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Background: This study aimed to examine clinicopathologic characteristics, treatment patterns, and survival rates in a contemporary population-based cohort of adult prostate sarcoma patients. Methods: In the Surveillance, Epidemiology, and End Results database (2004–2020), adult patients with prostate sarcoma were identified. Descriptive statistics, Kaplan–Meier analyses, smoothed cumulative incidence plots, and Cox regression models were used. Results: Of 125 patients, 45 (36%) harbored leiomyosarcoma, 17 (14%) had rhabdomyosarcoma, 15 (12%) had stromal sarcoma, 17 (14%) had sarcoma not otherwise specified (NOS), and 31 (25%) had other sarcoma subtypes. Metastatic stage was most common in the rhabdomyosarcoma patients (44%) and least common in the leiomyosarcoma (21%) and stromal sarcoma (20%) patients. Most of the rhabdomyosarcoma patients received the combination of systemic and radiation therapy with (24%) or without radical surgery (35%), whereas most of the leiomyosarcoma and stromal sarcoma patients underwent radical surgery with (22 and 13%) or without (22 and 47%) radiation. In the overall population, the median overall survival was 27 months. The 5-years overall versus cancer-specific versus other-cause mortality rates were respectively 71 versus 58 versus 13%. In the multivariable Cox regression models, the highest overall mortality was exhibited by the patients with metastatic disease (hazard ratio [HR] 2.87; 95% confidence interval [CI] 1.55–5.31; p < 0.001) or unknown disease stage (HR 2.94; 95% CI 2.20–7.21; p = 0.019). Conversely, of all the histologic subtypes, only stromal sarcoma distinguished itself by lower overall mortality (HR 0.41; 95% CI 0.18–0.96; p = 0.039). Conclusions: Four major histologic subtypes were identified. Among most adult sarcoma patients, treatment patterns vary according to histology, from multimodal therapy to radical prostatectomy alone. These treatment differences reflect equally important heterogeneity in survival patterns. [ABSTRACT FROM AUTHOR]

PRRX1‐fused mesenchymal neoplasm is a recently identified, rare subcutaneous soft tissue neoplasm that is characterized by fusion of PRRX1 (exon 1) with NCOA1 (exon 13) in the majority of reported cases. Although initially considered to be fibroblastic, a possibility of neural or neuroectodermal differentiation has been suggested in a subset of cases. We report a 26‐year‐old female with a 4.0 cm painless mass located in the subcutis of the left thigh. Microscopically, the tumor was well‐circumscribed and multinodular and was composed of relatively monomorphic ovoid to spindle cells arranged in loose fascicles, trabeculae, and cords within alternating myxoid and fibrous matrix, and vascularized stroma. Mitotic figures were scarce and necrosis was not observed. By immunohistochemistry, the neoplastic cells demonstrated focal co‐expression of S100 protein and SOX10 and were negative for epithelial membrane antigen, smooth muscle actin, desmin, CD34, STAT6, HMB45, Melan‐A, and MUC4. The expression of Rb1 was retained. Targeted RNA‐sequencing identified a novel transcript fusion of PRRX1 (exon 1)::NCOA1 (exon 15), which was further confirmed by reverse transcription polymerase chain reaction and Sanger sequencing. The tumor was narrowly excised and no tumor recurrence or metastasis was identified after 13 months of follow‐up. In summary, we report a new case of PRRX1‐fused mesenchymal neoplasm, expanding the molecular genetic spectrum and providing further support for possible neural or neuroectodermal differentiation of this emerging soft tissue tumor entity. [ABSTRACT FROM AUTHOR]

Cellular angiofibroma (CA) is a relatively recently described, rare, benign soft tissue tumor that predominantly occurs in the vulvoperineal region. It was first described in 1997 as a mesenchymal-origin tumor composed of connective (fibrous) tissue and blood vessels. We report a case of vaginal CA in a 40-year-old woman who presented with pressure symptoms, incidentally diagnosed with a vaginal mass and clinically suspected as vaginal myoma. This case was diagnosed based on histomorphology and immunohistochemistry and underwent surgical treatment. This tumor was a diagnostic dilemma for other benign and aggressive spindle cell tumors in the same area, such as endometrial stromal nodule, spindle cell lipoma, solitary fibrous tumors, and mixed tumors of the vagina.

Abstract Background Cellular angiofibroma is a rare benign mesenchymal tumor that mostly occurs in the genital area. Its occurrence outside this region, particularly in the pelvis, is extremely rare. To our knowledge, this study reports the first case of cellular angiofibroma occurring in the pelvic cavity, except for one case reported in the retroperitoneum. Case presentation A 25-year-old female patient with chronic, intermittent, dull pain in the lower abdomen that lasted for several months was referred to our clinic. Imaging studies revealed a tumor in the pelvic cavity anterior to the bladder. The radiographic characteristics of this tumor indicated a hypervascular nature, suggesting the possibility of a pheochromocytoma or a neuroendocrine tumor. The patient underwent surgical excision of the lesion. To date, no recurrence has been observed four months after excision. Conclusions Cellular angiofibroma, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Immunohistochemical staining can help confirm the diagnosis of this condition. Treatment is generally straightforward, involving local excision of the tumor followed by postoperative monitoring.

