Foreskin leiomyosarcoma: a case report. / [Leiomiosarcoma de prepucio: reporte de un caso]

Introduction: Primary skin leiomyosarcomas are infrequent neoplasms. They correspond to 2-3% of skin sarcomas and are most frequently located on the lower extremities, trunk and genitals. Methods: We present a case of a 73-year-old man with a 4-month evolution of foreskin leiomyosarcoma. The lesion was biopsied for histopathological study with HE and immunohistochemistry with smooth muscle actin, specific muscle actin, CD34, p63 and S-100 (-). Results: We observed a leiomyosarcoma of high histological grade and mitotic count. It was positive by immunohistochemistry for smooth muscle actin, while the other markers were negative. Surgical limits were compromised so a reoperation with wide margins of healthy tissue was necessary. Conclusion: The skin lesions should be removed all, without exception, since they can be neoplasms of variable biological behavior. The histological study must be complemented with immunohistochemistry to differentiate them from other neoplasms. For the prognosis, the histological grade, size, location and the possibility of resection with wide margins must be taken into account.

Introducción: Los leiomiosarcomas primarios de piel son neoplasias infrecuentes. Corresponden al 2-3 % de los sarcomas cutáneos y se localizan con mayor frecuencia en las extremidades inferiores, tronco y genitales. Método: Presentamos un caso de un varón de 73 años con un leiomiosarcoma en prepucio de 4 meses de evolución. Se le practicó biopsia excisional de la lesión para estudio histopatológico con HE e inmunohistoquímica con actina de músculo liso, actina muscular específica, CD34, p63 y S-100 (-). Resultados: Observamos un leiomiosarcoma de alto grado histológico y recuento mitótico. Presentó positividad por inmunohistoquímica para actina de músculo liso, en tanto que los otros marcadores fueron negativos. Los límites quirúrgicos estuvieron comprometidos por lo que fue necesaria una reintervención con amplios márgenes de tejido sano. Conclusión: Las lesiones de piel deben extirparse todas, sin excepción, ya que pueden tratarse de neoplasias de conducta biológica variable. El estudio histológico debe complementarse con inmunohistoquímica para diferenciarlas de otras neoplasias. Para el pronóstico se debe tener en cuenta el grado histológico, el tamaño, la localización y la posibilidad de resección con amplios márgenes.

Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review.

BACKGROUND: Primary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. METHODS: A series of 6 cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. RESULTS: Optimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavorraphy was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3 ± 9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9 ± 2 months. CONCLUSION: The diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease.

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

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

Surgical treatment of vena cava leiomyosarcomas: Series of cases in a referral center and literature review. / Tratamiento quirúrgico de los leiomiosarcomas de vena cava. Serie de casos en un hospital de tercer nivel y revisión de la literatura.

INTRODUCTION: Primary tumors of the inferior vena cava are rare tumors of mesenchymal origin. They arise from the smooth muscles of the vena cava wall. Due to its low prevalence, there are few definitive data on its treatment and prognosis. Its treatment is based on general oncological principles. METHODS: A series of six cases operated from 2010 to 2020 were analyzed. Different parameters related to the demographic characteristics, the tumor, the treatment received, and the results obtained in survival and morbidity were analyzed. In addition, a bibliographical review of the currently available evidence was carried out. RESULTS: Optimal surgical resection was accomplished in all patients with R0 in 4/6 and R1 in 2/6. The greatest morbidity occurred in a patient who died in the intraoperative period. Cavography was performed in one patient and cavoplasty in 5/6 using cryopreserved graft in 3/6 and prothesis in 2/6. The 50% were still alive at the end of the follow-up (with a mean follow-up of 10.7 months). The mean survival was 11.3±9.07 months. 3/6 patients presented hematogenous recurrences with a disease-free interval of 9±2 months. CONCLUSION: The diagnosis and treatment of inferior vena cava leiomyosarcoma is still a challenge. Due to its low prevalence, it will be difficult to establish a totally standardized treatment and its approach is recommended in specialized centers. On the other hand, a multicentric study should be made to collect the most cases as possible in order to advance in the understanding of the approach to this disease.

Uterine pleomorphic leiomyosarcoma with osteoclastic giant cells: Case report with peritoneal washing cytology and cell block study.

Uterine leiomyosarcoma (LMS) with osteoclastic giant cells (OGCs) is extremely rare. However, its morphological appearance and aggressive behavior may have resulted in its being diagnosed as so-called giant cell malignant fibrous histiocytoma (MFH) in the past. Effusions are not uncommon in LMS and may be indicative of an unfavorable prognosis. We report a case with the cytological appearance of a uterine LMS with OGCs metastatic to lower pelvic peritoneum. The pelvic washing specimen consisted of three-dimensional aggregates of atypical cells. The cytohistologic and immunohistochemical study obtained from the cell block and the tumor mass showed overlapping features such as bizarre pleomorphic spindle cells containing numerous evenly dispersed OGCs. The malignant tumor cells showed extensive positivity for desmin, h-caldesmon and multifocal positivity for smooth muscle actin (SMA) whereas OGCs stained with CD68. We stress the usefulness of performing cell block and subsequent immunohistochemistry in order to make an accurate cytohistologic correlation.

