Primary leiomyosarcoma with osteosarcomatous differentiation of the breast

ABSTRACT Primary leiomyosarcoma with osteosarcomatous differentiation of the breast is an uncommon entity. We present the case of a 37-year-old female who presented with a lump in the breast and pulmonary lesions on PET-CT, for which she underwent a toilet mastectomy. Histopathological examination revealed a tumor with cells arranged in sheets. These tumor cells had moderate eosinophilic cytoplasm, highly pleomorphic, irregular hyperchromatic nuclei, coarse chromatin, and prominent nucleoli. Areas with spindle-cell morphology were noted. Osteoid was seen intermingling with the tumor along with numerous osteoclast-like multinucleate giant cells. A wide panel of Immunohistochemistry was applied, and Desmin, h-Caldesmon, SMA, and Vimentin were positive. The patient died 3 months post-surgery and had a recurrence at the surgical site.

Conjunctival leiomyosarcoma in a patient with xeroderma pigmentosum: 5-year follow-up without recurrence / Leiomiossarcoma da conjuntiva em paciente com xeroderma pigmentoso: acompanhamento de 5 anos sem recidivas

ABSTRACT Conjunctival leiomyosarcoma is a very rare soft tissue malignancy. Herein, we describe a conjunctival leiomyosarcoma case in a patient with another rare disease, xeroderma pigmentosum. The 27-year-old single-eyed xeroderma pigmentosum patient complained of exophytic mass covering the ocular surface in her left eye. A vascular, hemorrhagic mass covering the entire ocular surface of the left eye was identified on the examination. Thus, total mass excision surgery was performed. The pathological diagnosis was compatible with conjunctival leiomyosarcoma. Additional chemotherapy, radiotherapy, or surgery were not accepted by the patient. No recurrence or metastasis was observed during the 5-year follow-up. Both primary conjunctival leiomyosarcoma and xeroderma pigmentosum are very rare diseases. Conjunctival masses in xeroderma pigmentosum patients should be approached carefully, and histopathological examination is warranted. For conjunctival leiomyosarcoma, early diagnosis, localized, unspread disease, and complete resection provide the best prognosis.

RESUMO O leiomiossarcoma da conjuntiva é um tumor ma­ligno de tecidos moles muito raro. Aqui é descrito um caso de leiomiossarcoma da conjuntiva em um paciente com xeroderma pigmentoso, que também é uma doença rara. Um paciente de 27 anos de idade com xeroderma pigmentoso de olho único queixou-se de uma massa exofítica cobrindo a superfície ocular do olho esquerdo. Ao exame, foi observada uma massa vascular hemorrágica cobrindo toda a superfície ocular do olho esquerdo. Foi realizada uma cirurgia de excisão total dessa massa. O diagnóstico patológico foi compatível com leiomiossarcoma da conjuntiva. O paciente recusou qualquer quimioterapia, radioterapia ou cirurgia adicional. Nenhuma recidiva ou metástase foi observada durante o acompanhamento de 5 anos. Tanto o leiomiossarcoma primário da conjuntiva quanto o xeroderma pigmentoso são doenças muito raras. Massas conjuntivais em pacientes com xeroderma pigmentoso devem ser abordadas com cuidado e deve-se realizar um exame histopatológico. Para o leiomiossarcoma conjuntival, o diagnóstico precoce, uma doença localizada e não disseminada e a ressecção completa proporcionam o melhor prognóstico.

Tratamiento quirúrgico del leiomiosarcoma de vena cava inferior

Introducción: La baja incidencia del leiomiosarcoma de la vena cava inferior dificulta tanto la estandarización del diagnóstico como el tratamiento. Objetivo: Presentar el manejo realizado en nuestro centro de un paciente que desarrolló un leiomiosarcoma de vena cava inferior, una patología de baja incidencia y que las posibilidades de realizar un rescate quirúrgico son muy bajas. Resultados: Se presenta el caso de un paciente de 54 años con una tumoración sólida en porción infrarrenal y yuxtarrenal de vena cava inferior de 71 × 76 × 117 mm compatible con leiomiosarcoma de vena cava, con infiltración de uréter derecho que ocasiona uropatía obstructiva derecha grado I-II sin alteración de la función renal, que fue resecada y reconstruida mediante prótesis sin complicaciones. Discusión: Se discute la fisiopatología, el diagnóstico y manejo en relación con el caso presentado. Conclusión: la baja incidencia de estos tumores dificulta tanto la estandarización del diagnóstico como del tratamiento, aunque la cirugía sigue siendo el tratamiento de elección.

