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Introduction and Importance: Scrotal Leiomyoma is a benign and rare mesenchymal tumor that grows slowly. It was reported for the first time in 1858 by Forster. The Patients with Scrotal Leiomyoma is an asymptomatic painless mass., Case Presentation: The patient was a 45-year-old man, who presented with a painless scrotal mass to our hospital. On physical examination, he had a single, firm, and mobile mass about 2-3 cm in diameter within the right scrotum without tenderness. The left side was normal. Pre-operative scrotal sonography, a 2.5 cm hypoechoic extra testicular mass with sharp borders was reported. Intraoperatively The mass was excised totally and the testis was preserved. On microscopic examination revealed interlacing bundles of spindle-shaped cells with no mitotic figures or nuclear pleomorphism. On immunohistochemical analysis, the spindle cells were positive for SMA and negative for S100, and leiomyoma was confirmed., Clinical Discussion: For diagnosing leiomyoma, scrotal ultrasound is a first-line and noninvasive method that is confirmed by histologic examination. In histology, smooth muscle bundles are seen. Immunohistochemistry studies can confirm the diagnosis by identifying specific markers like vimentin, desmin, and smooth muscle actin. It is important to differentiate leiomyoma from a malignant type known as leiomyosarcoma., Conclusion: We report a rare case presentation of a 45-year-old man with scrotal leiomyoma that is confirmed by histology. It is important to differentiate leiomyoma from malignant course., Competing Interests: Conflict of interest statement The authors declare no conflict of interest., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Leiomyosarcoma is a rare type of soft tissue sarcoma especially giant ones (size >5 cm). It is usually arises from the uterus and retroperitoneum. Ischiorectal space is not a common site for primary leomyosarcoma. There are only few case reports about Ischiorectal leiomyosarcoma. We present a case of 25 yrs. old female patient, who presented with a swelling on left buttock. It measures 9 by 9.5 by 18 cm on MRI. Complete tumor excision done and immunohistochemistry tests confirmed the diagnosis of leiomyosarcoma. Due to its rarity, unusual site and large size leiomyosarcoma poses a great challenge for a surgeon both to diagnose and manage it. Early diagnosis and management improves prognosis and decreases recurrence rate because sarcomas have high recurrence rate. It is highly recommended to have multidisciplinary approach. The case report highlights on both diagnostic and therapeutic challenges associated with giant leiomyosarcoma. There are no strict guidelines to follow when managing this type of cases but rather approach has to be individualized. Since there are only few case reports on this specific pathology we advocate for surgeons to share their experience on similar cases. • We present a rare case of giant ischiorectal tumor in a 25 yrs old female. • Initial symptom was swelling on the left buttock, with current size of about 10cm by 6 cm. • On pelvic MRI 9*9.5*18cm lobulated, elongated left pararectal space mass and has clear fat plane with the rectum. • Complete excision with retained sphincter function and margin free status. Immunohistochemistry diagnosis leiomyosarcoma. [ABSTRACT FROM AUTHOR]

Primary thyroid leiomyosarcoma is an extremely rare soft tissue sarcoma, characterized by high malignancy and poor prognosis. Currently, only 13 cases of primary thyroid leiomyosarcoma have been described in the medical literature (limited to English). A 76-year-old female presented with a giant neck mass. Physical examination revealed a large, firm mass in the left thyroid gland. No symptoms such as hoarseness or dysphagia were noted at the time of presentation. The patient underwent unilateral thyroidectomy and cervical lymph node dissection. Pathologic findings revealed a low-grade sarcoma with spindle-shaped tumor cells in an interwoven, sheet-like distribution. Immunohistochemistry showed positivity for desmin, SMMHC, STAT6, CK19, and Galectin3, but negativity for S-100, MyoD1, CD34, CK (AE1/AE3), CD117, and CD56. The findings were consistent with thyroid leiomyosarcoma. The treatment of primary thyroid LMS presents challenges due to its atypical symptoms and high malignance, highlighting the imperative for further exploration of therapeutic strategies to improve the outcomes. • Primary thyroid leiomyosarcoma is rare. Only 13 cases have been described in the medical literature. • Primary thyroid leiomyosarcoma is extremely aggressive, with a high risk of recurrence and metastasis. • The diagnosis of primary thyroid leiomyosarcoma relies on the biopsy and immunohistochemical analysis. • Patients often experience tumor recurrence, distant metastasis, or even mortality shortly following surgery. [ABSTRACT FROM AUTHOR]

Uterine smooth muscle tumors of uncertain malignant potential (STUMP) are extremely rare, defined as a uterine smooth muscle tumors that cannot be diagnosed as benign or malignant and does not satisfy all the criteria for leiomyosarcoma or leiomyoma. A 48-year-old woman who presented with a history of heavy menstrual bleeding and pelvic pain. Physical examination revealed an enlarged uterus. Ultrasonography showed lobular and enlarged uterus containing multiples leiomyomas. A subtotal hysterectomy was performed. A Pathological analysis revealed a uterine mass diagnosed as a smooth muscle tumor of uncertain malignant. Uterine STUMPs are rare and are commonly diagnosed by histopathological evaluation following myomectomy or hysterectomy. The most common clinical manifestations of uterine STUMP are the same as leiomyomas. Prognosis for the patient is unclear and there is a risk of recurrence with the tumors. However, considering their potential risk of recurrence and metastasis, it is advisable to maintain six-monthly controls for 5 years and then annual controls for 5 years more. Due to the rarity of uterine STUMP, there are no specific guidelines for their treatment and control. The scientific literature needs to be constantly updated in order to identify masses suspected of malignancy before surgery and improve patient management and follow-up. • Uterine smooth muscle of uncertain malignant potential (STUMP) are extremely rare • Uterine STUMPs are commonly diagnosed by histopatholological evaluation following myomectomy or hysterectomy. • Total hysterectomy is an optimal treatment for stumps among women without a desire of fertility to prevent recurrences. • All uterine STUMP cases should be the object of a multidisciplinary approach [ABSTRACT FROM AUTHOR]

