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Lymph node metastases in non-well differentiated thyroid cancer (non-WDTC) are common, both in the central compartment (levels VI and VII) and in the lateral neck (Levels II to V). Nodal metastases negatively affect prognosis and should be treated to maximize locoregional control while minimizing morbidity. In non-WDTC, the rate of nodal involvement is variable and depends on the histology of the tumor. For medullary thyroid carcinomas, poorly differentiated thyroid carcinomas, and anaplastic thyroid carcinomas, the high frequency of lymph node metastases makes central compartment dissection generally necessary. In mucoepidermoid carcinomas, malignant peripheral nerve sheath tumors, sarcomas, and malignant thyroid teratomas or thyroblastomas, central compartment dissection is less often necessary, as clinical lymphnode involvement is less common. We aim to summarize the medical literature and the opinions of several experts from different parts of the world on the current philosophy for managing the neck in less common types of thyroid cancer. ispartof: CANCERS vol:15 issue:4 ispartof: location:Switzerland status: published

Leiomyosarcomas of the sigmoid colon are aggressive and extremely rare tumors representing less than 0.1% of all colorectal malignancies. Few cases have been reported in the literature and their imaging features need to be more detailed. We report the case of a 70 year old male patient, smoker, with a history of arterial hypertension, admitted for abdominal pain and hypogastric mass. Computed tomography has shown a voluminous heterogeneously enhancing tumor process with a necrotic center, attached to the sigmoid wall, with multiple secondary peritoneal and hepatic masses. The anatomopathological examination of the biopsy sample with immunohistochemistry allowed the diagnosis of leiomyosarcoma of the sigmoid colon. Clinical presentation and radiological features of leiomyosarcomas are non-specific, and the definitive diagnosis is only established after an anatomopathological examination.

The authors present an unusual case of a leiomyosarcoma of the distal tibia. Leiomyosarcoma tumors typically originate from smooth muscle tissue. It is rare for it to derive from bone and even rarer to be found in a bone of the lower limb. Given this extreme rarity in addition to nonspecific findings on plain film radiographs and magnetic resonance imaging (MRI), biopsy was needed in this case. It was only through immunochemistry staining that a definitive diagnosis was made. As such, this case is an illustrative example of an aggressive, though rare, primary lesion of the bone which should be considered in the differential diagnosis of a lytic intramedullary lesion. This case also highlights the need for careful evaluation of imaging features suggesting a potentially aggressive lesion requiring appropriate work up in a timely fashion.

Background Primary leiomyosarcoma (LMS) of the gastrointestinal (GI) tract is rare. Limited literature exists regarding the clinical characteristics and outcome for patients with localised and metastatic disease. Methods A retrospective chart review was performed for patients greater than 18 years of age diagnosed with GI LMS at The Royal Marsden Hospital between 1 January 2000–1 May 2020. Descriptive statistics were performed. Patients were censored at data cut-off date of 27 June 2020. Results Forty-six patients with a median age at diagnosis of 54 years (range 25–85) were identified. Fifteen percent (n = 7) of patients previously received abdominal radiation for an unrelated cancer. All patients with localised disease (n = 36) had resection with oncological margins. For patients who underwent potentially curative surgery, median recurrence-free survival (mRFS) was 13 months (0.4–183 months), and half of these patients (n = 18) developed recurrent disease post resection (distant n = 16, local n = 2). Median overall survival (mOS) was 27 months for patients with distant recurrence. Twenty-one percent (n = 10) of patients presented with synchronous metastatic disease and their mOS was 19 months. Median progression-free survival (mPFS) for patients treated with conventional chemotherapy ranged from 2.0 to 8.0 months. Conclusion The risk of recurrence is significant, and recurrence-free survival was short even with complete oncologic resection. The relationship of prior abdominal radiotherapy to the development of GI LMS warrants further investigation. Outcomes with systemic therapy for metastatic disease were poor and there is a need for the development of more effective systemic therapies.