Leiomyosarcoma and Pleomorphic Dermal Sarcoma: Guidelines for Diagnosis and Treatment. / Leiomiosarcoma y sarcoma pleomórfico dérmico: directrices para el diagnóstico y tratamiento.

There are 3 types of leiomyosarcoma of the skin: dermal, subcutaneous, and metastatic cutaneous. Dermal leiomyosarcoma arises from smooth muscle fibers in arrector pili muscles, genital dartos muscles, and the nipple-areola complex. It is an intermediate-grade tumor associated with a tendency for local recurrence (24%) and low metastatic potential (4%). Subcutaneous leiomyosarcoma originates from smooth muscle in blood vessel walls and has higher rates of local recurrence (37%) and metastasis (43%). Plemorphic dermal sarcoma typically affects elderly patients and arises in sun-exposed areas (e.g., the scalp). Its histologic and immunohistochemical characteristics are similar to those of atypical fibroxanthoma, but it is more aggressive (metastasis rate of 10-20%). Histologically, it can be distinguished from atypical fibroxanthoma by the observation of subcutaneous tissue invasion, perineural invasion, and foci of necrosis.

Endoscopic ultrasound-guided fine-needle aspiration cytology in the diagnosis of leiomyomas of the gastrointestinal tract.

We investigated the efficiency and accuracy of endoscopic ultrasound-guided fine-needle aspiration cytology (EUS-FNAC) in the diagnosis of gastrointestinal leiomyoma (GIL). Between January 2009 and May 2018 we performed 795 EUS-FNAC studies of lesions of the gastrointestinal (GI) tract for various clinical indications. A diagnosis of GIL by cytological and cell block study was made in 14 patients (57.1% males, mean age 53.6 years, range 22-84 years). 7 tumors (50%) were detected incidentally. The lesions ranged in size from 2 to 10cm (mean size 4.4cm). The location of the tumors was: esophagus 7 (50%), stomach 6 (42.9%) and rectum 1(7.1%). The mean size of the symptomatic tumors was 5.2cm (range 3-10cm). The follow-up of the 14 patients varied from 1 to 108 months (median 39.5 months), during which no recurrence or evidence of lesion progression was observed. Imaging alone was not sufficient for an accurate diagnosis to be made. The pathological diagnosis was based on a combination of cytological, histopathological, and immunohistochemical features. The intracytoplasmic eosinophilic globule is a useful marker of paucicellular GIL differentiating it from gastrointestinal stromal tumor and leiomyosarcoma. EUS-FNAC is a reliable, accurate, and safe method for the diagnosis of GIL.

Tumor de músculo liso uterino de potencial maligno incierto (STUMP): reporte de caso y revisión de la literatura / Uterine smooth muscle tumor of uncertain malignant potential (STUMP): case report and literature review

Myometrial smooth muscle neoplasms are the most common gynecologic tumors with a prevalence of 70-80% at age 50. Among women undergoing hysterectomy or myomectomy for a suspected diagnosis of leiomyoma, 0.01% receive a diagnosis of STUMP. Clinically, the average age of presentation is between 41-48 years. Tumors ranging from 3 to 30 cm have been described. Signs and symptoms are similar to leiomyomas, such as abnormal uterine bleeding, anemia, dysmenorrhea, pelvic pain, pelvic mass, infertility or other types of pain secondary to compression of adjacent organs. Due to the limited literature available, there is no definite management consensus, and treatment and follow-up options are limited to observational studies. The standard treatment is total hysterectomy with or without bilateral salpingo-oophorectomy; if fertility has already been completed, there is no role for adjuvant hormonal therapy or chemotherapy. The overall 5-year survival is 92-100%. A case of a 31-year-old woman with STUMP is presented.

[Meningeal metastasis of uterine leiomyosarcoma. Case report and literature review]. / Metástasis meníngea de leiomiosarcoma uterino. Reporte de caso y revisión de literatura.

Brain metastases are the most commonly seen intracranial lesions in adults. What is more, meningiomas are the most common primary intracranial tumours after gliomas and their imaging characteristics are well known in both CT and MRI scans. However, there are lesions that can mimic meningiomas in imaging studies, including metastases of extracranial tumours, confronting us with a diagnostic and therapeutic challenge. We present the case of a patient with meningeal metastasis of a uterine leiomyosarcoma that was not known at the time of the surgical intervention.

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.

Clinical and Histopathologic Findings of Cutaneous Leiomyosarcoma: Correlation with Prognosis in 12 Patients. / Leiomiosarcoma cutáneo: características clínicas, histopatológicas y correlación pronóstica en 12 pacientes.