Radical surgical resection with negative margins is the mainstay of treatment for retroperitoneal vascular leiomyosarcomas. Given the retroperitoneal location of these tumors, open surgery is, historically, the chosen surgical approach, however, it is burdened with high postoperative morbidity. In selected cases, the small dimension of the tumor and a favorable location, allow to perform a minimally invasive treatment. A 67-year-old female patient with a diagnosis of a leiomyosarcoma arising from the left renal vein underwent a robotic resection of the left renal vein with preservation of the left kidney and a relative outflow trough the gonadal vessels. The patient was discharged on the fourth postoperative day without any complications and there was no tumor recurrence noted during the 24-month follow-up period. Vascular retroperitoneal leiomyosarcomas are very rare tumors requiring a complete en bloc gross tumor resection in order to achieving microscopically negative margins on the vein of origin. Thanks to the preoperative histological diagnosis and radiological study of the neoplasm, it was possible to proceed to a highly personalized and minimally invasive treatment with respect of oncological criteria. In selected cases, a minimally invasive surgery of vascular leiomyosarcoma could be a feasible and safe treatment option. • Vascular leiomyosarcoma is a very rare soft tissue sarcoma. • Surgery with a complete primary resection and with negative margins is the gold standard treatment. • Open surgery is burdened with high postoperative morbidity. • In selected cases a minimally invasive surgery of vascular leiomyosarcoma could be pursued. [ABSTRACT FROM AUTHOR]

• Intestinal leiomyosarcoma is a rare tumor with a decreased incidence due to redefinition by WHO. • Perforation is an atypical presentation of intestinal leiomyosarcoma. • Invasion of the surrounding intestine weakened the intestinal wall resulting in perforation. The majority of gastrointestinal sarcoma is gastrointestinal stromal tumors and intestinal leiomyosarcoma is rare. Small intestinal mesenchymal tumors are often large at diagnosis, and they commonly present with bleeding or intussusception. We report a perforation associated with intestinal leiomyosarcoma. A 66-year-old man presented with severe epigastric pain. A physical examination showed tachycardia and a diffusely tender and rigid abdomen. Computed tomography showed a massive tumor and free air. A laparotomy was performed to treat lower digestive perforation. Massive tumor, which invaded surrounding intestine, was 20 cm in size at the ileum. The involved intestine was perforated. We confirmed that feeding artery was superior mesenteric artery and performed partial intestinal resection. His clinical course was uneventful and discharged 10 days postoperatively. The pathological findings showed spindle shaped and the tumor invaded the mucosa at the perforated site. Immunohistochemical spectrum resulted c-kit negative, S-100 negative, Desmin positive, alpha smooth muscle actin(αSMA) positive and Ki-67 30–40 %. The pathological findings were leiomyosarcoma. Gastrointestinal sarcoma is sometimes found by bleeding. In our patient, leiomyosarcoma invaded surrounding intestine, it made the intestine wall frail and caused perforation. The intestinal perforation which was involved by leiomyosarcoma has been rarely reported to the best of our knowledge since WHO refined leiomyosarcoma. Although intestinal leiomyosarcoma is rare, we should know that it can involve surrounding intestines and make them perforated. [ABSTRACT FROM AUTHOR]

• The occurrence of extra-dermatological cancers has been rarely reported in Xeroderma Pigmentosum, we present an exceedingly rare association of XP and renal Leiomyosarcoma in 14-year-old patient. • Radical nephrectomy remains the treatment of choice in order to confirm the diagnosis and guide a multidisciplinary approach. • The potential benefits of adjuvant therapy should be discussed with selected patients to improve the poor prognosis of this condition. • Physicians must be aware of this rare association and renal imaging should be performed at the least clinical sign. Xeroderma pigmentosum (XP) is a rare genetic disorder causing extreme sensitivity to ultraviolet (UV) rays. It is transmitted by an autosomal recessive mode. This disease is classically characterized by a high risk of skin cancer. The occurrence of extra-dermatological cancers in patients followed for XP has been rarely reported in the literature. We present the case of a 14-year-old adolescent with an exceptional association of XP and renal leiomyosarcoma. A 14-year-old adolescent was presented with an abdominal mass revealed by hematuria and abdominal pain. Computed tomography (CT) of the abdomen showed an enhancing, heterogeneous right renal mass, consistent with the features of renal tumor. After non conclusive biopsy, a total nephrectomy of the right kidney was performed. Based on gross specimen observation and immunochemical analysis, the patient was diagnosed with high-grade renal leiomyosarcoma. An adjuvant chemotherapy was indicated and the patient was referred to medical oncology. At 12 months follow-up, the patient is in total remission; The CT scan did not show any locoregional or distant recurrence The risk of renal tumor is increased in patients followed for XP. Attending physicians should ask for renal imaging at the slightest sign of clinical call. [ABSTRACT FROM AUTHOR]

• GIST is the most common mesenchymal neoplasm of the digestive tract. • Have been described in the mesentery, omentum, gallbladder, bladder wall, and uterus (EGIST). • EGISTs are extremely rare and are usually detected incidentally. • This is the first reported case of a metachronous EGIST in utero. The gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor in the digestive tract. Currently, GIST is the name given to CD117 positive mesenchymal tumors, primary of the digestive tract, mesentery, and retroperitoneum. Nevertheless, they have been reported in the mesentery, omentum, gallbladder, bladder wall, and few cases in the uterus; known as extragastrointestinal stromal tumors (EGIST). Seventy-six-year-old woman with a history of the third recurrence of pelvic tumor located in the uterus initially diagnosed as uterine leiomyosarcoma. CT and MRI showed a tumor in the uterine corpus of approximately 10 cm. It was decided to perform the surgical rescue. The immunohistochemistry and anatomic pathology report revealed a tumor compatible with a uterine EGIST. It was decided to perform adjuvant treatment with imatinib. Currently, the patient continues to be disease-free 20 months after the surgery. For years, GIST has often been confused with leiomyosarcoma, given that they are histologically almost indistinguishable. The IHC analysis for KIT (CD117) has become essential in the GIST diagnosis. On the other hand, stromal tumors arising outside the gastrointestinal tract are rare (5%), which have a histological and biological behavior similar to that of GISTs. EGISTs are extremely rare and often incidentally detected. Currently, evidence about this location is scarce. According to the literature, this is the first case of uterine EGIST with a metachronous presentation. [ABSTRACT FROM AUTHOR]