Introduction: The low incidence of leiomyosarcoma of the inferior vena cava hinders both the standardization of diagnosis and treatment. Objective: To present the management carried out in our center of a patient who developed an inferior vena cava leiomyosarcoma, a low incidence pathology with uncertain surgical rescue. Results: 54-year-old patient with a solid tumor in the infrarenal and juxtarenal portions of the inferior vena cava of 71 × 76 × 117 mm compatible with leiomyosarcoma of the vena cava, with infiltration of the right ureter that causes right obstructive uropathy grade I-II without kidney function changes; tumour was resected and continuity reconstructed with a prosthesis without complications. Discussion: The pathophysiology, diagnosis and management are commented. Conclusion: the low incidence of these lesions makes it difficult to standardize both diagnosis and treatment, although surgery remains the treatment of choice.

Robot-assisted laparoscopic enucleation in the treatment of leiomyosarcoma of urinary bladder: A case report / 中南大学学报(医学版)

Leiomyosarcoma of urinary bladder (LMS-UB) is a highly malignant mesenchymal tumor, accounting for less than 0.5% of all bladder malignancies, with a predominant clinical presentation of hematuria. Here we report a case of low-grade LMS-UB. A 44-year-old male patient was admitted to the hospital with urodynia for 2 weeks. The patient's pelvis CT showed a mass on the right part of the bladder. For this reason, he was initially diagnosed with bladder cancer. We performed a robot-assisted laparoscopic enucleation of the bladder tumor and low-grade LMS-UB was diagnosed with the histopathological examination. He underwent 5 cycles of adjuvant chemotherapy after surgery. At 19months postoperative follow-up, the patient had no symptoms, recurrence, or distant metastasis. There is no report on the treatment of LMS-UB with minimally invasive enucleation worldwide. This case provides a new comprehensive treatment method of enucleation combined with adjuvant chemotherapy for early low-grade LMS-UB to reduce complications and improve patients' quality of life after surgery.

Leiomiosarcoma de vena cava inferior, reconstrucción mediante interposición de prótesis y anticoagulación

El leiomiosarcoma primario de vena cava es una neoplasia poco frecuente originada a nivel de las células musculares lisas de la túnica media. Representa el 2% de todos los leiomiosarcomas y el 60% de los tumores de vena cava. Presentamos el caso clínico de una paciente de 64 años que consulta por dolor lumbar derecho. La tomografía computada evidencia una gran masa retroperitoneal que engloba la vena cava inferior y contacta con el riñón derecho; el estudio histopatológico de la biopsia certifica un leiomiosarcoma. Se realiza tratamiento quirúrgico mediante resección tumoral con vena cava inferior e interposición de prótesis sintética y nefrectomía derecha. El estudio anatomopatológico de la pieza certifica un leiomiosarcoma de alto grado sin compromiso de la cápsula renal con bordes de resección libres. La resección radical con márgenes negativos actualmente ofrece la mejor tasa de supervivencia. Aún se encuentra en discusión la indicación de la anticoagulación en pacientes con colocación de prótesis.

Primary leiomyosarcoma of the vena cava is a very rare neoplasm that originates from the smooth muscle cells of the tunica media. It represents 2% of all the leiomyosarcomas and 60% of the vena cava tumors. We report a case of 64-year-old patient consulting for a lower right back pain. The computed tomography shows a large retroperitoneal mass which encompasses the inferior vena cava and involves of right kidney. The surgical treatment of tumor resection is performed with right nephrectomy and interposition of Dacron prosthesis. The anatomopathological study shows a high-grade leiomyosarcoma, not compromising the renal capsule, with resection free. Radical resection with negative margins currently offers the best survival rate. Anticoagulation treatment for patients with prosthesis placement is still under discussion.

O leiomiossarcoma primário da veia cava é uma neoplasia rara originada das células musculares lisas da túnica média. Representa 2% de todos os leiomiossarcomas e 60% dos tumores de veia cava. Apresentamos o caso clínico de um doente de 64 anos que consultou por lombalgia direita. A tomografia computadorizada revelou grande massa retroperitoneal que envolvia a veia cava inferior e contatava o rim direito; o estudo citológico da biópsia revelou um leiomiossarcoma. O tratamento cirúrgico foi realizado por excisão tumoral e de veia cava inferior com interposição de prótese sintética e nefrectomia direita. O diagnóstico anatomopatológico foi leiomiossarcoma de alto grau sem envolvimento da cápsula renal com bordas de ressecção livres de patologia. A ressecção radical com margens negativas atualmente oferece a melhor taxa de sobrevida. A indicaçãode anticoagulação em pacientes com colocação de prótese ainda está em discussão.