Ahlam M Al-Kharabsheh,1 Bushra Khalaf AlTarawneh,2 Seham M Abufraijeh,1 Omar Abu Azzam1 1Department of Obstetrics and Gynecology, Faculty of Medicine, Mutah University, Alkarak, Jordan; 2Department of Pathology, Faculty of Medicine, Mutah University, Alkarak, JordanCorrespondence: Ahlam M Al-Kharabsheh, Obstetrics and Gynecology department, Faculty of Medicine, Mutah University, Alkarak, Jordan, Tel +962-798009216, Email alkharabsheh@mutah.edu.joAbstract: Aggressive angiomyxoma is a locally invasive, rarely diagnosed mesenchymal tumor that predominantly affects the perineal region of women in their reproductive years. It is typically found in areas such as the vagina, vulva, and other pelvic soft tissues, but its presentation can be highly variable. We describe a particularly unique case of aggressive angiomyxoma, presenting in an atypical manner within the vaginal region of a 31-year-old virgin woman. This case is noteworthy for the unusual growth pattern of the angiomyxoma—located strictly within the vaginal epithelium, showing no signs of invasion into surrounding tissues, which is not typical of the aggressive nature of this type of tumor. The patient presented with a mass that protruded through the hymen, an uncommon presentation that led to initial speculation about the nature of the mass. The definitive diagnosis of aggressive angiomyxoma was made by meticulous histopathological examination after surgical excision of the mass. This case emphasizes the critical importance of considering aggressive angiomyxoma in the differential diagnosis of vaginal masses, particularly those that do not exhibit invasive characteristics. The case also highlights the need for increased awareness among clinicians about the potential for atypical presentations of this rare tumor, to facilitate timely and accurate diagnosis, and to guide appropriate management strategies. This report contributes to the growing body of literature on aggressive angiomyxoma, highlighting the variability in its presentation and the need to heighten suspicion in atypical cases.Keywords: aggressive angiomyxoma, vaginal cyst, vaginal tumor, mesenchymal tumor, case report

Simple Summary: Soft tissue sarcomas (STSs) are rare cancers, making up less than 1% of all adult malignancies. Traditionally, the primary treatment for STS has been surgical resection. However, non-surgical approaches are becoming increasingly important in specific clinical situations. In this review, we explore the role of non-surgical treatments in managing STS, including their use as a bridge to surgery, as alternatives to surgery, for improving surgical outcomes, and for managing cases where surgery is not an option. Our findings highlight the effectiveness of these strategies in enhancing patient care and outcomes, providing a more personalized and less invasive approach to STS treatment. This review aims to advance the understanding and application of non-surgical methods in the treatment of soft tissue sarcomas. Mesenchymal tumors originate from mesenchymal cells and can be either benign or malignant, such as bone, soft tissue, and visceral sarcomas. Surgery is a cornerstone treatment in the management of mesenchymal tumors, often requiring complex procedures performed in high-volume referral centers. However, the COVID-19 pandemic has highlighted this need for alternative non-surgical approaches due to limited access to surgical resources. This review explores the role of non-surgical treatments in different clinical scenarios: for improving surgical outcomes, as a bridge to surgery, as better alternatives to surgery, and for non-curative treatment when surgery is not feasible. We discuss the effectiveness of active surveillance, cryoablation, high-intensity focused ultrasound, and other ablative techniques in managing these tumors. Additionally, we examine the use of tyrosine kinase inhibitors in gastrointestinal stromal tumors and hypofractionated radiotherapy in soft tissue sarcomas. The Sarculator tool is highlighted for its role in stratifying high-risk sarcoma patients and personalizing treatment plans. While surgery remains the mainstay of treatment, integrating advanced non-surgical strategies can enhance therapeutic possibilities and patient care, especially in specific clinical settings with limitations. A multidisciplinary approach in referral centers is vital to determine the optimal treatment course for each patient. [ABSTRACT FROM AUTHOR]

Background: Soft tissue tumors with fusions or amplifications of the GLI1 gene have distinctive molecular characteristics and have recently been considered a unique pathological entity, thus named " GLI1 -altered soft tissue tumors." It is a rare mesenchymal neoplasm that involves soft tissues at any site. Case presentation: We report an example of this condition in a 13-year-old Chinese male patient who presented with a mass in the tongue. The tumor was multilobulated; the tumor cells were arranged in nests and sheets, had a rich, delicate fibrovascular network, and were separated by a hyalinized fibrous stroma. The tumor cells were epithelioid to ovoid, with variable eosinophilic to pale vacuolated cytoplasm and round to oval nuclei. Immunostaining revealed that the tumor cells were positive for CDK4 and CD56. Fluorescence in situ hybridization (FISH) for GLI1 translocation was positive, with a high level of amplification of the translocated segment. Literature review: We present a comprehensive literature review of this condition, focusing on its clinical presentation, histological features, immunohistochemical profile, molecular characteristics, and prognosis. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumor (SFT) is a type of fibroblastic neoplasm that can occur in various parts of the body, with SFT of the pineal gland being exceedingly rare. We report the case of a 58-year-old male presenting with recurrent hiccups, acid reflux, and headache. Magnetic resonance imaging revealed an occupying lesion in the pineal region, suggestive of a neoplastic process. Intraoperatively, the lesion was located in the pineal region, exhibiting a grayish-red color, and was largely resected. Pathological examination confirmed the diagnosis of solitary fibrous tumor (CNS WHO Grade 1). Postoperatively, the patient was supplemented with radiotherapy, and long-term follow-up showed no signs of recurrence or metastasis. [ABSTRACT FROM AUTHOR]

Background: Cellular angiofibroma is a rare benign mesenchymal tumor that mostly occurs in the genital area. Its occurrence outside this region, particularly in the pelvis, is extremely rare. To our knowledge, this study reports the first case of cellular angiofibroma occurring in the pelvic cavity, except for one case reported in the retroperitoneum. Case presentation: A 25-year-old female patient with chronic, intermittent, dull pain in the lower abdomen that lasted for several months was referred to our clinic. Imaging studies revealed a tumor in the pelvic cavity anterior to the bladder. The radiographic characteristics of this tumor indicated a hypervascular nature, suggesting the possibility of a pheochromocytoma or a neuroendocrine tumor. The patient underwent surgical excision of the lesion. To date, no recurrence has been observed four months after excision. Conclusions: Cellular angiofibroma, although rare in the pelvic cavity, should be considered in the differential diagnosis of hypervascular pelvic space-occupying lesions. Immunohistochemical staining can help confirm the diagnosis of this condition. Treatment is generally straightforward, involving local excision of the tumor followed by postoperative monitoring. [ABSTRACT FROM AUTHOR]