• Here we reported a rare case of ileo-ileal intussusception caused by small bowel leiomyosarcoma. • Actually the case reported in literature of ileo-ileal intussusception are rare and mechanism is still unclear. • The cases of leiomyosarcoma present in the literature are few and even rarer those in adults. Intussusception is the telescoping of one segment of the bowel into an adjacent bowel segment, causing venous congestion, edema, and blood supply reduction. We present a case of ileo-ileal intussusception in an adult patient with intestinal obstruction caused by a rare mesenchymal malignant lesion of the distal ileum: Leiomyosarcoma (LMS). A 90-year-old Caucasian man presented to the hospital with a two-day history of abdominal pain, nausea, and bowel occlusion. Preoperative Computer Tomography (CT) showed a solid mass with stratified walls in the lumen of the cecum with the classics "bulls-eye" appearance with concentric rings, suggestive of intussusception. The patient underwent emergency laparotomy with evidence of a small bowel wall tumor driving ileo-ileal intussusception with ischemic damage. Ileocecal resection was performed without postoperative complications. Histopathological examination showed a tumor on the muscular layer of the small bowel. The definitive diagnosis was LMS. Adult intussusception is a rare condition, with an incidence of 2/1 000 000 cases per year worldwide. About 60% of patients suffering from this disease require surgery. Clinical presentation can be non-specific because of its no characteristic signs and symptoms. The most common presenting symptom is abdominal pain with bowel obstruction sings. Intussusception can occur anywhere along the small and large intestine and it is typically associated with a Lead Point (LP). The LP may be benign or malignant conditions. Infrequent malignant causes include LMS. Diagnosis of intussusception is relatively challenging because of its non-specific symptoms. CT scan is the examimation of choice for the diagnosis because of its peculiar images. In adults, surgical treatment is recommended with laparoscopic or open approach according to surgeon expertise, sometimes in an emergency setting. [ABSTRACT FROM AUTHOR]

• Leiomyosarcoma is a rare tumor accounting for less than 1% of adult malignancies. • So far, only approximately 450 cases have been reported in literature. • Surgical resection is the treatment of choice. • Case report of a patient with leiomyosarcoma originating from the inferior vena cava. • The patient is free of disease recurrence 24 months after surgery. Leiomyosarcomas are rare and heterogeneous group of tumors that account for less than 1% of adult malignancies. More than 50% of all vascular leiomyosarcomas occur in the lower part of vena cava. Since the first description of Perl and Virchow in 1871, only approximately 450 cases have been reported in literature. The patient presented due to abdominal pain and weight loss. Based on the imaging evaluations a retroduodenal tumor with compression of the inferior vena cava was observed. In the explorative laparotomy a leiomyosarcoma originating from the inferior vena cava was identified. Considering the extensive intramural and intraluminal tumor manifestation, the patient underwent a segmental resection of the vena cava. Reconstruction was achieved by implanting a polytetrafluoroethylene (PTFE) prosthesis. Postoperatively a stenosis developed due to a pericaval haematoma with consecutive compression of the prosthesis. An angiographic implantation of a stent was successfully performed. In the 24-month follow-up, the patient is free of symptoms and tumors. Leiomyosarcomas of the vena cava are classified anatomically according to their relationship to the liver and renal vessels. The clinical symptoms depend on the affected segment. The therapy of choice is radical en bloc tumor resection. After resection, the options for reconstruction include placement of a synthetic graft, primary repair and patch repair. Due to a variety of topographic and tumor biological sarcoma manifestations, no standard has been established for the resection of this entity. The extent of resection should be planned individually. [ABSTRACT FROM AUTHOR]

• Leiomyosarcoma of the inferior vena cava are very rare neoplasm with a limited responsiveness to medical therapy. • Surgery seems to be the only chance of cure. • Complex vascular resection are often necessary and required accurate reconstruction technique. • Peritoneal patch is an efficient and safe option to repair venous defects and it is gaining great attention in recent years. Leiomyosarcomas (LMs) of the inferior vena cava (IVC) are very rare neoplasms seldom reported in the literature. The majority of patients does not present with specific abdominal pain and IVC LMs are used to become symptomatic with the increase of tumor volume. The role of chemotherapy or radiotherapy is not yet defined and surgical resection seems to be the only chance to improve survival rates. We present a case of a 58-year-old female with a recent diagnosis of IVC LM who underwent surgery with a partial resection of the anterior wall of the vein using a lateral and partial vein clamping. The primary repair of the defect could result in stricture of the vein, so a parietal peritoneum patch was used for the vein reconstruction. The postoperative course was uneventful. Actual evidence suggests that vascular sarcomas have limited responsiveness to cytotoxic chemotherapy and chemoradiotherapy, so surgery is the treatment of choice. Major surgery entailing multivisceral and complex vascular resection is usually necessary to achieve negative margins and accurate vascular reconstruction techniques are mandatory to avoid serious circulatory complications. Different kinds of graft (biological or synthetics) are available for the reconstruction, with intrinsic advantages and limitations. The use of peritoneal patches seems a valid and cheap option for vascular reconstruction and it is gaining great attention in recent years. This case demonstrates that peritoneal graft could be a safe option to manage IVC defects in expert hands. A brief review of literature is also included. [ABSTRACT FROM AUTHOR]

