expression and/or ancillary tests for tumor-specific molecular alterations may be necessary for definitive diagnosis.

Hemoperitoneum due to spontaneous bleeding from a smooth muscle tumor of uncertain malignant potential: A rare case report.

INTRODUCTION AND IMPORTANCE: Smooth muscle tumors of uncertain malignant potential (STUMPs) are uncommon tumors representing an extremely rare cause of hemoperitoneum. CASE PRESENTATION: We report a case of a 48-year-old Caucasian, premenopausal woman that presented in the emergency department with acute abdominal pain. There was no remarkable past medical and surgical history except from a known uterine leiomyoma. The ultrasound and the computed tomography imaging showed an intraperitoneal fluid collection and a heterogenous uterine mass. The patient underwent emergent exploratory laparotomy; a subserosal uterine tumor was identified with an actively bleeding vessel on its surface. The uterine lesion was completely excised and the histopathology set the diagnosis of a STUMP. After consultation on the significance of this finding with the patient, an abdominal total hysterectomy and bilateral salpingo-oophorectomy were scheduled and performed and the subsequent histopathology detected no malignancy. CLINICAL DISCUSSION: This case demonstrates that a STUMP may be a rare cause of acute intraperitoneal bleeding. Careful evaluation of clinical history, imaging findings and, if needed, surgical exploration are important for the diagnosis, while appropriate follow-up is also of major importance for the management of these rare tumors. CONCLUSION: We presented an extremely rare case of hemoperitoneum due to spontaneous bleeding from a STUMP. From an oncological perspective, this case poses a diagnostic, management and follow-up challenge.

Primary Retroperitoneal Smooth Muscle Tumor of Uncertain Malignant Potential (STUMP): A Diagnostic Enigma.

Retroperitoneal sarcomas represent a group of rare malignant neoplasms with complex clinical management and often a poor prognosis. An elderly male presented with a slowly progressive, right-sided abdominal lump for four months associated with loss of appetite and abdominal discomfort. Abdominal examination revealed an apparent retroperitoneal lump in the right lumbar and umbilical region, which was well-defined, and firm in consistency with the bosselated surface. Contrast-enhanced computed tomography (CECT) of the abdomen and pelvis revealed a heterogenous lobulated malignant appearing retroperitoneal lesion arising from the right anterior perirenal space with a differential of retroperitoneal sarcoma. Wide local excision of the tumor was done. Histopathology of the lesion revealed a smooth muscle tumor of uncertain malignant potential (STUMP). The patient is asymptomatic and recurrence-free after 24 months of follow-up.

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.

Antiretroviral therapy achieved metabolic complete remission of hepatic AIDS related Epstein-Barr virus-associated smooth muscle tumor.

Epstein-Barr virus-associated smooth muscle tumor (EBV-SMT) is a rare mesenchymal tumor which occurs in immunocompromised patients. The immune status is an important factor in the treatment of EBV-SMTs, but the efficacy of antiretroviral therapy (ART) is not elucidated in acquired immune deficiency syndrome (AIDS) related EBV-SMTs. Here, we report the first successful case of a 29-year-old man with hepatic AIDS related EBV-SMT treated with ART solely. Positron emission tomography scan was useful for the evaluation of disease status. Recent advances in ART that enables to restore patient's immune status rapidly may change the treatment strategy in AIDS related EBV-SMT.

Epstein-Barr virus-associated smooth muscle tumors in immunocompromised patients: Six case reports.

BACKGROUND: Epstein-Barr virus associated smooth muscle tumor (EBV-SMT) is a rare oncological entity. However, there is an increasing incidence of EBV-SMTs, as the frequency of organ transplantation and immunosuppression grows. EBV-SMT diagnosis relies on histopathology and immunochemical staining to distinguish it from post-transplant lymphoproliferative disorder (PTLD). There is no clear consensus on the treatment of EBV-SMTs. However, surgical resection, chemotherapy, radiation therapy, and immunosuppression reduction have been explored with varying degrees of success. CASE SUMMARY: Our case series includes six cases of EBV-SMTs across different age groups, with different treatment modalities, adding to the limited existing literature on this rare tumor. The median latency time between immunosuppression and disease diagnosis is four years. EBV-SMTs present with variable degrees of aggressiveness and seem to have worse clinical outcomes in patients with tumor multiplicity and worse immunocompetency. CONCLUSION: It is imperative to continue building on this knowledge and keeping EBV-SMTs on the differential in immunocompromised individuals.

[Leiomyosarcoma of the nasal fossa. A case report]. / Leiomiosarcoma de fosa nasal. A propósito de un caso.

A 54-year-old female patient presented with a left nasal obstruction. On physical examination a pink delimited mass in the left nostril was observed. A cranial computed tomography scan revealed an expansive mass in the upper anterior third of the left nasal fossa, partially obstructing it. Endoscopic resection of the mass was performed. Histopathology revealed an atypical mesenchymal proliferation formed by cells disposed in disorganized and interconnected long bundles. Tumor cells had abundant eosinophilic cytoplasm and an oval, vesicular and hyperchromatic nucleus. Frequent mitotic figures were observed, many of them atypical. Necrosis was not observed. Immunohistochemistry showed tumor cells to be positive for calponin, muscle specific actin, caldesmon and smooth muscle specific myosin. Ki-67 index proliferation was 30%. A diagnosis of leiomyosarcoma of the nasal fossa was established.