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare soft tissue sarcoma associated with poor prognosis. Patients are often asymptomatic or present with nonspecific abdominal symptoms, which delays initial diagnosis and contributes to poor oncologic outcome. Key imaging modalities include ultrasonography (US), computed tomography (CT), and magnetic resonance imaging (MRI). Characteristic imaging features include imperceptible caval lumen, dilation of the IVC, heterogeneous enhancement of the tumor, and development of extensive collateral circulation. Surgical resection is the mainstay of treatment, while chemotherapy and/or radiation may serve as therapy adjuncts. This article reviews the pathology, clinical findings, imaging features and management of IVC leiomyosarcoma.

Primary leiomyosarcoma in testis is an uncommon tumor with few cases reported. It generally develop after radiotherapy or long term taking anabolic steroid medication . We report a 53-year-old male patient with primary testis leiomyosarcoma who presented with painless testicular enlargement without any known predisposing factors. Ultrasound revealed a large heterogeneous left testicular solid lesion. Alpha-fetoprotein (AFP) and beta-human chorionic gonadotrophin (beta-HCG) levels in serum were normal. Left radical orchidectomy following with histology assessment established a diagnosis of primary leiomyosarcoma of testis. No data of cancer metastasis was established. The patient didn’t receive any adjuvant therapy. There wasn’t any evidence of recurrence after 1 year follow-up. Leiomyosarcoma must be one of the differential diagnoses of seronegative tumors in testis. The motivation for this paper is the extreme infrequency of the situation and the differential diagnosis by all expansive inguinoscrotal tumors.

e23526 Background: Leiomyosarcoma is a rare malignancy that may occur in bone. This investigation described the characteristics and outcomes of primary leiomyosarcoma of bone (PLB) compared to soft tissue leiomyosarcoma (SLMS). Methods: This study was a retrospective review using the Surveillance, Epidemiology, and End Results (SEER) database from 1975 to 2016. Continuous and categorial variables were described, and Cox regression were used to identify factors of prognostic significance. Results: Seven thousand five-hundred two patients met inclusion criteria. Seventy-four (1%) were PLB and 7428 (99%) were SLMS. On multivariable analysis for high-grade SLMS radiation (neoadjuvant: HR, 0.56; 95% CI, 0.4-0.8; p = 0.003, and adjuvant: HR, 0.75; 95% CI, 0.6-0.9; p = 0.008) and surgery (procedure specific) improved DSS, while age (HR, 1.013; 95% CI, 1.0-1.1; p < 0.001), tumor size greater than or equal to 5 cm (HR, 3.16; 95% CI, 2.3-4.2; p < 0.001), abdomen/pelvis/trunk/thorax tumors (HR, 1.31; 95% CI, 1.1-1.6; p = 0.006), and distant metastases at presentation (HR, 2.6; 95% CI, 2.1-3.3; p < 0.001) negatively influenced DSS. Conclusions: Radiation and surgery positively influence survival in high-grade SLMS while chemotherapy appears to have no benefit. Surgery alone appears to lower the mortality risk in PLB.

Primary vertebral leiomyosarcoma of the spine is extremely rare. Due to its rarity, the number of studies on pathology, treatment and prognosis is limited. Tissue Biopsy and Immunohistochemical staining techniques provide unequivocal diagnosis of leiomyosarcoma. The mainstay of treatment of spinal leiomyosarcoma is surgery, with or without radiotherapy and embolization. The principles of surgery comprise resection of tumor, decompression and stabilisation of the spinal cord and prevention of tumor recurrence. The Tomita total en-bloc resection is the preferred surgical technique. We hereby present a literature review of spinal leiomyosarcoma and a case report of the first case of a 56-year-old lady in Malaysia who was diagnosed with L3 vertebral leiomyosarcoma and lung metastasis.