The management of patients with complex oncological histories poses unique challenges, particularly when they are on targeted chemotherapy agents known for specific side effects. This case report illuminates the multifaceted complexities encountered in such scenarios, with a focus on the rare complications associated with targeted therapies. We present a 50-year-old male with an extensive oncological background, including childhood retinoblastoma and radiation-induced leiomyosarcoma. Recently diagnosed with skull base osteosarcoma, he was undergoing treatment with Regorafenib. Admitted with sepsis due to Pseudomonas aeruginosa -induced community-acquired pneumonia, his clinical course was complicated by lung cavitation leading to a spontaneous pneumothorax. This report highlights the absence of empyema, a crucial differential in the diagnosis. This case unravels the intricate interplay between targeted chemotherapy, concurrent medications like prednisone, and their potential to cause severe complications such as pneumonia and pneumothorax. It delves into the mechanisms by which Regorafenib can lead to lung cavitation and abscess formation, a rare but significant risk. The importance of a multidisciplinary approach for prompt diagnosis and treatment, including surgical intervention, is highlighted. The pathology of the surgically resected lobe revealed metastatic high-grade leiomyosarcoma, adding another layer of complexity to the case. This case serves as a cautionary tale highlighting the need for vigilant monitoring of patients on targeted chemotherapy agents, especially those with complex medical histories. It highlights the importance of considering potential drug-related complications and the rationale behind therapeutic choices, including antibiotic selection and surgical decision-making, in the management of acute medical conditions in these patients. • Complex oncological history with childhood retinoblastoma, radiation-induced leiomyosarcoma, and skull base osteosarcoma. • The patient presented with sepsis and community-acquired pneumonia while on chemotherapy and prednisone. • Spontaneous pneumothorax required urgent surgical intervention, revealing metastatic leiomyosarcoma in the resected lobe. • The case illustrates the potential for cavitation and subsequent complications with targeted chemotherapy agents. • Emphasizes the need for a multidisciplinary approach and careful medication monitoring. [ABSTRACT FROM AUTHOR]

• Leiomyosarcoma of the spermatic cord is a rare entity. • The diagnosis of spermatic cord leiomyosarcoma is difficult based on imagery alone. • The standard treatment is radical orchidectomy with high ligation of the spermatic cord. Leiomyosarcoma of the spermatic cord is a rare entity. It presents as a firm and painless intrascrotal mass. For diagnosis radiological methods such as ultrasound, CT or MRI are necessary. Radical orchiectomy is the gold standard of treatment. We reported a one case of a 66-year-old man that he has suffered from a painless hard mass in the right hemiscrotum since three years. Ultrasound revealed a paratesticular mass. Further workup revealed no distant metastasis before surgery. Right radical orchiectomy was performed, the final pathologic examination showed a leiomyosarcoma of the spermatic cord. The patient remained disease-free twelve-month during follow up after surgery. Leiomyosarcoma of the spermatic cord is a rare condition, it arises from mesenchymal cells of the spermatic cord and occurs mainly in elderly patients. The diagnosis of spermatic cord leiomyosarcoma is difficult if based only on imaging procedures, it is revealed by histological examination post surgery. No treatment protocol has yet been established for paratesticular leiomyosarcoma due to the rarity of the disease. Standard treatment is radical orchidectomy with high ligation of the spermatic cord. The benefit of adjuvant chemotherapy, radiotherapy, and retroperitoneal lymphadenectomy are not well understood and may differ according to the surgeon's preferences. The lack of such cases results in the challenging nature of the diagnosis and treatment of leiomyosarcoma of the spermatic cord. Additional studies are needed to better define optimal management strategies. [ABSTRACT FROM AUTHOR]

Leiomyosarcoma is a malignant neoplasm that is derived from smooth muscle cells in walls of small blood vessels or branch of the inferior vena cava, the uterus and the gastrointestinal tract. Different treatment options are present for the treatment of LMS. However, due to the rarity of LMS, the optimal treatment option is still to be discussed and determined. A 51-year-old male patient, previously healthy, presented for perianal pain. Biopsy of the mass found showed spindle cell tumors with mild atypia, dense cellularity, and pelvic MRI with contrast showed a well-circumscribed mass of the anus, developed between the layers of the external sphincter with possible invasion of the internal sphincter consistent with Leiomyosarcoma Grade I. Wide excision was performed. Close follow-up should be done every 3 to 6 months for the first 2 to 3 years, every 6 to 12 months for the following 3 years, and annually afterwards. The symptoms of LMS include rectal bleeding with rectal and/or abdominal pain, weight loss, constipation, altered bowel motion and protruding mass. Treatment options include wide local excision, abdominoperineal resection, low anterior resection, bloc resection and pelvic exenteration. Patients who underwent wide local excision show a higher local recurrence rate as compared to patients who underwent radical resection. Distant metastasis is higher in patients who underwent radical resection. The treatment options of anal LMS are controversial. At present, very few cases have been reported, thus no universally accepted standard of surgical treatment has been established. • Patients who undergo wide local excision show higher local recurrence rate • Treatment options include wide local excision, abdominoperineal resection, low anterior resection, bloc resection and pelvic exenteration. • No universally accepted standard of surgical treatment has been established. • Close follow-up should be done every 3 to 6 months for the first 2 to 3 years. [ABSTRACT FROM AUTHOR]