Ressecção cirúrgica de leiomiossarcoma de veia cava inferior retro-hepática sem reconstrução vascular: relato de caso / Surgical resection of retrohepatic inferior vena cava leiomyosarcoma without vascular reconstruction: case report

Resumo O leiomiossarcoma de veia cava inferior (LVCI) é um raro tumor maligno mesenquimal. Seu tratamento cirúrgico é um desafio, pois necessita combinar margens cirúrgicas livres com reconstrução vascular, usando prótese ou enxerto autólogo, sutura primária ou ligadura simples sem reconstrução da veia. A ligadura é possível graças ao lento crescimento do tumor, permitindo o desenvolvimento de circulação venosa colateral. Apresentamos um caso de LVCI tratado por ressecção radical sem reconstrução vascular. Paciente feminina, 48 anos, com dor abdominal em hipocôndrio direito, astenia e sintomas dispépticos pós-prandiais. Tomografia de abdome revelou massa de formação expansiva localizada no segmento infra-hepático da veia cava inferior com redução da luz do vaso. Na cirurgia, o clampeamento da veia não indicou repercussões hemodinâmicas, sugerindo formação de circulação colateral suficiente. Decidiu-se pela ressecção radical em toda a porção da veia cava retro-hepática e ligadura da veia cava sem reconstrução vascular. A paciente evoluiu sem intercorrências.

Abstract Inferior vena cava leiomyosarcoma (IVCL) is a rare malignant mesenchymal tumor. Surgical treatment is a challenge because it must combine free surgical margins with vascular reconstruction, using prosthetic or autologous grafts, primary suture, or simple ligation without vein reconstruction. The ligation option is possible thanks to the slow growth of the tumor, allowing collateral venous circulation to develop. We present a case of an IVCL treated with radical resection without vascular reconstruction. The patient was a 48-year-old female with abdominal pain in the right upper quadrant, asthenia, and postprandial dyspeptic symptoms. Abdominal tomography revealed a mass with an expansive formation located in the infrahepatic segment of the inferior vena cava and reduced vessel lumen. During surgery, vein clamping did not provoke hemodynamic repercussions, suggesting sufficient collateral circulation formation. It was decided to perform a radical resection of the entire portion of the retrohepatic vena cava and ligate the vena cava without vascular reconstruction. The patient recovered without complications.

Uterine Epithelioid Leiomyosarcoma Arising Within Leiomyoma in A Postmenopausal Woman: A Case Report

@#Uterine leiomyosarcoma is a malignant smooth muscle tumour of the uterus. It is rare and accounting for less than 2% of cases in malignant gynaepathology. To date, only a few reported cases of leiomyosarcoma arising from leiomyoma documented in the literature. We shared an uncommon occurrence of leiomyosarcoma arising from leiomyoma. Presented herein is a case of a ‘rare epithelioid subtype’ of leiomyosarcoma arising from a leiomyoma in a postmenopausal woman. We highlighted the importance of recognizing the possibilities of this event to allow for a timely diagnosis of leiomyosarcoma and to provide insights on management of patients presented with clinically presumed fibroid.

Primary leiomyosarcoma of ovary: A rare malignancy as an incidental finding

Primary leiomyosarcoma (PLMS) of the ovary is extremely rare tumors comprising 1% of ovarian tumors. About 3% of all ovarian malignancies are primary ovarian sarcomas. Only 72 cases have been reported till date. A 57-year-old postmenopausal female presented with abdominal pain for the last 6 months. Ultrasonography and MRI revealed a heterogeneously enhancing solid lobulated mass in the left adnexa abutting the fundus of the uterus and bowel loops. The endometrial cavity was normal. Ovarian markers CA 125, CEA, CA 19.9, and all hematological parameters were within normal limits. LDH was near normal (284 IU/ml). The specimen was sent for frozen section and a diagnosis of malignant spindle cell lesion of ovary was rendered. Histopathology of the ovarian mass revealed intersecting fascicles of tumor cells consisting of ovoid to spindle-shaped cells having a moderate amount of cytoplasm. Bizarre and atypical cells were seen singly dispersed and in small aggregates along with the brisk mitotic activity. Focal areas of necrosis and hemorrhage were also noted. Immunohistochemistry showed strong positivity for smooth muscle actin and Caldesmon while focal positivity for Desmin and Epithelial Membrane Antigen (EMA) was noted. The lesion was negative for Inhibin, Calretinin, and CD 117 and S100. The final diagnosis of primary ovarian Leiomyosarcoma was given based on histopathology and Immunohistochemistry. PLMS of the ovary are rare incidental findings in postmenopausal women. These are highly malignant tumors and carry a poor prognosis. Hence, early diagnosis and surgical treatment with cytoreduction improve patient survival.