Apart from the lethal midline carcinoma (NUT carcinoma), NUTM1 translocation has also been reported in mesenchymal tumors, but is exceedingly rare. Here, we describe a series of 8 NUTM1-rearranged sarcomas to further characterize the clinicopathologic features of this emerging entity. This cohort included 2 males and 6 females with age ranging from 24 to 64 years (mean: 51 y; median: 56 y). Tumors occurred in the colon (2), abdomen (2), jejunum (1), esophagus (1), lung (1) and infraorbital region (1). At diagnosis, 6 patients presented with metastatic disease. Tumor size ranged from 1 to 10.5 cm (mean: 6 cm; median: 5.5 cm). Histologically, 4 tumors were composed of primitive small round cells to epithelioid cells intermixed with variable spindle cells, while 3 tumors consisted exclusively of small round cells to epithelioid cells and 1 tumor consisted predominantly of highgrade spindle cells. The neoplastic cells were arranged in solid sheets, nests, or intersecting fascicles. Mitotic activity ranged from 1 to 15/10 HPF (median: 5/10 HPF). Other features included rhabdoid phenotype (4/8), pronounced nuclear convolutions (2/8), prominent stromal hyalinization (2/8), focally myxoid stroma (1/8), foci of osteoclasts (1/8), and necrosis (1/8). By immunohistochemistry, all tumors showed diffuse and strong nuclear staining of NUT protein, with variable expression of pancytokeratin (AE1/AE3) (2/8), CK18 (1/8), CD99 (3/8), NKX2.2 (2/8), cyclin D1 (2/8), desmin (2/8), BCOR (2/8), S100 (1/8), TLE1 (1/8), and synaptophysin (1/8). Seven of 8 tumors demonstrated NUTM1 rearrangement by fluorescence in situ hybridization analysis. RNA-sequencing analysis identified MXD4::NUTM1 (3/7), MXI1::NUTM1 (3/7), and MGA::NUTM1 (1/7) fusions, respectively. DNA-based methylation profiling performed in 2 cases revealed distinct methylation cluster differing from those of NUT carcinoma and undifferentiated small round cell and spindle cell sarcomas. At follow-up (range: 4 to 24 mo), 1 patient experienced recurrence at 8.5 months, 4 patients were alive with metastatic disease (5, 10, 11, and 24 mo after diagnosis), 3 patients remained well with no signs of recurrence or metastasis (4, 6, and 12 mo after diagnosis). Our study further demonstrated that NUTM1-rearranged sarcoma had a broad range of clinicopathologic spectrum. NUT immunohistochemistry should be included in the diagnostic approach of monotonous undifferentiated small round, epithelioid to high-grade spindle cell malignancies that difficult to classify by conventional means. DNA-based methylation profiling might provide a promising tool in the epigenetic classification of undifferentiated sarcomas. [ABSTRACT FROM AUTHOR]

Copyright of China Journal of Oral & Maxillofacial Surgery is the property of Shanghai Jiao Tong University, College of Stomatology and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: Mesenchymal uterine tumors are a diverse group of neoplasms with varying biological potential. Many of these neoplasms can have overlapping morphologic similarities, which, in some instances, render their diagnosis and categorization thorough histomorphologic examination inconclusive. In the last decade, an exponential amount of molecular data aiming to more accurately characterize and, consequently, treat these tumors have accumulated. Objective: The goal of this narrative review is to provide a pathologic review, a genetic update, and to know the new therapeutic avenues of primary uterine mesenchymal neoplasms. [ABSTRACT FROM AUTHOR]

Background: Leiomyosarcoma (LMS) originating from the adrenal gland is exceedingly rare, constituting a minute fraction of soft tissue sarcomas. Due to its rarity, with less than 50 documented cases in English medical literature, the diagnosis and management of adrenal LMS remain challenging. The aim of this study was to perform a review of the literature, in order to evaluate the prognosis of these rare cancers and report our specific case. Methods: A systematic review of the literature was conducted using PubMed, Web of Science, Google Scholar, and Scopus databases, up to December 2020. The search utilized MeSH terms such as "Adrenal Gland Neoplasms," "Leiomyosarcoma," "Adrenalectomy," and "Smooth Muscle Tumor." The inclusion criteria focused on studies reporting patients with a histopathological diagnosis of adrenal leiomyosarcoma. The PRISMA guidelines were followed to ensure a comprehensive analysis. Results: Out of 63 identified studies, 43 met the inclusion criteria and were reviewed. These studies highlighted the rarity and aggressive behavior of adrenal leiomyosarcoma. Surgical excision remains the cornerstone of treatment, often complemented by adjuvant therapies. The reviewed case involved a 52-year-old woman who underwent a right laparoscopic adrenalectomy for a 9 × 7 × 6 cm grade 3 leiomyosarcoma. Despite subsequent adjuvant chemotherapy, hepatic metastases were detected, illustrating the aggressive nature of the disease. The literature underscores the importance of histopathological analysis and long-term surveillance for managing disease progression. Conclusions: Optimal management of adrenal leiomyosarcoma requires a multidisciplinary approach and meticulous follow-up. The rarity of the disease poses challenges for standardizing treatment, but surgical excision and tailored adjuvant therapies show promise. Further research is essential to refine treatment strategies and improve prognosis for this rare malignancy. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumors of the pleura are extremely rare mesenchymal tumors that originate from the visceral or parietal pleura, accounting for less than 5% of all pleural tumors. Solitary fibrous tumors are diagnosed based on clinical, radiological and needle biopsy findings. They are often asymptomatic and can reach very large sizes in the thorax, upon which they may produce such symptoms as cough, chest pain, dyspnea and hemoptysis. These tumors are generally benign, but are identified as malignant in 10-20% of cases, and carry the risk of recurrence, making early diagnosis very important. The treatment option is total resection followed by close follow-up. We present here a very rare case with a solitary fibrous tumor of the pleura. [ABSTRACT FROM AUTHOR]