Our study aims to expose the experience of our department in dealing with bladder leiomyosarcomas and illustrate the management tools of this rare pathology. We present a retrospective study of 4 cases of bladder leiomyosarcoma, gathered in the Department of Urology during the period of 1996–2022. All our patients were exclusively male and aged between 35 and 73 years. No history of pelvic irradiation nor chemotherapy was found in our patients. Three patients had high-grade leiomyosarcoma and pT2 stage whereas only one had a low-grade tumour. Three patients underwent radical treatment by cystoprostatectomy with extensive bilateral pelvic lymph node dissection and one patient was treated by endoscopic re-resection and endoscopic monitoring. We noticed 2 lymph nodes and liver metastasis recurrences in 2 patients treated by radical surgery while 2 patients didn't present recurrences at two years of follow-up. To date, there is no clear and precise therapeutic approach for the treatment of bladder leiomyosarcoma. Little is known about the long term survival associated with these tumours. All studies agree that the prognosis for bladder leiomyosarcoma is poor, if not diagnosed early, especially those presenting with an undifferentiated tumour grade, distant metastatis and treated without surgical therapy. Bladder leiomyosarcoma is a rare and highly aggressive tumour. The anatomopathological examination provides diagnosis and prognosis assessment. Radical surgery remains the most suitable therapeutic approach. • Bladder leiomyosarcoma is rare and highly malignant. Diagnosis and prognosis are based on pathological examination. • The FNCLCC system seems to have the best performance in terms of objectivity and assessment of differentiation, mitotic activity and necrosis. • There is no consensus on management. • Radical surgery is the most suitable treatment option. • Chemotherapy and radiotherapy are promoted by some teams. [ABSTRACT FROM AUTHOR]

• Leiomyosarcoma and osteosarcoma are exceedingly rare subtypes of breast sarcoma. • Given the exceeding rarity of breast leiomyosarcoma and osteosarcoma, consensus on ideal management of this diseases is still on debate. • We report a case of an 87-year-old female with giant breast leiomyosarcoma and osteosarcoma, treated by surgical resection and radiotherapy. • Osteosarcoma may have a rapid growth pattern with no other clinical features distinguishable to other malignant breast neoplasms. Breast sarcomas are a rare group of malignant tumors accounting for less than 1% of all malignant neoplasms of the breast and fewer than 5% of all sarcomas. We report a case of an 87-year-old caucasian female who recurred to the emergency department with complaints of a painful mass of the left breast with purulent discharge. Observation revealed a volumous mass in the inferior quadrants of the breast, ill defined, with petrous consistency, areas of necrosis, and inflammatory signs. She was admitted to Surgery ward for further study and therapy of a probable inflammatory tumor of the breast. Magnetic resonance image was obtained, raising suspicion on papillary carcinoma and classified the breast as BIRADS5. Microbiological and cytological exams of the exudate were negative. An incisional biopsy of the tumoral mass was also obtained, and the patient discharged while waiting for surgery. Histological exam and immunohistochemical essay were compatible with leiomyosarcoma. Left mastectomy was performed and the patient was discharged with no morbidities on the 5th day after surgery. Histological exam of mastectomy piece showed a metaplastic carcinoma, with osteosarcomatous and focal leiomyosarcomatous differentiation. The lesion was classified as pT4N0M0 and subsequent radiotherapy was performed. Twenty months after surgery the patient was being followed-up on Oncology and Senology consultations and remained asymptomatic. Consensus on ideal management of this diseases is still on debate. Some authors defend the treatment of this entity in a similar way to sarcoma of the breast. More studies are needed to better understand this entity. [ABSTRACT FROM AUTHOR]

• Leiomyosarcoma is a rare soft tissue cancer. • Retroperitoneal tumors must be ruled out if the tumor is suspected to be a regional recurrence. • Early diagnosis and concerted therapeutic efforts are important for managing misdiagnosed metastatic gastric cancer. Leiomyosarcoma is a rare soft tissue cancer that arises from smooth muscle cells that form involuntary muscles. Here, we report a rare case of retroperitoneal leiomyosarcoma that mimicked a metastatic tumor arising from a gastric cancer. A 43-year-old man underwent radical total gastrectomy for gastric cancer. He underwent adjuvant chemotherapy with oral 5-fluorouracil (5-FU) for 2 years and regular follow-up. Twenty-nine months after gastrectomy, computed tomography (CT) was performed and revealed a solitary localized mass measuring 43 mm in diameter among retroperitoneal lymph nodes of the posterior inferior vena cava. This finding indicated that the previous gastric cancer recurred either in the lymph node or retroperitoneum. Second-line chemotherapy, consisting of oxaliplatin and 5-FU (FOLFOX) was administered in four cycles. However, serial CT scans showed increased tumor size. Retroperitoneal tumor did not respond to treatment, surgical resection was performed. Pathological findings led to a diagnosis of pleomorphic and epithelioid leiomyosarcoma without metastatic adenocarcinoma of the stomach. When a diagnosis of locoregional recurrence of gastric cancer in the retroperitoneum is considered, retroperitoneal leiomyosarcoma should also be considered in the differential diagnosis. Early diagnosis and concerted therapeutic efforts are important for managing misdiagnosed metastatic gastric cancer. [ABSTRACT FROM AUTHOR]

Introduction Primary leiomyosarcomas of the breast are extremely rare. Because of this rarity, only a small number of studies have been published about the disease; diagnosis, treatment, and prognosis are not well-described. Our work has been reported in line with the SCARE criteria. Presentation of case We present a 20-year-old female with primary breast leiomyosarcoma who despite mastectomy, developed lung metastases three years later. Discussion We discuss the rarity of breast leiomyosarcoma and risk of metastatic disease even with treatment. We review the literature and provide an outline of available data to shed light on the best strategies to manage this aggressive disease. Conclusion Physicians and surgeons treating breast leiomyosarcoma must be vigilant of potential lung metastases in order to optimize short- and long-term oncologic outcomes. [ABSTRACT FROM AUTHOR]

Introduction Primary pancreatic leiomyosarcoma is extremely rare. We report a case in which six additional resections were required to treat recurrent tumors in a 5-year period following the primary operation. Presentation of case A 69-year-old man presented with a pancreatic tumor. Abdominal computed tomography scan showed a large heterogeneous mass with a necrotic area arising from the pancreatic body. We performed distal pancreatectomy, splenectomy, and wide resection of the transverse mesocolon. Histopathological examination confirmed the diagnosis of a pancreatic leiomyosarcoma. We repeatedly performed surgery on recurrent tumors. Discussion Primary pancreatic leiomyosarcoma is considered to be a highly aggressive malignancy. The most effective treatment is complete surgical resection with tumor-free margins. Even when tumors recur, it is possible to improve the prognosis by further resection. Conclusion Long-term survival is achievable by repeated resection of recurrent tumors. [ABSTRACT FROM AUTHOR]