Leiomioma submucoso de gran tamaño: una presentación inusual. Reporte de Caso / Large submucosal leiomyoma: an unusual presentation

Se ha postulado que más del 70 % de las mujeres antes de la menopausia son diagnosticadas de leiomiomas; de estas un 25 % presentan sintomatología grave. La ecografía es la modalidad de imagen de primera elección para su estudio. Se tiene registros de progresión de leiomiomas a leiomiosarcomas, que conllevan a un mal pronóstico y son responsables de una cuarta parte de las muertes por neoplasias uterinas, con una sobrevida a 5 años que van desde 46-53 %, lo cual incentiva el diagnóstico precoz y eficaz de masas uterinas. El caso reportado es una presentación inusual de un leiomioma de gran tamaño, que por los hallazgos de imagen se reportó como una neoplasia maligna, su aspecto macroscópico totalmente atípico reforzaba la sospecha imagenológica. Solo se pudo determinar el diagnóstico definitivo mediante estudio histopatológico posterior a histerectomía. Las evaluaciones ginecológicas de rutina deben incluir exámenes especializados de imagen pélvica, la ausencia de presentación típica o síntomas característicos de una patología no debe ser motivo para obviar exámenes complementarios que permitan un diagnóstico oportuno y tratamiento eficiente de leiomiomas, incluso en casos de presentaciones inusuales y desafiantes para el diagnóstico, como el que se ha reportado en esta obra.

It has been postulated that more than 70% of premenopausal women are diagnosed with leiomyomas; Of these, 25% have severe symptoms. Ultrasound is the imaging modality of choice for its study. There are reports of progression from leiomyomas to leiomyosarcomas, which leads to a poor prognosis, and is responsible for a quarter of deaths from uterine neoplasms, with a 5-year survival ranging from 46-53%; this should encourage early and effective diagnosis of uterine masses. The reported case is an unusual presentation of a large leiomyoma, which due to its imaging characteristics, was reported as a malignant neoplasm. Its completely atypical macroscopic appearance reinforced the imaging diagnosis suspicion. The definitive diagnosis could only be determined by histopathological study after hysterectomy. Routine gynecological evaluations should include specialized pelvic imaging exams, the absence of a typical presentation or characteristic symptoms of a pathology should not be a reason to obviate ancillary testing that would allow a timely diagnosis and effective treatment of leiomyomas, even in unusual and challenging presentations, such as with the patient in this case report.

Inferior vena cava leiomyosarcoma with liver metastasis at presentation in a young male: A challenging diagnostic quandary

Leiomyosarcomas of vascular origin are very rare tumors, predominantly affecting the inferior vena cava (IVC). Although vascular leiomyosarcomas are slow-growing, their non-specific and late presentation results in delayed diagnosis which portends a very poor prognosis. Here we report a case of a 24-year-old man who presented with abdominal pain since 15 days and was found to have unresectable metastatic leiomyosarcoma of the inferior vena cava at initial diagnosis.