Background: Breast leiomyosarcoma is a rare subtype of breast sarcoma, constituting 5–10% of the cases. Less than one-third of reported cases are located in the nipple papillary region, making it an extraordinarily rare malignancy, responsible for less than 0.1% of all malignant breast tumors. As no radiological criteria allow definitive diagnosis of papillary leiomyosarcoma, histopathological and immunohistochemical examination of suspicious lesions is required. The prognosis is generally optimistic compared with those for other breast sarcomas.Case presentation: A 54-year-old previously healthy woman reported the appearance of a nodule in her right nipple in the preceding 9 months, associated with pruritus. Physical examination revealed that the lesion had a cystic consistency and measured approximately 3.0 cm. Imaging examinations showed no involvement of the adjacent breast parenchyma or axillary region and the lesion was classified as Breast Imaging-Reporting and Data System (BI-RADS) category IV. The lesion was excised, and the histopathological diagnosis was well-differentiated leiomyosarcoma of the mammary papilla, with immunohistochemical examination showing positivity for smooth-muscle tissue antigens. A staging CT examination was performed, showing no evidence of distant metastasis. After 2 years of follow-up, the patient shows no sign of tumor recurrence.Conclusion: Although rare, leiomyosarcoma must be included in differential diagnosis of breast masses, especially those involving the periareolar region. Due to its good prognosis, reporting on this type of tumor is important to guide therapeutic planning, and to identify and track possible complications of its underdiagnosis.

Objectives Primary leiomyosarcoma in the femoral vein is an extremely rare vascular disorder and is often misdiagnosed. In order to improve the knowledge and treatment of this disease, a case of leiomyosarcoma in the femoral vein was reported. Methods We report the case of a 58-year-old woman with a leiomyosarcoma of the femoral vein and treated successfully by surgical resection. After surgery, no recurrence had been noted follow-up.Subsequently, we reviewed information derived from 31 previously published cases from 1954 to 2019. Results A total of 31 patients diagnosed with femoral vein leiomyosarcoma were presented in previous studies; in all of these patients, 14 (45.2%, 14/31) patients were men. Of these, the median age was 51.6 years old (ranging from 3 to 84). Conclusions Complete resection with adjuvant chemotherapeutic was the main strategy to treat the disease. Prognosis remains poor if metastasis was present, especially pulmonary metastasis.

Leiomyosarcoma is a malignant tumor of smooth muscle origin and commonly diagnosed in the uterus, gastrointestinal tract, and mucous membranes or as metastasis to the skeletal system. Primary leiomyosarcoma of bone is an uncommon type of tumor. Patient with primary bone sarcoma may present with a pathological fracture following a trivial injury, resulting in pain, swelling, and deformity. The pathological fracture needs to be evaluated with a high index of suspicion and proper investigations must be performed prior to fracture fixation. We present a case of primary leiomyosarcoma of humerus, who presented as pathological fracture and underwent inadvertent plating at a peripheral hospital. We treated this patient with wide resection of the tumor and the reconstruction was performed with an implant (Kuntscher’s nail and extra-articular plate) with bone cement spacer. The use of this novel method allowed us to perform implant fixation with small distal humerus bone segment and preserve native elbow joint. In this case report, we aim to highlight the importance of thorough preoperative evaluation and keeping a high index of suspicion while dealing with suspected pathological fractures to differentiate between primary and metastatic disease.

Background: Primary leiomyosarcoma (LMS) of the breast is an extremely rare histologic subtype of malignant breast tumors. While breast sarcomas account for 0.0006% of all breast malignancies, primary leiomyosarcoma constitutes only 5-10% of breast sarcomas themselves. To date, the total number of reported cases in the literature is less than 70 cases with the first case reported in 1968. This is, to the best of our knowledge, the first case reported from Lebanon, with only 2 cases previously reported in the Middle East, specifically in Morocco. Case Report: We report the case of a 48-year-old, white, previously healthy female patient, presenting for a breast mass. She first noticed it three months prior to presentation in her left breast and reports that since then, the lump has been growing and her breast has become more painful and erythematous over time. Physical examination revealed an immobile and tender mass of around 12 cm in diameter. A core biopsy was done followed by a left modified radical mastectomy with lymph node dissection. Pathology of the specimen showed a tumor composed of spindle cells arranged in fascicles invading the dermis, with irregular nuclei and prominent nucleoli. Immunohistochemical staining confirmed the diagnosis of high-grade leiomyosarcoma. Conclusion: We presented this case of leiomyosarcoma with lymph node metastasis to contribute to the scarce literature regarding this disease. Due to the rarity of this diagnosis, not enough data exists regarding treatment and prognosis. Physicians need to review the literature for relevant cases to achieve the best outcome for their patients. Keywords: breast mass, leiomyosarcoma, rare, case report, breast sarcomas, lymph node