Introduction To describe an unusual primary vertebral leiomyosarcoma in thoracic spine. Presentation of case An isolated lesion of the T11 vertebra in a 62-year-old woman with no neurologic deficit is reported. Imaging findings indicated a nonspecific high-grade malignant lesion. TC-guided biopsy failed thus open incisional biopsy was needed. A diagnosis of low-intermediate mesenchymal sarcoma was made. A total en bloc spondylectomy of T11 was performed with three-column reconstruction. The histology and immunostaining showed the appearance of leiomyosarcoma. After diagnosis, post-operative radiation therapy was performed. Metastatic lesion was ruled out by CT scans of the chest, abdomen and pelvis, in addition to total body radionuclide scanning and 18-F-FDG-PET. After five years of follow-up, no signs of local recurrence, metastasis or distant lesions suggesting a primary lesion were observed. Discussion Vertebral primary leiomyosarcoma is exceedingly rare. Primary vertebral leiomyosarcoma diagnosis must be performed when the metastatic origin is excluded. For the treatment of primary tumors, total en bloc spondylectomy (TES) is the technique of choice to achieve marginal or wide tumor resection, decrease the risk of local recurrence and remote lesions and increase survival. Conclusions A well-planned pre-operative study and a wide surgical excision can result in local tumor control and long-term survival. This case presents the longest disease-free survival period of a primary leiomyosarcoma in spinal location after total en bloc spondylectomy. [ABSTRACT FROM AUTHOR]

Introduction Although rare, cases of abdominal sarcomatosis (AS) after laparotomy/laparoscopic interventions for uterine smooth muscle tumors of uncertain malignant potential (STUMP) have been reported. Presentation of case We describe a rare case of diffuse abdominal sarcomatosis in a patient that some year earlier had undergone myomectomy for a suspected uterine myoma, which was histologically proven to be a STUMP. Once the patient was admitted at our Department, she underwent a diagnostic laparoscopy that confirmed a condition of peritoneal sarcomatosis disseminated through the entire abdomen, and then a laparotomic total hysterectomy, bilateral salpingo-oophorectomy, and total omentectomy, achieving a complete cytoreduction. Histological examination showed high-grade uterine leyomiosarcoma (LMS). Since there is no evidence of any clinical benefit of adjuvant treatment, given the risk of disease recurrence, we decided, with the patient’s agreement, to conduct close follow-up with a Positron Emission Tomography (PET)/Computed Tomography (CT) scan every 3 months and diagnostic laparoscopy every 6 months, even in the absence of PET/CT positivity. After 2 years PET/CT showed a relapse of LMS in the perigastric region and, therefore the patient underwent a diagnostic/operative laparoscopy with complete removal of the neoplastic recurrence. To date, the patient is disease-free. Discussion and conclusion STUMPs should be submitted to a frequent surveillance for their risk of recurrence, dissemination and transformation into LMS, even many years after the first diagnosis. A follow-up including surgical re-exploration with laparoscopy and PET/CT imaging may allow early detection and timely treatment of the relapse with good long-term outcome, as demonstrated by our case. [ABSTRACT FROM AUTHOR]

Introduction Leiomyosarcoma of the gastrointestinal tract is very rare, with a reported frequency of less than 0.1% of all malignancies of the colorectum. It is important to diagnose leiomyosarcoma definitively by immunohistochemical profiling of smooth muscle actin, desmin, and CD34. True leiomyosarcoma of the colorectum diagnosed by immunnohistochemical profiling is extremely rare that only 13 reports have been published in reviews of resected gastrointestinal mesenchymal tumors after 1998. In addition, lymph node involvement is rare in patients with leiomyosarcoma. Herein we report an aggressive case of LMS in a rectosigmoid lesion with lymph node metastasis. Case presentation A 76-year-old woman visited our hospital complaining of intermittent anal bleeding that had lasted 5 months. Image studies aiming at examining the cause of her anal bleeding revealed a tumor located between the right ovary, uterus, and the rectosigmoid. Histopathology of biopsied materials from the colonoscopy suggested a malignant tumor of mesenchymal origin. Surgical resection was performed with curative intent. The tumor was diagnosed as leiomyosarcoma by pathological examination. Moreover, one of the 31 regional lymph nodes retrieved was metastasized by leiomyosarcoma. Eight months later, follow-up CT scans revealed multiple recurrent lesions in the liver and peritoneum. Despite systematic chemotherapy, she deceased 12 months after the surgery. Conclusion It is crucial to diagnose leioyosarcoma precisely based on immunohistochemistry, and thereby distinguish it from GIST. Although lymph node metastasis is rare, lymphadenectomy appears to be important for high-risk LMSs to perform R0 resection. Further investigation on leiomyosarcoma cases so far is required to establish standard treatment strategies. [ABSTRACT FROM AUTHOR]