Gastric schwannomas (GS) are rare mesenchymal tumors from Schwann cells in the gastrointestinal (GI) tract, representing 2–6% of such tumors. We report a 52-year-old woman who experienced abdominal pain, hematemesis, and melena, initially suspected of having a GI stromal tumor through ultrasound and computed tomography abdomen. Despite no active bleeding found during an upper endoscopy, she underwent a successful open subtotal gastrectomy, with histopathology confirming GS. The diagnosis of GS, which may mimic other GI conditions, relies heavily on imaging and histopathological analysis due to its nonspecific symptomatology, including the potential for both upper and lower GI bleeding. This case underscores the diagnostic challenges of GS and highlights surgical resection as the preferred treatment, generally leading to a favorable prognosis. [ABSTRACT FROM AUTHOR]

PEComa is a rare mesenchymal tumor with unique features, sometimes manifesting in younger patients and can exhibit malignant transformation. We present a 24-year-old woman with urinary symptoms and hematuria. Imagining revealed a protruding mass in the bladder dome, raising suspicion for adenocarcinoma due to its location and vascular appearance. Pathology revealed PEComa. Clinicians should inquire about macroscopic hematuria and assess the entire urinary tract even in young patients with apparent urinary tract infection. Practitioners should be mindful of PEComa tumors, especially in cases involving young patients with tumors concerning the bladder dome. A variety of immunohistochemical techniques facilitate the diagnosis.

Cellular angiofibroma is a rare benign mesenchymal tumor, typically occurring in the vulvar region of middle-aged women. This report highlights the importance of histological analysis in diagnosing this uncommon condition and emphasizes its benign nature and straightforward management. We present a case of a 58-year-old North African woman who had a large, well-defined mass in the left labia majora, which had been evolving over 2 years. MRI confirmed the resectability of the tumor by delineating its boundaries. The tumor, despite its slow growth leading to delayed diagnosis, was effectively treated with wide surgical excision. Diagnosis was confirmed through histological and immunohistochemical evaluations, revealing spindle cell proliferation with thick-walled vessels. Cellular angiofibroma, although initially alarming due to its size, is generally managed successfully with surgery and prognosis is favorable with a low risk of recurrence. [ABSTRACT FROM AUTHOR]

Objective: Aggressive Angiomyxoma (AA) of the vulva is a slow-growing mesenchymal tumour with a tendency to local invasion and recurrence. Case report: We report two cases of vulvoperineal masses that were diagnosed to be Aggressive Angiomyxomas after surgical excision. Both patients presented to the Gynaecology OPD of All India Institute of Medical Sciences, Bathinda, Punjab, India, in 2020 and 2022 with complaints of a mass coming out of introitus of three years duration and 14 years duration, respectively. The first patient was managed by surgical excision of the mass via abdominoperineal approach, while the second patient underwent vaginal hysterectomy along with the removal of the mass. Both patients were given GnRH analogues after the surgery to avoid any further recurrences and have been in remission on follow-ups so far. Conclusion: Due to its rare occurrence, clinicians should consider the possibility of AA while encountering patients with vulvovaginal masses to avoid misdiagnosis and delayed management. [ABSTRACT FROM AUTHOR]

Congenital pulmonary airway malformations are rare developmental lung anomalies, five types of which have been classified by Stocker. The condition is generally diagnosed prenatally during routine prenatal ultrasonography, however, some cases are asymptomatic and so may not be diagnosed until later in life. Clinically, the condition can present in newborns with shortness of breath, cyanosis and respiratory distress, and may lead to recurrent infections in later ages, and malignant transformations have been defined in literature. Here, we present a case that may be of interest to literature not only due to the diagnosis later in life, but also due to the development of a mesenchymal tumor in the background. [ABSTRACT FROM AUTHOR]

Copyright of Colo-Proctology is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Background: We present the case of a patient with solitary fibrous tumor of the masticator space with unusual extension. Case presentation: A 43-year-old woman presented with a painless mass with intraoral extension on the right cheek. The B-scan sonograph and magnetic resonance imaging revealed the extension of the tumor. The biopsy performed under local anesthesia raised the suspicion of a solitary fibrous tumor. Tumor excision included a preoperative tumor embolization. The surgical removal of the tumor included a partial parotidectomy on the right side, insertion of masseteric and temporalis muscle, resection of the middle part of the zygomatic bone and stabilization of the bone with a plate, mobilization of the tumor from the maxillary sinus and the pterygopalatine fossa through an endoscopic approach and an approach via partial resection of the anterior wall of the maxillary sinus after identifying and sparing the infraorbital nerve. The histological findings confirmed the diagnosis of solitary fibrous tumor. The patient's treatment completed with radiation therapy, and 2.5 years later, there was recurrence in the right temporal area. Conclusions: To our knowledge, this is the second reported case of solitary fibrous tumor arising in the masticator space and the only case with extension intraorally and in the paranasal sinuses. Tumor embolization and complete surgical excision are the most frequently recommended treatments. [ABSTRACT FROM AUTHOR]