Leiomyosarcoma (LMS) of the tongue is an extremely rare malignant mesenchymal tumor. Visceral lesions are the most common and account for about 7% of all soft-tissue sarcomas. The lesion is rare in the head and neck, making up 3%–10% of all leiomyosarcomas. Primary leiomyosarcoma of the tongue is extremely rare leiomyosarcomas andrepresents 5–6% of all soft tissue sarcomas and usually, they arise in the uterus, gastrointestinal tract, and retroperitoneum. The most common sites of head and neck regions are the oral cavity (22%), the sinonasal tract (19%), and the facial skin-subcutis (17%). Smooth muscle tumors in the oral cavity usually arise from the buccal mucosa, the gingiva, and the oral floor,but only a few numbers of cases arise in the tongue.The present case shows the tumor cells arranged in fascicles and interlacing bundles. The nuclei of individual tumor cells are elongated and hyperchromatic with a moderate amount of eosinophilic cytoplasm. The tumor cells were strongly positive for immunoreactivity with Vimentin, and SMA. However, CK, P63, EMA, desmin, CD99, and S100 were negativefrom these immunohistochemical results, the pathological diagnosis was leiomyosarcoma at the tip of the tongue.The tumor cells were strongly positive vimentin and SMA. However, CK, P63, EMA, desmin, S100, and CD99 were negative. From these immunohistochemical results, the pathological diagnosis was leiomyosarcoma, left side of the tongue.

Primary leiomyosarcoma of the inferior vena cava (IVC) is a rare and fatal disease. Imaging is the main diagnostic method. CT and MRI images of IVC leiomyosarcoma have been published, but ultrasonographic features have been scarcely described, especially with contrast-enhancement. We report the case of a patient in whom ultrasonography incidentally revealed a mass in the IVC. Contrast-enhanced ultrasonography showed heterogeneous enhancement in the arterial phase with some irregular non-enhanced areas, and mild clearance in the venous phase. The location and nature of the lesion as shown by ultrasonography were confirmed by CT and MRI.

Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. Clinical presentation and imaging features are non-specific and can mimick the most frequent primary liver tumors namely hepatocellular carcinoma and intrahepatic cholangiocarcinoma. We report here two cases of PHL including one from the portal vein. The literature was searched for studies reporting cases of PHL reported from 2011 and 2019. The two patients were operated with R0 resection. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. Surgery remains the mainstay of the management of PHL. R0 resection is the main prognostic factor. Our literature search identified 16 additional cases from 12 reports. Preoperative diagnosis of PHL needs a high degree of suspicion due to atypical clinical presentation and non-specific imaging features. Surgery is the mainstay of the management of PHL. R0 resection is the main prognostic factor.

Introduction Primary leiomyosarcomas (LMS) of the mediastinum are extremely rare malignant mesenchymal tumors developing from soft tissues or great vessels. We present a case of a primary leiomyosarcoma of the middle mediastinum in which long-term survival was achieved. Case report A 77-year-old man presented to us for examination with an extrapleural sign in his upper mediastinum on chest X-ray. Computed tomography (CT) revealed a well-circumscribed mass in the middle mediastinum. Thoracoscopic resection of the mediastinal tumor and immunohistological findings, which were positive for smooth muscle actin (SMA), HHF-35, vimentin, and desmin confirmed primary leiomyosarcoma. It recurred twice with solid right pulmonary metastases, which were resected. He was followed-up for a total of 9 years and 6 months from the first surgery with no signs of recurrence after his last surgery. Conclusion Surgical resection of both the primary tumor and pulmonary metastases remains the mainstay of treatment of primary leiomyosarcomas.