Leiomyosarcomas of the inferior vena cava are scarce. Surgery is the only potential curative treatment. Such a surgery is complex and technically demanding, requiring a huge experience in both visceral and vascular surgery. Indeed, enlarged visceral resection may be needed to achieve a free tumor margins as the tumor may invade the duodenum, the head of the pancreas and the liver. Moreover, vascular reconstruction is mandatory which might be complex as both venal reins are usually invaded by the tumor like in our case below. A 53-year-old woman presented with abdominal vague pain. Imaging consisting in abdominal CT-scan with contrast and magnetic resonance angiography were suggestive of the diagnosis of leiomyosarcoma of the inferior vena cava. A surgical complete resection with free margins was conducted. A primary repair of the inferior vena cava and the right renal vein walls. End-to-side anastomosis with a polytetrafluorethylene (PTFE) prosthesis was mandatory to reconstruct the renal left vein to the inferior vena cava. Postoperative outcomes were uneventful. Pathology examination confirmed the diagnosis. The optimal management of leiomyosarcoma of inferior vena cava is controversial. Surgery remains the cornerstone of treatment. However, not all patients are qualified for surgical resection. The prognosis depends on the early diagnosis and histology type. The management of leiomyosarcomas of inferior vena cava requires a multidisciplinary consensus involving experienced surgery and oncology teams. • Leiomyosarcoma are rare malignant tumors. • Symptoms are nonspecific, making multiplanar imaging with contrast the cornerstone to suspect the diagnosis. • Surgical complete resection is the gold standard therapy. • Surgery is challenging, as it requires experienced surgeons in vascular reconstruction. • Pathology examination with immunochemistry set the final diagnosis. [ABSTRACT FROM AUTHOR]

Primary leiomyosarcoma is an uncommon form of stromal breast sarcoma. Approximately 73 cases have been documented in English-language literature to date. To our knowledge, this is the first report from Indonesia of an adolescent female with primary leiomyosarcoma of the breast. A 30-year-old Southeast Asian female presented with a tumor in her left breast. Clinical examination revealed a 12 × 8-centimeter tumor. The supraclavicular, subclavicular, and axillary lymphadenopathy were not palpable. An ultrasound revealed a Breast Imaging Reporting and Data System category 5. Abdominal ultrasonography and chest x-ray were normal, as were blood chemistry and routine blood tests. A wide excision with a surgical margin of 2 cm was performed. Pathological investigation identified the mass as a leiomyosarcoma. The pelvis, abdomen, and lung CT scan metastatic workups were negative. The patient is well 8 months post-surgery, with no signs of recurrence. Wide local excision has been the mainstay of treatment for leiomyosarcoma; however, there is no accepted standard of treatment due to the rarity of the disease. Breast leiomyosarcomas have a more favorable prognosis than other breast neoplasms; however, patients must be closely monitored for recurrence or metastases. While there are no known predictors of outcomes, the margins of the initial surgery, mitotic activity, and atypia cellularity are more indicative of malignancy. • Primary leiomyosarcoma is an uncommon form of stromal breast sarcoma. • To our knowledge, this is the first report in Indonesia of an adolescent female with primary leiomyosarcoma of the breast. • A wide excision with a surgical margin of 2 cm was performed. • Eight months post-surgery, the patient was well and showed no recurrence. [ABSTRACT FROM AUTHOR]

Introduction Greater omentum leiomyosarcomas are rare tumors with only a few cases reported in literature. Presentation of case We report the case of a 68-year-old man who consulted complaining of diffuse abdominal pain without a palpable mass at physical examination. Imaging studies revealed a solid-cystic lesion in the right lower quadrant. Surgical resection was performed and the tumor was diagnosed as a leiomyoscarcoma by histological and immunohistochemical examinations. Discussion Surgical resection of all lesions seems to be a reasonable therapeutic approach if resection is feasible. Chemotherapy may be used in selected cases. Conclusion More cases are needed to define the best treatment approach of this disease. [ABSTRACT FROM AUTHOR]

We present a case of a 70-year-old female with Leiomyosarcoma (LMS) of the inferior vena cava (IVC). Although this is an extremely rare entity, in contradistinction, it is also the most common primary malignancy of the IVC [5]. The patient has a history of sarcoidosis, hypertension, diabetes mellitus type two, and chronic obstructive pulmonary disease (COPD). She presented with a complaint of bilateral lower extremity edema and was admitted where a computerized tomography (CT) scan of the abdomen and pelvis showed a large mass filling the IVC, a finding confirmed by magnetic resonance imaging. Radical resection of the retroperitoneal tumor was carried out including portions of the inferior vena cava with en bloc radical right nephrectomy and right adrenalectomy. The pathologic diagnosis of inferior venal caval leiomyosarcoma (IVC LMS) was made with positive immunostains for desmin, vimentin and smooth muscle actin. The rarity of this entity, clinical presentation along with concomitant sarcoidosis makes this an interesting case. • Leiomyosarcoma of the inferior vena cava with bilateral lower extremity edema. • The link between sarcoidosis and sarcoma that has yet to be determined. • Sarcoidosis within lymph nodes mistakenly leading to higher tumor stage. • Bilateral lower extremity edema secondary to tumor thrombus in the IVC. [ABSTRACT FROM AUTHOR]

INTRODUCTION Leiomyosarcoma of the small bowel is an extremely rare form of gastrointestinal malignancy. Small bowel tumours are usually asymptomatic at the early stages, and difficult to visualise by upper and lower endoscopy. PRESENTATION OF CASE An 83-year-old gentleman presented in surgical outpatient clinic with chronic anaemia, abdominal discomfort and a single episode of malaena. Initial OGD and colonoscopy were both unremarkable. Subsequent CT revealed a mass in the right iliac fossa of likely small bowel origin, leading to an urgent laparotomy and resection with primary anastomosis. Histopathology showed a high grade leiomyosarcoma with no signs of metastasis and confirmatory immunological staining. Post-surgery follow up remains unremarkable. DISCUSSION Leiomyosarcomas of the small bowel are extremely rare entities, particularly following the advent of robust immunohistological diagnostic methods allowing differentiation from GISTs. As small bowel tumours are often not visualised by upper and lower endoscopy, further investigations to visualise the small bowel are crucial, generally in the form of magnetic resonance enterography, CT colonography or wireless capsule endoscopy. CONCLUSION The treatment of such tumours remains predominantly centred around surgical resection, and prognosis is dependent on tumour size and histological staging. [ABSTRACT FROM AUTHOR]