Objective: Aggressive Angiomyxoma (AA) of the vulva is a slow-growing mesenchymal tumour with a tendency to local invasion and recurrence. Case report: We report two cases of vulvoperineal masses that were diagnosed to be Aggressive Angiomyxomas after surgical excision. Both patients presented to the Gynaecology OPD of All India Institute of Medical Sciences, Bathinda, Punjab, India, in 2020 and 2022 with complaints of a mass coming out of introitus of three years duration and 14 years duration, respectively. The first patient was managed by surgical excision of the mass via abdominoperineal approach, while the second patient underwent vaginal hysterectomy along with the removal of the mass. Both patients were given GnRH analogues after the surgery to avoid any further recurrences and have been in remission on follow-ups so far. Conclusion: Due to its rare occurrence, clinicians should consider the possibility of AA while encountering patients with vulvovaginal masses to avoid misdiagnosis and delayed management.

Abstract Plexiform fibromyxoma is a rare mesenchymal tumor identified in recent years and presents as a gastrointestinal submucosal tumor that is typically located in the gastric antrum. We report a case of gastric plexiform fibromyxoma in which the diagnosis was difficult despite repeated tissue sampling. Before visiting our hospital, the patient had been followed up for 3 years without a definitive diagnosis despite serial examinations, including computed tomography, endoscopy, endoscopic ultrasound, and endoscopic ultrasound‐guided fine‐needle aspiration. Endoscopic ultrasound‐guided fine‐needle aspiration was reperformed, and endoscopic submucosal dissection for deep biopsy was conducted for differential diagnosis of the tumor. However, histological analysis with immunostaining of tumor samples obtained using these techniques cannot provide a reliable diagnosis. Finally, the tumor was resected surgically because of its increasing size, and subsequent microscopic analysis revealed a multinodular plexiform growth pattern of spindle‐like cells with myxoid stroma. Immunohistochemically, the tumor cells were positive for smooth muscle actin but negative for c‐kit, CD34, and S100. Based on these findings, the patient was diagnosed with plexiform fibromyxoma. No evidence of residual or recurrent tumors was observed at 24 months postoperatively.

Aggressive angiomyxoma is a mesenchymal tumor that is frequently found in women of reproductive age mostly appearing in the vulva, perineal region, head, neck, trunk, and lower limbs. A 33-year-old multiparous Somali woman presented to the outpatient department of Dr Sumait Hospital, with a complaint of swelling around her vulva. The patient reported that the mass had progressively grown for the past 8 years and that she experienced stigma from her previous spouses. Her past medical history and socioeconomic status were unremarkable. During the physical examination, a well-defined pedunculated mass measuring 14 cm × 8 cm, originating from the labia majora, was noted. The mass was pinkish, non-tender, non-reducible, and had a soft consistency. Some areas of ulceration measuring 2 cm × 2 cm, covered with necrotic granulation tissues, were also noticed. With the patient’s written informed consent, a biopsy was performed, and the histopathology results revealed aggressive angiomyxoma. Following confirmation of the diagnosis, the mass was surgically excised under sterile conditions and spinal anesthesia. Aggressive angiomyxoma is an important consideration when evaluating vulvovaginal growth in females. Given its tendency for local recurrences, it is vital to establish an accurate diagnosis.

Background: Mesenchymal uterine tumors are a diverse group of neoplasms with varying biological potential. Many of these neoplasms can have overlapping morphologic similarities, which, in some instances, render their diagnosis and categorization thorough histomorphologic examination inconclusive. In the last decade, an exponential amount of molecular data aiming to more accurately characterize and, consequently, treat these tumors have accumulated. Objective: The goal of this narrative review is to provide a pathologic review, a genetic update, and to know the new therapeutic avenues of primary uterine mesenchymal neoplasms.

The benign tumor uterine leiomyoma (UL) develops from the smooth muscle tissue that constitutes the uterus, whereas malignant tumor uterine sarcoma develops from either the smooth muscle tissue or stroma and is different from UL and endometrial cancer. Uterine sarcoma is broadly classified into three types: uterine leiomyosarcoma, endometrial stromal sarcoma (ESS), and carcinosarcoma. Although uterine leiomyosarcoma and ESS are both classified as uterine sarcoma, they significantly differ in terms of their sites of occurrence, symptoms, and treatment methods. Uterine leiomyosarcoma develops from the muscle tissue constituting the wall of the uterus and accounts for approximately 70% of all uterine sarcoma cases. In contrast, ESS develops from the stromal tissue beneath the endometrium and accounts for approximately 25% of all uterine sarcoma cases. ESS is classified as either low grade (LG) or high grade (HG). This case report aimed to highlight the importance of histopathologic examinations based on surgical specimens. Herein, we reported the case of a 45-year-old woman suspected of having submucosal leiomyoma of the uterus based on imaging results. Transvaginal ultrasonography and endometrial biopsy or partial dilation and curettage were performed. Contrast-enhanced magnetic resonance imaging (MRI) revealed a 32-mm mass projecting from the posterior wall of the uterus into the uterine cavity. T2-weighted imaging revealed a low signal within the mass; thus, submucosal UL was suspected. Histopathologic examination of surgical specimens obtained from a patient suspected of having submucosal UL after contrast-enhanced MRI indicated that the patient had ESS. Despite the remarkable advancements in medical imaging technology, the accuracy of contrast-enhanced MRI for detecting uterine mesenchymal tumors is limited. Therefore, histopathologic diagnosis based on surgical specimens should be performed when medical grounds for diagnosing a benign tumor on contrast-enhanced MRI are lacking.