Leiomiosarcoma de fosa nasal. A propósito de un caso / Leiomyosarcoma of the nasal fossa. A case report

Paciente mujer de 54 años que acude a consulta por un cuadro de obstrucción nasal. En la exploración física se observa una lesión rosada, bien delimitada, en la fosa nasal izquierda. Se realiza TAC de macizo facial en la que se observa una masa expansiva a nivel del tercio anterosuperior de la fosa nasal izquierda. Se realiza resección endoscópica. Histológicamente se observa una proliferación mesenquimal atípica constituida por células que forman haces largos desorganizados y entrecruzados. Las células tumorales presentan un citoplasma amplio eosinófilo y núcleo ovalado, vesiculoso e hipercromático. Se aprecian frecuentes figuras mitóticas, muchas de ellas atípicas. No se observa necrosis. En el estudio inmunohistoquímico se evidenció inmunorreactividad de las células tumorales frente a calponina, actina muscular específica, caldesmón y miosina específica de músculo liso. El índice de proliferación frente a KI-67 fue de un 30%. Con todos estos hallazgos se estableció el diagnóstico de leiomiosarcoma de fosa nasal.(AU)

A 54-year-old female patient presented with a left nasal obstruction. On physical examination a pink delimited mass in the left nostril was observed. A cranial computed tomography scan revealed an expansive mass in the upper anterior third of the left nasal fossa, partially obstructing it. Endoscopic resection of the mass was performed. Histopathology revealed an atypical mesenchymal proliferation formed by cells disposed in disorganized and interconnected long bundles. Tumor cells had abundant eosinophilic cytoplasm and an oval, vesicular and hyperchromatic nucleus. Frequent mitotic figures were observed, many of them atypical. Necrosis was not observed. Immunohistochemistry showed tumor cells to be positive for calponin, muscle specific actin, caldesmon and smooth muscle specific myosin. Ki-67 index proliferation was 30%. A diagnosis of leiomyosarcoma of the nasal fossa was established.(AU)

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.

Benign and malignant proliferation in idiopathic nephrotic syndrome: a French cohort study.

BACKGROUND: There seems to be a possible link between nephrotic syndrome (NS) and lymphoproliferative syndrome, but it remains poorly understood. METHODS: This multicentric and retrospective study focuses on children, who developed idiopathic NS and malignant or benign proliferation between 2000 and 2021. RESULTS: Eleven patients were included, with a median age of 4 years. Only one had a steroid-resistant nephrotic syndrome (SRNS). The maintenance therapy before the proliferation was in majority tacrolimus or mycophenolate mofetil (MMF), but three patients did not receive treatments. The proliferation was mainly a Hodgkin's lymphoma (45%) or a lymphoproliferative disease (36%), in a median time after the NS of two years. Viruses were found in seven cases (EBV in five cases and HHV-8 in two). CONCLUSION: The association between proliferative syndrome and idiopathic NS may not be fortuitous, possibly with a common lymphocytic disturbance. Genetic analyses could improve the comprehension of these manifestations in the future. A higher resolution version of the Graphical abstract is available as Supplementary information.

A case of Epstein-Barr virus-associated smooth muscle tumor of the posterior interosseous nerve mimicking schwannoma.

Here we report a case of Epstein-Barr virus (EBV)-associated smooth muscle tumor (SMT) of the peripheral nerve in a young man seropositive for human immunodeficiency virus (HIV). Initially, the lesion was clinically and radiologically confused with a schwannoma of the forearm's posterior interosseous nerve. The diagnosis was corrected by histological examination, which revealed a well-defined tumor consisting of eosinophilic spindle cells, positive for α-smooth muscle actin on immunohistochemistry and positive for EBV-encoded early RNA (EBER) on in situ hybridization. EBV-associated SMTs are well described in the literature; they are frequently multiple and arise in many organs. They occur preferentially in young adults with poorly controlled and chronic HIV infection. The prognosis is influenced by the complications of immunodeficiency. To our knowledge, this is the first description of a peripheral nerve location. Because EBV-associated SMT should be considered in the differential diagnosis of a tumor in the peripheral or central nervous systems in immunocompromised patients, EBV should be tested in these locations. Thus, a cause of immunodeficiency should be identified when the diagnosis of EBV-associated SMT is made.

Canine smooth muscle tumors: A clinicopathological study.