Primary Leiomyosarcoma of Liver � Case Report of a Rare Cancer

Primary Hepatic Leiomyosarcoma are very rare Tumours with only 30 cases reported Worldwide1. Patient complains of nonspecific symptoms and often presents with Enlarged Liver. Diagnosis is usually delayed and patients have poor prognosis2. 35-year-old woman presented to Outpatient Department (OPD) complaining of dull aching pain and mass in upper part of abdomen for 6 months. On palpation liver was enlarged and lower border extended up to umbilicus. Ultrasonography of abdomen showed Liver enlarged 23 cm with multiple hypoechoic nodules noted in both lobes of liver. Triphasic Contrast-Enhanced Computed Tomography (CECT) abdomen multiple variable sized Peripherally Enhanced Hypodense Nodules in both lobes of Liver suggestive of Multifocal Hepatocellular Carcinoma (HCC)/ Metastasis. Tumour markers S alpha fetoprotein, S. CA 19-9, S. CEA was within normal limits. CECT Chest was normal. Positron Emission Tomography and Computed Tomography (PET-CT) was done, which showed Liver enlarged 24.5cm with FDG avid multiple nodules in both lobes of Liver and multiple enlarged FDG avid periportal, Portocaval and Peripancreatic Lymph Nodes. Rest of body organs were normal. Biopsy from the Liver showed Mesenchymal Tumour composed of spindle cells arranged in fascicular growth pattern, nucleus cigar shaped with atypia and mitotic figures seen. On Immunohistochemistry (IHC) Tumour was positive markers were Smooth Muscle Actin (SMA), VM, Desmin and H-caldestron and negative for CK, S-100, SOX-10, CD-117 and Dog-1. From above work up a diagnosis of primary Leiomyosarcoma of Liver was made. Palliative Chemotherapy was offered to the patient.

Primary leiomyosarcoma of the thyroid gland

Sarcoma is a rare tumor of the thyroid gland, primary thyroid leiomyosarcoma (LMS) being even rarer. We present a case of LMS of the thyroid in a middle-aged female. Histopathologic examination in conjunction with immunohistochemistry helped to clinch the diagnosis. Knowledge of this entity is important to distinguish it from anaplastic thyroid carcinoma (ATC) and other sarcomas arising in the thyroid and adjacent soft tissue. The prognosis of thyroid LMS is dismal with an extremely poor survival rate.

Primary ovarian leiomyosarcoma in a 72-year-old nulligravid and the 2022 provisional diagnostic criteria for primary ovarian leiomyosarcoma / Philippine Journal of Obstetrics and Gynecology

@#Primary ovarian leiomyosarcoma (POLMS) is an exceedingly rare neoplasm accounting for only 0.1% of all ovarian malignancies and most commonly occurring in postmenopausal women. Prognosis is poor with only a 20% 5-year survival rate. Surgery remains to be its mainstay treatment. Discussed here is a 72-year-old nulligravid with hypogastric pain. Transrectal ultrasound showed a right ovarian new growth, probably malignant on International Ovarian Tumor Analysis (IOTA) simple rules, with a 79.2% risk of malignancy by IOTA ADNEX and an unremarkable uterus. Other workups were normal. She underwent primary cytoreductive surgery. Histopathologic diagnosis was ovarian leiomyosarcoma with positive immunohistochemical staining for desmin, S-100 protein, smooth muscle actin, and epithelial membrane antigen. She refused adjuvant chemotherapy postoperatively. The latest published literature on POLMS was also reviewed to develop the provisional criteria for its prompt diagnosis, thereby decreasing the heterogeneity of the diagnostic approach as well as supporting future researches on manifestations, clinical courses, and therapeutic plans.

Primary ovarian leiomyosarcoma in a postmenopausal woman diagnosed using immunohistochemistry / Philippine Journal of Obstetrics and Gynecology

@#Primary ovarian leiomyosarcoma is a very rare tumor which is most commonly seen in postmenopausal women. Primary ovarian leiomyosarcoma has a very poor prognosis, with less than 20% of patients being alive at 5 years. The case is a 51‑year‑old female who presented with a lower abdominal mass secondary to an ovarian new growth. An exploratory laparotomy, peritoneal fluid cytology, unilateral salpingo‑oophorectomy with malignant frozen section of affected ovary, which revealed malignant tumor, proceeded with surgical staging total abdominal hysterectomy contralateral salpingo‑oophorectomy, then proceeded to complete surgical staging with infracolic omentectomy, Jackson‑Pratt drain insertion was performed. Microscopic and immunohistochemical findings established the diagnosis of primary ovarian leiomyosarcoma. Surgery is the cornerstone of treatment, while the role of chemotherapy and radiotherapy is still not clear because substantial data are lacking. The prognosis of primary pure ovarian leiomyosarcomas is extremely poor and there is no established treatment modality for this rare type of tumor.