Aggressive angiomyxoma is a mesenchymal tumor that is frequently found in women of reproductive age mostly appearing in the vulva, perineal region, head, neck, trunk, and lower limbs. A 33-year-old multiparous Somali woman presented to the outpatient department of Dr Sumait Hospital, with a complaint of swelling around her vulva. The patient reported that the mass had progressively grown for the past 8 years and that she experienced stigma from her previous spouses. Her past medical history and socioeconomic status were unremarkable. During the physical examination, a welldefined pedunculated mass measuring 14 cm × 8 cm, originating from the labia majora, was noted. The mass was pinkish, non-tender, non-reducible, and had a soft consistency. Some areas of ulceration measuring 2 cm × 2 cm, covered with necrotic granulation tissues, were also noticed. With the patient's written informed consent, a biopsy was performed, and the histopathology results revealed aggressive angiomyxoma. Following confirmation of the diagnosis, the mass was surgically excised under sterile conditions and spinal anesthesia. Aggressive angiomyxoma is an important consideration when evaluating vulvovaginal growth in females. Given its tendency for local recurrences, it is vital to establish an accurate diagnosis. [ABSTRACT FROM AUTHOR]

Superficial myofibroblastoma is a rare benign mesenchymal tumor that presents a challenge in accurate preoperative diagnosis because of its overlapping radiological and histological features. A 27-year-old woman presented with a history of increasing abdominal girth over the prior year and pelvic mass for 1 month. Imaging confirmed the presence of a giant well-circumscribed cystic-solid tumor involving both the extraperitoneal pelvis and vagina. After exploration and excision, superficial vaginal myofibroblastoma was diagnosed pathologically. The patient underwent surgical excision and had no postoperative complications at the 1-month follow-up. Imaging features and clinical reasoning can aid in differentiating superficial myofibroblastoma from more aggressive entities or malignant tumors and guide suitable and appropriate surgical approaches.

Since the publication of the 2020 World Health Organization classification of soft tissue and bone tumors, the classification of "fibroblastic" tumors has expanded to include a novel subset of tumors characterized by PRRX1::NCOA1/2 gene fusions. These tumors defy conventional classification and are morphologically distinct, characterized by a multi-nodular growth of bland spindle cells suspended in a myxo-collagenous stroma with mild cytologic atypia, "staghorn-like" vessels, and variable perivascular hyalinization. Mitotic activity is rare, and necrosis is not identified. Herein, we present six additional cases of PRRX1-rearranged mesenchymal tumors, including five cases with PRRX1::NCOA1 fusion and one case with PRRX1::KMT2D fusion. Three cases (3/6, 50%) demonstrated focal co-expression of S100 protein and SOX10, thereby expanding the immunohistochemical profile of this emerging entity. Like prior reported cases, there was no evidence of malignant behavior on short-term follow-up. The novel fusion, PRRX1::KMT2D, further expands the molecular spectrum of this entity and leads to a proposed revision of the provisional nomenclature to "PRRX1-rearranged mesenchymal tumor" to both accommodate non-NCOA1/2 fusion partners and allow for the possibility of partial neural or neuroectodermal differentiation. [ABSTRACT FROM AUTHOR]

Gastrointestinal stromal tumor (GIST) are the most common mesenchymal tumor of the gastrointestinal tract and most common malignancy of the small intestine. Although the exact prevalence of GIST is not known, GIST was recognized as tumor it's arise from interstitial cells of Cajal. Most of the GISTs driven by a KIT or platelet-derived growth factor receptor alpha (PDGFRA) mutation. Histologically, GISTs look like spindle cell tumors most of the time, but they can be epitheoid or mixed type. Clinically, patients with small GISTs remain asymptomatic. In another case, patients with larger GISTs present with various symptoms depending on the location of the GISTs. Most of GISTs are located in the stomach or small bowel. But, they can involve almost any segment of the gastrointestinal tract from distal esophagus to anus. Diagnosis is suspected on imaging and endoscopic studies, and confirmed by tissue acquisition with immunohistochemical staining. The various endoscopic modalities of resection are increasingly tried. Surgical resection is the treatment of choice. In management of larger GISTs, tyrosine kinase inhibitors are extremly useful. Treatment options for metastatic GISTs also include radiotherapy, chemotherapy, hepatic artery embolization, chemoembolization, and radiofrequency ablation. [ABSTRACT FROM AUTHOR]

Primary adrenal leiomyosarcoma is a very rare mesenchymal tumor that arises from smooth muscle cells in the wall of the central adrenal vein or its branches (1). Less than 50 cases have been published in the English literature (2). The tumors are aggressive and often metastasize. This report describes a case of primary adrenal leiomyosarcoma that presented as intermittent left flank pain of 6 months duration in an otherwise healthy 58-year-old Caucasian female. The patient was initially imaged with an abdominal ultrasound, which revealed a left suprarenal mass. A follow-up CT of the abdomen and pelvis confirmed a malignant appearing left adrenal mass. A subsequent PET-CT demonstrated increased metabolic activity within the adrenal mass without evidence of metastasis. Biopsy proven metastasis eventually was detected on surveillance CT studies over the course of 2.5 years. Since this is such a rare malignancy, documenting its imaging findings with multiple modalities is of importance to add to the medical literature and help further characterize its imaging features.