Less than 50 cases of Primary Leiomyosarcomas of the Penis (PLOP) have been reported despite this PLOPs are the second most common sarcomas of the penis. The usual site for the development of PLOP is the shaft or base of the penis but the malignancy can affect any site of the penis. PLOPs are likely to be superficially located above the tunica albuginea in comparison with deep seated PLOPs. Superficial PLOPs may be asymptomatic or they may present as lumps, ulcers or nodules on the penis. Deep PLOPs may manifest with dysuria and difficulty with micturition when they compress the urethra. Diagnosis of PLOP is based upon the pathology examination features of specimens of the penile lesion that show interlacing fascicles of spindled-cells that have abundant eosinophilic cytoplasm, focal juxta-nuclear vacuoles and blunt ended nuclei and moderate to severe nuclear atypia, rarely atypical multi-nucleated giant cells and focal necrosis tend to be seen. There may be evidence of many mitoses. Immunohistochemistry studies of PLOP tend to show tumor cells that exhibit positive staining for desmin, muscle specific-actin, and alpha smooth muscle actin. Most often superficial PLOPs tend to be treated by wide complete excision of the tumor with tumor free and clear surgical resection margins. Deep seated PLOPs have also been treated by complete excision in various forms depending upon the site and size of the tumor and some of these have included Wide excision, partial penectomy and total penectomy alone and sometimes with adjuvant chemotherapy/radiotherapy. Some cases of deep PLOPs tend to be associated with the development of recurrences and metastases as well as death of the patient and hence deep PLOPs are aggressive tumors. Some people are of the opinion that chemotherapy and radiotherapy are not effective for the adjuvant treatment of PLOP. For this reason it would appear that there is need for the development of new chemotherapy medicaments that would effectively destroy PLOP tumor cells. There is need to undertake a global multi-center treatment trial of immunotherapy, chemotherapy and radiotherapy in order to streamline the treatment of PLOPs especially deep PLOPs.

Context.—Leiomyosarcoma of bone is a rare primary osseous sarcoma characterized by smooth muscle differentiation and absence of malignant osteoid formation. Leiomyosarcoma of bone is diagnostically challenging; this can be improved with greater awareness of this entity and the ability to differentiate it from its histologic mimics. Because of its rarity, only a small number of studies are available in the literature. These factors contribute to our limited understanding of its pathology, prognosis, and treatment.Objective.—To review the clinicopathologic features of leiomyosarcoma of bone and present the most up-to-date understanding of its behavior and management in accordance with the current literature.Data Sources.—Review of pertinent literature on the major features, current knowledge thereof, and the authors' experience in the diagnosis and management of leiomyosarcoma of bone.Conclusions.—Leiomyosarcoma of bone is a rare but well-recognized primary osseous sarcoma that may arise de novo or in association with radiation. Although it is diagnostically challenging, awareness of this rare sarcoma and knowledge of its key histomorphologic and immunohistochemical features allow for accurate diagnosis.

Primary leiomyosarcomas of the colon (PLC) are rare tumors, representing 0.12% of all colon malignancies. We report a 59-year-old man with weight loss, mild anemia, and rectal bleeding. Colonoscopy revealed a 3.2 × 2.6-cm mass at the ileocecal valve. Histopathological examination of the biopsy showed a spindle cell neoplasm that stained positive for smooth muscle actin, caldesmon, and desmin. A diagnosis of PLC was made. Subsequently, a laparoscopic right hemicolectomy was performed, and no local recurrence was noted 6 months after the resection. Given the rarity of PLC, more studies on the clinical features and treatments of this tumor are warranted.

Both MRI and CT scan can determine tumor size and its extension. PLV have a poor prognosis if surgical resection cannot be achieved. We recommend no reconstruction for type II PLV if venous contact is less than 180° or where the implantation base does not exceed one third of the vena cava.

Highlights • 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.