Canine smooth muscle tumors (SMTs) commonly develop in the alimentary and female genital tracts and less frequently in soft tissue. The definition of histological criteria of malignancy is less detailed for SMTs in dogs than in humans. This study evaluated the clinicopathologic features of canine SMTs and compared the veterinary and human medical criteria of malignancy. A total of 105 canine SMTs were evaluated histologically and classified according to both veterinary and human criteria. The Ki67 labeling index was assessed in all SMTs. Estrogen receptor (ER) and progesterone receptor (PR) expression was evaluated for soft tissue SMTs. Follow-up data were available in 25 cases. SMTs were diagnosed in the female genital tract (42%), alimentary tract (22%), and soft tissue (20%). Soft tissue SMTs frequently arose in the perigenital area, pelvic cavity, and retroperitoneum. A subset of soft tissue SMTs expressed ER and/or PR, resembling the gynecologic type of soft tissue SMT in humans. SMTs were less frequently malignant when assessed with human criteria than with veterinary criteria, better reflecting their benign behavior, especially in the genital tract where human criteria tolerate a higher mitotic count for leiomyoma. Decreased differentiation was correlated with increased proliferation, necrosis, and reduced desmin expression. Mitotic count, Ki67 labeling index, and necrosis were correlated with metastases and tumor-related death. Further prognostic studies are warranted to confirm the better performance of the human criteria when assessing SMT malignancy, especially genital cases, to confirm their usefulness in ER/PR-expressing soft tissue SMTs, and to better define the most useful prognostic parameters for canine SMTs.

Concurrent Epstein Barr virus-associated smooth muscle tumor and myeloid sarcoma of the liver and acute myeloid leukemia in a patient post kidney transplant: a case report and review of the literature.

Background: Epstein Barr virus-associated smooth muscle tumors (EBV-SMT) are rare neoplasms that can occur in immunocompromised individuals. The native or transplanted liver is the most commonly involved site in post transplant patients. Systemic therapies have been utilized in EBV-SMT with modest activity. Case Description: We describe a 23-year-old female kidney transplant recipient who presented with acute myeloid leukemia (AML) and hepatic myeloid sarcoma (MS). Although it was not recognized initially, her liver biopsy revealing MS at diagnosis was posthumously found to have synchronous EBV-SMT. She underwent anthracycline based induction and achieved a complete remission of her AML by bone marrow biopsy. Due to a persistent hepatic mass, she was given salvage chemotherapy including fludarabine, etoposide, cytarabine, decitabine, and venetoclax for presumed refractory MS. Re-biopsy of the liver revealed the absence of MS and presence of EBV-SMT, which subsequently grew rapidly and precluded her from a liver tumor resection. The patient underwent sirolimus mammalian target of rapamycin (mTOR) therapy with palliative intent, but the patient's EBV-SMT progressed shortly after. At time of autopsy, the patient remained in complete remission from AML/MS, but was found to have multifocal progressive metastatic EBV-SMT. Conclusions: To our knowledge this is the first reported case of synchronous AML/MS and post transplant hepatic EBV-SMT that underwent treatment for AML/MS. Our report suggests that the chemotherapeutic agents utilized for AML/MS may have poor efficacy against EBV-SMT.

Adoptive Cell Transfer of Allogeneic Epstein-Barr Virus-Specific T Lymphocytes for Treatment of Refractory EBV-Associated Posttransplant Smooth Muscle Tumors: A Case Report.

Posttransplant smooth muscle tumors (PTSMTs) are rare Epstein-Barr virus (EBV)-associated neoplasms, mostly occurring after solid organ transplantation. Current therapeutic strategies include surgery and reduction of immunosuppressive medication. We describe for the first time a novel treatment approach for PTSMT by adoptive cell transfer (ACT) of EBV-specific T cells to a 20-year-old patient with a medical history of cardiac transplantation, posttransplant lymphoproliferative disease, and multilocular PTSMT. During ACT, mild cytokine release syndrome occurred, while no unexpected safety signals were recorded. We observed in vivo expansion of EBV-specific T cells and reduction of EBV viremia. Best response was stable disease after 4 months with reduction of EBV viremia and normalization of lactate dehydrogenase levels. ACT with EBV-specific T cells may be a safe and efficacious therapeutic option for PTSMT that warrants further exploration.

Metastatic Smooth Muscle Tumor of Uncertain Malignant Potential after Laparoscopic Presuming Myomectomy.

A 38-year-old para-2 female underwent laparoscopic myomectomy with uncontained morcellation. Three years later, she complained of epigastric pain. An intraperitoneal 3 cm mass beneath the umbilicus was showed on computed tomography (CT) scan. With the impression of gastrointestinal stromal tumor, she underwent open laparotomy at the general surgery department. A tumor was excised. Pathological examination showed that the tumor was consistent with a smooth muscle tumor of uncertain malignant potential smooth muscle tumors of uncertain malignant (STUMP). Six years postlaparoscopic myomectomy, during a regular follow-up, three parauterine masses were found on ultrasonography and CT scan. She underwent laparoscopic surgery for hysterectomy, bilateral salpingectomy, and excision of the masses. The masses were again diagnosed as STUMP. This case presents a recurrence of a rare type of smooth muscle tumor after uncontained morcellation. If myomas are to be removed with morcellation, it should only be used appropriately with a compatible containment system, and the risk of occult malignancy should be counseled.