Abstract Background Gestational trophoblastic neoplasia (GTN) is rare, and it is even rarer for GTN to merge with primary malignant tumors in other organs. Herein is described a rare clinical case of GTN combined with primary lung cancer and mesenchymal tumor of the sigmoid colon, followed with literature review. Case presentation The patient was hospitalized due to diagnosis of GTN with primary lung cancer. Firstly, two cycles of chemotherapy including 5-fluorouracil (5-FU) and actinomycin-D(Act-D) was given. Laparoscopic total hysterectomy and right salpingo-oophorectomy was performed during the third chemotherapy. During the operation, a 3*2 cm nodule was removed which was protruded from the serous surface of the sigmoid colon, and the nodule was confirmed mesenchymal tumor pathologically, in accord with gastrointestinal stromal tumor. During the treatment of GTN, Icotinib tablets were taken orally to control the progression of lung cancer. After 2 cycles of consolidation chemotherapy of GTN, she received thoracoscopic lower lobe of right lung lobectomy and the mediastinum lymph nodes removal. She undertook gastroscopy and colonoscopy and the tubular adenoma of the descending colon was removed. At present, the regular follow-up is taken and she remains free of tumors. Conclusions GTN combined with primary malignant tumors in other organs are extremely rare in clinical practice. When imaging examination reveals a mass in other organs, clinicians should be aware of the possibility of a second primary tumor. It will increase the difficulty of GTN staging and treatment. We emphasis the importance of the collaboration of multidisciplinary teams. Clinicians should choose a reasonable treatment plan according to the priorities of different tumors.

Myofibroblastoma is a rare benign breast stromal tumor comprised of fibroblasts and myofibroblasts, most commonly presenting in postmenopausal women and older men. This report discusses the case of a 69-year-old male diagnosed with myofibroblastoma after mammography, targeted ultrasound, and core needle biopsy of a retroareolar mass. The features of myofibroblastoma are presented.

Background: Leiomyoma of the pancreas is an extremely rare entity. There are currently only three reported cases, all of which were small, asymptomatic, and incidentally found tumor. Methods: We have reported the first case of leiomyoma of the pancreas in a young woman with a large symptomatic mass. Results: A 31-year-old woman presented with chronic abdominal pain. Computed tomography scans showed a huge heterogeneously enhancing mass, located between duodenum and pancreatic head. The patient underwent pancreaticoduodenectomy and the histology confirmed leiomyoma of the pancreas. Conclusions: This case adds the knowledge that this extremely rare entity could be manifested as symptomatic mass in a young patient. [ABSTRACT FROM AUTHOR]

Osteonecrosis of femoral head (ONFH) is clinically common and easily diagnosed via imaging examination, especially when there is a definite cause, such as a fracture, long-term hormonotherapy, etc. However, some rare neoplastic lesions of the femoral head can mimic its image performance in some situations, leading to misdiagnosis. We present the case of a 57-year-old male with bone pain in the left hip joint that persisted for 2 years. CT and MRI images were performed and both were suggestive of ONFH. Unexpectedly, the histopathologic results of left proximal femur resection revealed the diagnosis of phosphaturic mesenchymal tumor (PMT), a rare mesenchymal tumor. His hip pain was obviously relieved after surgery, and the course of 1-year follow-up was uneventful. [ABSTRACT FROM AUTHOR]

Solitary fibrous tumors (SFTs) originating from the external auditory canal are uncommon; only few cases have been reported in the literature. In this article, we report a case of a 35-year-old man who presented with a 6-month history of a gradual swelling in the entrance of the left external auditory meatus associated with hearing loss. The tumor was surgically removed, and histological examination showed spindle-cell proliferation with a collagenous stroma. Immunohistochemically, the tumor cells were positive for CD34 confirming the diagnosis of an SFT. Although SFTs are benign, complications such as relapses and metastasis after excision were reported. Thus, a careful and long follow-up is recommended. [ABSTRACT FROM AUTHOR]

Angiomyxoma is an infrequent tumor that originates from the mesenchyme and is primarily found in the female pelvic and perineal regions. Its diagnosis before surgery can be challenging due to its vague clinical manifestations and non-specific radiological features. One common symptom of this condition is painless swelling occurring around the pelvis and perineum. We report here a case study of a 36-year-old woman who complained of a large abdominal mass initially suspected to be retroperitoneal. The magnetic resonance imaging (MRI) results pointed towards a serous cystadenoma, while further examination during surgery suggested an initial possible diagnosis of degenerative fibroid. However, subsequent histopathological analysis confirmed that it was actually an angiomyxoma. It is crucial to consider aggressive angiomyxoma (AA) as one of the potential diagnoses for painless swellings in the genitofemoral region, particularly among women who are fertile and capable of reproduction. This particular case exemplifies the significance of including uncommon tumors like uterine angiomyxoma when making a differential diagnosis for pelvic masses, regular follow-up and monitoring for patients with a history of uterine masses, and the significance of histopathological examination to confirm the diagnosis of rare tumors. [ABSTRACT FROM AUTHOR]

Objective: Osteosarcoma (OS) is an aggressive, highly metastatic, relatively drug-resistant bone tumor with poor long-term survival rates. The presence and persistence of circulating tumor cells (CTCs) in the peripheral blood are believed to be associated with treatment inefficiency and distant metastases. A blood-based CTC test is thus greatly needed for monitoring disease progression and predicting clinical outcomes. However, traditional methods cannot detect CTCs from tumors of mesenchymal origin such as OS, and research on CTC detection in mesenchymal tumors has been hindered for years. Methods: In this study, we developed a CTC test based on hexokinase 2, a metabolic function-associated marker, for the detection and surveillance of OS CTCs, and subsequently explored its clinical value. Twelve patients with OS were enrolled as the training cohort for serial CTC tests. Dynamic CTC counting, in combination with therapy evaluation and post-treatment follow-up, was used to establish a model for predicting post-chemotherapy evaluation and disease-free survival, and the model was further validated with a cohort of 8 patients with OS. Results: Two dynamic CTC number patterns were identified, and the resulting predictive model exhibited 92% consistency with the clinical outcomes. This model suggested that a single CTC test has similar predictive power to serial CTC analysis. In the validation cohort, the single CTC test exhibited 100% and 87.5% consistency with therapy response and disease-free survival, respectively. Conclusions: Our non-invasive test for detection and surveillance of CTCs enables accurate prediction of therapy efficiency and prognosis, and may be clinically valuable for avoiding inefficient therapy and prolonging survival.