Prostate sarcomas originate from the mesenchymal tissues including smooth muscle, fibromuscular stroma, paraganglia, nerves, and blood vessels. They account for less than 0.1% of all prostate tumors and often present with obstructive symptoms. Patients with leiomyosarcoma of the prostate commonly have poor prognosis and their life expectancy varies between 0% and 60% depending on the stage of the disease at the initial diagnosis. Histopathological examination is essential for definitive diagnosis, and can be performed at an early stage using guided transrectal prostate biopsy. Since prostate specific antigen levels are generally normal, digital rectal examinations are extremely important. We report on a 50-year-old man who was diagnosed with leiomyosarcoma based on the histopathology of his transurethral prostatectomy specimen.

Background Leiomyosarcoma is a rare tumor that could originate from the gastrointestinal tract, uterus, kidney, retroperitoneum, and the soft tissues of the extremities. It accounts for only 1% of all gastrointestinal mesenchymal tumors and primary leiomyosarcoma of the stomach is extremely rare. Most cases reported as leiomyosarcoma of the stomach before the development of KIT immunohistochemistry might be gastrointestinal stromal tumors (GISTs) of the stomach and only 18 cases of leiomyosarcoma of the stomach have been reported since early 2000s. We report here a patient with leiomyosarcoma of the stomach treated by laparoscopic and endoscopic cooperative surgery (LECS). Case presentation A 59-year-old man was referred to our hospital for an early gastric cancer, which was initially treated by endoscopic submucosal dissection. Six months after his initial treatment, a follow-up esophagogastroduodenoscopy revealed a small polypoid lesion at the lesser curvature of the proximal stomach, which appeared to be a hyperplastic polyp. However, one and a half years later, the lesion grew and showed more irregular surface. Biopsy at the time revealed smooth muscle cell proliferation suggestive of leiomyoma. Three years later, the lesion grew even larger and biopsy showed pleomorphic spindle cells. Immunohistochemical study showed positive staining for alpha-smooth muscle actin and desmin, but negative for c-kit and CD34. Ki-67 labeling index was nearly 60%. Based on these findings, the diagnosis of leiomyosarcoma was established. The patient subsequently underwent a partial gastrectomy by LECS. The patient is currently in good condition without recurrence or metastasis at 12 months after surgery. Conclusions Leiomyosarcoma of the stomach is extremely rare. This is the first report of leiomyosarcoma of the stomach treated by LECS. We could also follow its appearance change through endoscopic examination for 3 years.

Background: We report this case to highlight clinical and pathological features of this rare entity. Methods: It is about a 48 year-old woman with an osteolytic lesion of the lower extremity of the left femur. Result: A biopsy of the lesion showed densely cellular malignant mesenchymal proliferation. It was made by bundles of smooth muscle cells with nuclei of moderate to severe atypia. The immunohistochemical study showed an intense and diffuse cytoplasmic positivity of tumor cells with the smooth anti-muscle. Desmin was focal positive. After neoadjuvant chemotherapie, a knee block resection was made. Macroscopically, it was a 10 cm sized tumor located in the distal part of tumor. Histologically tumor showed post chemotherapy effects estimated at 20%. The bone and muscle surgical limits were unscathed. Conclusion: Primary leiomyosarcoma of bone is a rare and a diagnostically challenging tumor entity.

Primary leiomyosarcoma is rare tumor approximately 1/100000 people. It is reported that only 2% of leiomyosarcoma is vascular in origin arising from inferior venacava (IVC). Considering its incidence, it is more common in females and middle segment of IVC is most commonly involved. We report here, case of 37-year-old female presented with right lank pain for one year then after radiological imaging neoplastic mass noted arising from infrahepatic and juxtarenal IVC and ultrasound guidance biopsy was taken that proved leiomyosarcoma of IVC. Surgery was performed with clear resection margins and regular follow-up was performed in our oncology OPD with the necessary imaging and laboratory work.