INTRODUCTION Retroperitoneal tumors are rare, mostly malignant. Locally aggressive, and more frequent in women in their 5th decade of life. Its symptoms are nonspecific, including abdominal pain and palpable mass. To diagnosis is helpful computed tomography and biopsy. It needs surgery for absolute healing. PRESENTATION OF CASE 67 years old man was admitted with back pain and fever. Abdominal imaging tests showed a 15 cm abdominal mass without clear organodependencia. Endoscopy with biopsies evidenced mesenchymal neoplasia of undetermined origin. In surgery we confirm its resecability and was necessary multiorgan resection. Pathologic diagnosis : well differentiated retroperitoneal leiomyosarcoma. Started adjuvant radiotherapy. In subsequent tests showed the presence of liver metastases. DISCUSSION Retroperitoneal tumors are developed from nerve, vascular, muscular, connective, supportive and fibroareolar tissue from this space. Its size does not modificate survival or resectability. We used TC and biopsy for its diagnose. Adjuvant therapy does not affect survival or quality of life, surgery remains the only curative option. Locoregional recurrence is the most influential figure in the prognosis. A large percentage of patients required a second surgery (between 45 and 82%). CONCLUSION The only curative option of retroperitoneal sarcomas is surgery, which usually requires multiple organ resection. Chemotherapy and radiotherapy are mostly a surgical supplement. Chemotherapy has not shown significant increase in survival. [ABSTRACT FROM AUTHOR]

INTRODUCTION Leiomyosarcomas are an infrequent cause of malignant superior vena cava syndrome (VCS). PRESENTATION OF CASE A 51-year old male patient was admitted for a three-day history of dyspnoea, dysphagia and erythema of the head and neck. Computed tomography and magnetic resonance imaging showed a lesion arising on the anterior mediastinum, which was in close proximity with a thrombus in the superior vena cava. Surgical excision was performed, including open resection of the primary tumour and an atrio-innominate vein bypass with 8-mm polytetrafluoroethylene (PTFE). Histology confirmed a leiomyosarcoma and postoperative radiotherapy sessions were performed. Due to evidence of enlargement of the thrombus, a second intervention was undertaken. In this procedure, a remainder of the primary tumour was resected and the superior vena cava reconstructed with an autologous pericardium patch. The patient recovered satisfactorily and was discharged on the seventh postoperative day, with no evidence for relapse after 10 months of follow-up. DISCUSSION Leiomyosarcomas comprise less than 2% of the tumours of the mediastinum and are a rare cause of paraneoplastic VCS. Male patients in their sixties are most commonly affected. Relapses seem to be common, and thus a careful follow-up is often recommended. CONCLUSION In spite of the limited data on the management of thoracic leiomyosarcomas, surgery is currently considered the mainstay of treatment. [ABSTRACT FROM AUTHOR]

Malignant tumors of the small bowel are rare. The jejunum, ileum, and duodenum represent the most common sites of intestinal leiomyosarcoma (LMS). Herein, we present a case of a 65-year-old patient having ileal LMS successfully treated with surgical resection. A 65-year-old patient, with no comorbidities, presented with chronic and paroxysmal abdominal pain. Upper endoscopy and colonoscopy showed no abnormalities. Thoracoabdominal computed tomography (CT) revealed an ileal lobulated, heterogeneously enhancing solid mass measuring 6 cm. Laparotomy was performed. Findings showed a lobulated ileal mass. We made an enlarged ileal resection with end-to-end anastomosis. The postoperative course was uneventful. Histology and IHC stains concluded into ileal LMS. No relapse of the disease was noted during the 4-month follow-up. Ileal LMS is a rare tumor originating from the smooth muscle cells within the muscularis mucosa or muscularis propria. CT colonography (CTC) and magnetic resonance enterography (MRE) represent good options to aid the diagnosis. Histologically, LMS often has a comparable morphological appearance to GISTs. IHC is essential to differentiate those tumors. Surgery is the only curative treatment. The prognosis is poor knowing that those tumors are discovered at advanced stages. Ileal LMS is a rare tumor originating from the smooth muscle cells. It has a comparable morphological appearance to GISTs. Immunohistochemistry is essential to confirm the diagnosis. Surgery is the only curative treatment. The prognosis is poor. • Small bowel leiomyosarcoma (LMS) wall is an extremely rare tumor. • LMS originates from the smooth muscle cells. • Immunohistochemistry is essential to confirm the diagnosis. • Those tumors have a low response rate to chemotherapy. • Surgery is the only curative treatment. [ABSTRACT FROM AUTHOR]

Abstract: INTRODUCTION: Metastatic tumors of the pancreas are uncommon and rarely detectable clinically. Metastases to the pancreas are rare. We present a patient with pancreatic metastases from a leiomyosarcoma of the uterus and review the literature about the clinical features of pancreatic metastasis and its surgical management. PRESENTATION OF CASE: A 40-year-old woman, who underwent hysterectomy, left oophorectomy, omentectomy and lymp node dissection for leiomyosarcoma of the uterus. At the follow up, the patient complained of non-specific abdominal discomfort. Preoperative diagnosis were pancreatic pseudocyst, cystadenoma or cystadenocarcinoma. At laparotomy, a cystic mass was found in the tail of the pancreas which was invased to the transverse colon mesenterium and the spleen. Distal pancreatectomy with splenectomy and transverse colon resection was performed. Histologically, the tumor was evaluated as poorly differentiated leiomyosarcoma. DISCUSSION: Metastatic lesions of the pancreas are uncommon and less than 2% of all pancreatic malignancies. However a few cases of leiomyosarcoma with metastases to the pancreas have been reported in the literature. Before deciding that the lesion in the pancreas was metastasis, primary leiomyosarcoma of the pancreas had to be ruled out. Histologically, leiomyosarcoma of the pancreas contains interlacing spindle cells with varying degrees of atypia and pleomorphism. The surgical approach to the pancreatic metastases must be aimed complete excision of the tumor with a wide negative margin of clear tissue and maximum preservation of pancreatic remnant if possible. CONCLUSION: In the absence of widespread metastatic disease, aggressive surgical approach with negative margins must be aimed. [Copyright &y& Elsevier]