Leiomiossarcoma de veia cava inferior como etiologia para dor abdominal crônica - um relato de caso / Leiomyosarcoma of the inferior vena cava as etiology of chronic abdominal pains - a case report

Resumo Os leiomiossarcomas de veia cava inferior são tumores raros, que representam menos de 0,7% de todos os leiomiossarcomas retroperitoneais. Eles são mais comuns em mulheres e causam quadros inespecíficos de dor abdominal crônica. Neste relato, apresentamos um caso de paciente do sexo feminino, de 53 anos de idade, com queixa de dor abdominal crônica periumbilical inespecífica com evolução há 8 meses, diagnosticada com leiomiossarcoma de veia cava inferior por angiotomografia computadorizada. A paciente foi tratada com ressecção completa do tumor e reconstrução da veia cava inferior, com interposição de prótese de dácron. O tratamento considerado padrão-ouro consiste na excisão cirúrgica completa, visto que esses tumores são resistentes a quimioterapia e radioterapia. O prognóstico desses pacientes está intimamente relacionado com a precocidade do diagnóstico, e, por isso, é de grande relevância o conhecimento dessa doença como diagnóstico diferencial de dor abdominal crônica e inespecífica por cirurgiões vasculares e cirurgiões gerais.

Abstract Inferior vena cava leiomyosarcomas are rare tumors that account for less than 0.7% of all retroperitoneal leiomyosarcomas. They are more common in women and cause nonspecific chronic abdominal pain. In this report, we present the case of a 53-year-old female patient complaining of chronic nonspecific periumbilical abdominal pain with initial onset 8 months previously who was diagnosed with inferior vena cava leiomyosarcoma by computed tomography angiography. The patient was treated with complete resection of the tumor and reconstruction of the inferior vena cava with interposition of a Dacron prosthetic graft. The treatment considered the gold standard consists of complete surgical excision, because these tumors are resistant to chemotherapy and radiotherapy. The prognosis of these patients is closely related to early diagnosis. Therefore, it is very important that vascular and general surgeons know that this disease is a possible differential diagnosis of chronic abdominal pains.

Metastasizing leiomyoma during pregnancy

Uterine leiomyoma is a benign tumor of myometrial tissue which affects women of reproductive age. Its prevalence increases with age and has a peak incidence at the age of forty. The term "metastasizing leiomyoma" refers to a tumor of dense connective tissue and smooth myometrial muscle cells located outside the uterus. This group of tumors can metastasize to different organs, the lung being its main focus. We present the case report of a 33-year-old female gravida 3, para 1, abortus 1, at 11 weeks of pregnancy, with pelvic masses. The diagnosis was metastasizing leiomyoma during pregnancy.

Clinical features and operative outcomes of retroperitoneal leiomyosarcoma / 中华普通外科杂志

Objective:To analyze the operative outcomes and postoperative pathological features of retroperitoneal leiomyosarcoma(RPLMS) undergoing surgeries.Methods:Medical records of RPLMS patients admitted to Peking University International Hospital from Jan 2015 through Dec 2020 were retrospectively reviewed.Results:Ninety-seven patients undergoing resectional surgeries were included in the study. Of whom, 49 cases were primary RPLMS. Others were recurrent sarcomas or sarcomas with incomplete resection in the first surgical intentions. The most frequent symptoms were abdominal pain and distention (30 cases) as well as lower back pain (23 cases). All patients underwent resectional surgeries with a R 0/R 1 rate of 84.5%. Sixty-four cases received extended surgeries with combined organs resection. External iliac artery resection with reconstruction were performed on 2 cases. And 19 patients underwent partial IVC resection in combination of sarcoma resection. The general postoperative morbidity was 26.8%, including 4 intestinal fistulas, 1 pancreatic fistula, 1 vesicovaginal fistula, 1 urinary fistula, 1 biliary fistula, 2 abdominal major bleeding, 7 IVC thrombosis, 3 gastroplegiaetc. One patient deceased within post-operative 30 d due to massive bleeding.Pathology found that spindle and pleomorphic cell types were most common subtypes of RPLMS. Conclusions:Surgery remains the mainstay in the treatment of RPLMS which often presents with atypical symptoms. Extended surgeries combining with multiple organ and major vascular resections could be only suggested in experienced sarcoma centers due to high risk of severe postoperative complications.