Objective: Primary leiomyosarcoma of bone (PLB) is a rare type of malignant bone tumor considered as a variant of the spindle cell sarcomas (SCS). The objective of this study was to analyze the clinicopathologic and the prognostic factors of patients with PLB treated at a single institution. Methods: We retrospectively reviewed the records of 22 patients with pathologically confirmed PLB. The data collected were: age, sex, tumor size and location, grade and stage of the disease and histopathologic features. Mean age was 45.5 years (range, 17 to 73 y). Location was: upper limb (27.3%), lower limb (68.2%) and pelvis (4.5%). Patients had high grade in 90.9% of the reports. Margins were negative in 77.3% of the cases. Histological reports describe spindly sarcomatous cells arranged in fascicles with increased vascular formation without osteoid or chondroid matrix production. On immunohistochemistry, smooth muscle actin and desmin where positive in all cases. Results: Mean follow-up time was 73.5 months (range, 5.3 to 331.1 m). We found 22.7% of local recurrence (LR). Distant metastasis (DM) was reported in 9 (40.9%) patients. Lung metastasis was the only DM affected site. Overall survival (OS) rate in 5 years was 59.1%. Predictors of OS were LR and DM. Conclusions: PLB is an extremely rare malignant bone tumor that has a higher rate of DM and similar OS prognosis compared with other bone sarcomas. Level of Evidence IV, Case Series. RESUMO Objetivos: O leiomiossarcoma primário do osso (LPO) é um tumor ósseo maligno raro, considerado uma variante do sarcoma de células fusiformes (SCF). O objetivo deste estudo foi fazer uma análise clínico-patológica e dos fatores de prognóstico dos pacientes diagnosticados com LPO tratados em uma instituição única. Métodos: Foram analisados retrospectivamente os prontuários de 22 pacientes com diagnóstico confirmado de LPO. Os dados coletados foram: idade, sexo, tamanho e localização do tumor, grau histológico, estádio da doença e as características histopatológicas. A média de idade foi 45,5 anos (de 17 a 73 a). A localização foi: membro superior (27,3%), membro inferior (68,2%) e pelve (4,5%). Os pacientes apresentaram alto grau em 90,9% dos relatos. As margens foram livres em 77,3% dos casos. Os relatos histológicos descrevem células sarcomatosas finas e compridas, arranjadas em fascículos, com aumento da vascularização e sem produção de matriz osteoide ou condral. No estudo imuno-histoquímico, a actina do músculo liso e a desmina foram positivas em todos os casos. Resultados: O tempo médio de seguimento foi 73,5 meses (de 5,3 a 331,1 m). Dos pacientes, 22,7% apresentaram recorrência local (RL). Metástase à distância (MD) foi reportada em 9 (40,9%) pacientes. O único local de MD foi o pulmão. O tempo médio de sobrevida em 5 anos foi de 59,1%. Os fatores preditivos de sobrevida global foram: RL e MD. Conclusão: O LPO é um tumor ósseo maligno extremamente raro que tem uma taxa maior de MD, com uma sobrevida global similar aos outros sarcomas ósseos. Nível de Evidencia IV, Série de Casos.

Abstract: The venous invasion from intraabdominal and retroperitoeal tumors (e.g., liver and kidney) is relatively frequent. Primary vascular (venous) tumors are uncommon. The authors review the case of a 67-year-old woman, who was admitted to the hospital in a poor general condition with jaundice. Laboratory findings indicated hepatic and renal insufficiency. Abdominal ultrasound detected a large retroperitoneal mass which was suspected to be of uterine origin. Abdominal CT showed a retroperitoneal mass that invaded the inferior vena cava, but there was no sign of primary intraabdominal or pelvic tumor. Due to the poor general condition of the patient, surgery was not performed. The patient died in some days after admission. Autopsy revealed a primary leiomyosarcoma of the inferior vena cava. Based on the literature data, primary leiomyosarcoma of the inferior vena cava is extremely rare. Leiomyosarcoma of the inferior vena cava can cause various symptoms, depending on the involved segment of the vein. Surgical intervention can be performed in early stages of the disease. In uncertain retroperitoneal tumors involving the inferior vena cava, radiologists should think of the possibility of tumor with vascular origin. Orv Hetil. 2019; 160(19): 756–761.