Abstract Background Leiomyosarcoma of visceral organs is uncommon, and pancreatic primary occurrence is even rarer. In terms of curative treatment, patients are generally managed with surgery alone, without significant data on the role or efficacy of adjuvant chemotherapy. Case presentation This manuscript presents a case of a 22‐year‐old female with advanced primary leiomyosarcoma of the pancreas, treated with radical surgery and adjuvant radiation therapy. Conclusion With a low‐survival rate, consideration of radiation therapy in some advanced and unresectable cases could be potentially beneficial.

Calcifying fibrous tumors (CFTs) are benign mesenchymal lesions primary to many anatomic sites, but are commonly identified in the abdomen, particularly the luminal gastrointestinal (GI) tract, of young or middle-aged adults. The clinical presentation of CFTs is largely non-specific and relegated to the site of origin and, thus, they are often incidentally identified via radiology. CFTs are managed with minimally invasive surgical management and are associated with favorable long-term outcomes. Relative to other GI organs, CFTs arising from the esophagus are seldom reported and only described heretofore in occasional case reports. We report a symptomatic CFT of the upper thoracic esophagus with discussion of clinicopathologic features including radiology, histology and immunohistochemistry and formulation of a differential diagnosis pertinent to mesenchymal tumors of the esophagus that may mimic CFTs. A brief review of other rare reports of esophageal CFTs is also provided.

Plexiform fibromyxoma (PF) is a rare mesenchymal tumor of which the pathogenesis and molecular changes are still unclear. Histologically, it is characterized by a cluster of bland spindle or ovoid cells growing in the mucoid or fibromyxoid stroma rich in small blood vessels. At present, surgical resection is the primary treatment for PF. [ABSTRACT FROM AUTHOR]

Background: Gestational trophoblastic neoplasia (GTN) is rare, and it is even rarer for GTN to merge with primary malignant tumors in other organs. Herein is described a rare clinical case of GTN combined with primary lung cancer and mesenchymal tumor of the sigmoid colon, followed with literature review. Case presentation: The patient was hospitalized due to diagnosis of GTN with primary lung cancer. Firstly, two cycles of chemotherapy including 5-fluorouracil (5-FU) and actinomycin-D(Act-D) was given. Laparoscopic total hysterectomy and right salpingo-oophorectomy was performed during the third chemotherapy. During the operation, a 3*2 cm nodule was removed which was protruded from the serous surface of the sigmoid colon, and the nodule was confirmed mesenchymal tumor pathologically, in accord with gastrointestinal stromal tumor. During the treatment of GTN, Icotinib tablets were taken orally to control the progression of lung cancer. After 2 cycles of consolidation chemotherapy of GTN, she received thoracoscopic lower lobe of right lung lobectomy and the mediastinum lymph nodes removal. She undertook gastroscopy and colonoscopy and the tubular adenoma of the descending colon was removed. At present, the regular follow-up is taken and she remains free of tumors. Conclusions: GTN combined with primary malignant tumors in other organs are extremely rare in clinical practice. When imaging examination reveals a mass in other organs, clinicians should be aware of the possibility of a second primary tumor. It will increase the difficulty of GTN staging and treatment. We emphasis the importance of the collaboration of multidisciplinary teams. Clinicians should choose a reasonable treatment plan according to the priorities of different tumors. [ABSTRACT FROM AUTHOR]

Primary pulmonary myxoid sarcoma is a rare lung sarcoma, mostly involving the central lung and harboring the EWSR1::CREB1 fusion. We report an exceptional case of primary pulmonary myxoid sarcoma arising in the peripheral lung and harboring an EWSR1::ATF1 gene fusion. A 67-year-old man presented with a solid nodule in the right lower lobe, and wedge resection was performed. Microscopically, the tumor consisted of reticular proliferation of uniform mildly atypical spindle cells within abundant myxoid stroma. Immunohistochemically, smooth muscle actin was positive but desmin was negative. Fluorescence in situ hybridization confirmed EWSR1 and ATF1 gene rearrangements. No recurrence was seen for 12 months. Pathological findings and gene rearrangements are important for the diagnosis of primary pulmonary myxoid sarcoma. Complete resection and careful observation are required. [ABSTRACT FROM AUTHOR]

Perivascular epithelioid cell tumors, also known as PEComas, are rare mesenchymal tumors composed mainly of epithelioid cells found in perivascular tissue. PEComas occur most frequently in the kidney, uterus, the gastrointestinal tract, liver, and retroperitoneum; those originating in the biliary tree are extremely rare. We report a case of benign PEComa of the cystic duct with positive TFE3 staining on immunohistochemistry. A 66-year-old woman was referred for a 20 mm mass adjacent to the common bile duct discovered incidentally on abdominal ultrasound. Laboratory data including tumor markers were unremarkable. The tumor appeared to arise from the cystic duct, showed early enhancement, and compressed the common bile duct on imaging studies. Endoscopic ultrasound-guided fine-needle aspiration revealed round- and spindle-shaped atypical cells with eosinophilic cytoplasm and brown deposits suggestive of melanin granules. Histological examination of the resected specimen revealed a tumor consisting of epithelioid cells forming an alveolar structure, with melanin pigmentation. Immunohistochemistry was positive for HMB-45 and TFE3, consistent with benign pigmented PEComa of the cystic duct. Melanotic, myogenic, and TFE3 staining are helpful when diagnosing PEComas arising in unusual locations. [ABSTRACT FROM AUTHOR]