Leiomiosarcoma primario de hueso en fémur distal metastásico a clavícula: reporte de un caso / Primary leiomyosarcoma of bone in the distal femur with clavicle metastasis: a case report

El leiomiosarcoma (LMS) es un tipo de tumor de células fusiformes de muy baja incidencia, que tiene un comportamiento agresivo, con alta tasa de mortalidad, por lo que el manejo debe ser quirúrgico, con una resección amplia de la lesión. No está claro el papel de la radio ni de la quimioterapia en su manejo. Presentamos a una paciente de 28 años que consultó por dolor de 2 meses en la rodilla derecha. Radiográficamente, se caracterizó como una lesión osteolítica pura en el fémur distal. La resonancia nuclear magnética (RNM) contrastada mostró áreas hipervasculares dentro del tumor. La gammagrafía mostró un marcado aumento en la captación de radiotrazadores. Se tomó una biopsia, con un informe de patología de LMS óseo bien diferenciado. Se trató con 3 ciclos de quimioterapia neoadyuvante preoperatoria con ifosfamida 1.000 mg/m2 en los días 1 a 3, además de doxorrubicina 70 mg/m2 , y resección quirúrgica de la lesión y salvamento de la extremidad con endoprótesis de rodilla. Una vez que se resecó la lesión, la paciente recibió quimioterapia adyuvante con 4 ciclos de gencitabina 1.000 mg/m2 entre los días 1 y 8, y doxetacel 70 mg/m2 el día 1. Durante los dos meses de seguimiento, la paciente presenóa una fractura en el tercio medio de la clavícula, compatible con una lesión patológica en radiografías y tomografía por emisión de positrones (TEP). La biopsia reveló una lesión metastásica de LMS óseo que fue tratada mediante resección quirúrgica de la clavícula. Este es un caso único, dado que, durante el seguimiento, recibió tratamiento adyuvante con quimioterapia y se evaluó con una TEP, con una evolución clínica satisfactoria y sin evidencia de nuevas lesiones

Leiomyosarcoma (LMS) is a type of spindle-cell tumor of very low incidence that tumor has an aggressive behavior, with high mortality rates; therefore, its management must be surgical, with a wide resection of the lesion. The role of radio and chemotherapy in its management is not clear. We present the case of a 28-year-old female patient who consulted for pain lasting 2 months in the right knee. Radiographically, it was characterized as a pure osteolytic lesion in the distal femur. Contrast magnetic resonance imaging (MRI) showed hypervascular areas within the tumor. The scintigraphy showed a marked increase in radiotracer uptake. A biopsy was taken, with a pathology report of well-differentiated osseous LMS. It was treated with 3 cycles of preoperative neoadjuvant chemotherapy with ifosfamide 1,000 mg/m2 in the first 3 days, as well as doxorubicin 70 mg/m2 , and surgical resection of the lesion and limb salvage with knee endoprosthesis. Once the lesion was resected, the patient underwent adjuvant chemotherapy, with 4 cycles of gencitabine 1,000 mg/m2 between days 1 and 8, and doxetacel 70 mg/m2 on day 1. During the 2-month follow-up, the patient presented a fracture in the middle third of the clavicle, which was compatible with a pathological lesion on radiographs and positron-emission tomography (PET) scans. The biopsy showed a metastatic lesion of bone LMS, which was treated by surgical resection of the clavicle. This is a unique case, given that, during the follow-up, the patient underwent adjuvant treatment with chemotherapy, and was evaluated with a PET scan, with a satisfactory clinical evolution and no evidence of new lesions.

Leiomiosarcoma retroperitoneal con resección de vena cava / Retroperitoneal leiomyosarcoma with resection of the vena cava

RESUMEN El leiomiosarcoma vascular es un tumor maligno de baja incidencia cuya localización más frecuente es la vena vava inferior (VCI). Se presenta habitualmente en la 6a década de la vida. Según su localización se describen 3 tipos de acuerdo con su relación con las venas suprahepáticas y renales. Los cuadros clínicos de presentación son inespecíficos, ya que suelen debutar como hallazgos o con síndromes de congestión venosa pélvica/miembros inferiores. Su tratamiento quirúrgico radical requiere un equipo multidisciplinario entrenado en cirugía retrope ritoneal y vascular.

ABSTRACT Vascular leiomyosarcomas are rare tumors and are usually localized in the inferior vena cava (IVC). They usually occur in the 6th decade of life. They are classified into 3 groups according to the relation with the hepatic and renal veins. The clinical presentation is unspecific, ranging from an incidental finding to symptoms of venous pelvis congestion of lower extremity edema. Radical resection is the treatment of choice and requires multidisciplinary team trained in retroperitoneal and vascular surgery.