INTRODUCTION: Cutaneous leiomyosarcoma is a malignant neoplasm derived from smooth muscle cells. Its low incidence hampers the development of specific protocols for diagnosis and treatment. OBJECTIVES: To describe the clinical and histopathologic characteristics of a series of primary and secondary cutaneous leiomyosarcomas and to determine how these characteristics correlate with prognosis. MATERIAL AND METHODS: We performed an observational, descriptive, retrospective study based on 17 cutaneous leiomyosarcomas in 12 patients diagnosed between January 1, 2000 and December 31, 2015. We recorded demographic data, clinical and histopathologic characteristics, outcome, and response to treatment. RESULTS: We included 5 men and 7 women, all aged more than 50 years at diagnosis. There were 4 cutaneous leiomyosarcomas (23%) in 4 patients, 2 subcutaneous leiomyosarcomas (11.5%) in 2 patients, and 11 skin metastases of leiomyosarcoma (65%) in 6 patients. The most frequently affected sites were the scalp (41%), lower limbs (17%), and trunk (17%). During follow-up, 50% of the cutaneous leiomyosarcomas recurred, 50% of the subcutaneous leiomyosarcomas presented distant metastases, and 83% of the patients with skin metastases of leiomyosarcoma died of their disease. LIMITATIONS: Ours was a retrospective review of a small case series at a single center. CONCLUSIONS: Cutaneous leiomyosarcoma is an uncommon malignant neoplasm. Our approach to diagnosis and therapy must take into account the marked heterogeneity in the prognosis of the various subtypes.

Leiomiosarcoma de vena cava inferior, reconstrucción mediante interposición de prótesis y anticoagulación / Leiomyosarcoma of the inferior vena cava, reconstruction with interposition of prosthesis and anticoagulation / Leiomiossarcoma da veia cava inferior, reconstrução por interposição de prótese e anticoagulação

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.

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.

Uterine leiomyosarcoma metastatic to thyroid shown by 18F-FDG PET/CT imaging.

About one third of focal thyroid uptakes in a fluorodeoxyglucose (FDG) positron emission tomography/computerized tomography (PET/CT) study are malignant, the most frequent histological type being papillary carcinoma. Metastases to the thyroid account for approximately 7.5% of thyroid malignancies and come mainly from kidney, lung, head and neck, and breast cancers. We report the case of a 64-year-old woman presenting a fast growing thyroid nodule whose primitive or metastatic origin was not obvious, for which 18F-FDG PET/CT helped in the diagnostic process and in the later management of the patient. Histopathologic findings finally revealed a metastasis of uterine leiomyosarcoma.

[Leiomyosarcoma of the inferior vena cava. Case report and literature review]. / Leiomiosarcoma de vena cava inferior. Caso clínico y revisión bibliográfica.

BACKGROUND: Large vessel sarcomas are rare tumours. Leiomyosarcoma of the inferior vena cava is the most common. About 300 cases have been reported in the literature. They tend to be large, and not develop metastasis. The prognosis of these tumours is poor. CLINICAL CASE: An 81 year-old woman who complained of pain in the right flank, with no other symptoms. Abdominal computed tomography showed a large retroperitoneal mass, which affected the inferior vena cava, with signs of thrombosis inside. It also encompassed the right renal vein and the right kidney. Excision of the tumour was performed in block, performing an autologous saphenous vein bypass between left the renal vein and proximal segment of inferior vena cava. DISCUSSION: Leiomyosarcomas of the inferior vena cava are classified according to their relationship with adjacent structures. The clinical signs and symptoms are generally non-specific. Diagnosis is made using computed tomography or magnetic resonance imaging, and biopsy of the retroperitoneal mass. Surgery is the only treatment capable of providing prolonged survival. The surgical management is determined by: the level of involvement, the extension, and the presence or absence of collateral veins. The role of adjuvant therapy is controversial. CONCLUSIONS: Inferior vena cava leiomyosarcomas remain a challenge for surgeons. At present, radical resection with negative margins, offers the highest survival rate. The best results are obtained with a multidisciplinary approach by experienced teams in the management of these tumours.

Primary orbital leiomyosarcoma / Leiomiosarcoma primario de órbita

Abstract Leiomyosarcoma is an aggressive mesenchymal malignant tumor which rarely presents in head and neck structures. There are few cases published in the literature, and clinical suspicion, diagnosis and early treatment are considered to be essential for dealing with the adverse outcomes derived from its poor prognosis. We report the first case of primary orbital leiomyosarcoma in Colombia, diagnosed in an older adult who consulted due to eight months of progressive growth of the left frontotemporal region along with dysarthria, vision loss and ipsilateral painful proptosis. Imaging studies revealed a large osteolytic lesion involving the orbit and central nervous system. An incisional biopsy was performed, reporting high-grade leiomyosarcoma. Once metastasis was ruled out, complete excision of the lesion was ordered, along with radiation therapy. After 10 months of close follow up, the patient had progressed well, with no local recurrences or distant metastases. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1937).

Resumen El leiomiosarcoma es un tumor maligno de origen mesenquimal de naturaleza agresiva que infrecuentemente se presenta en estructuras de cabeza y cuello. Son pocos los casos publicados en la literatura, siendo considerado su sospecha, diagnóstico y tratamiento temprano fundamentales para enfrentar los desenlaces adversos derivados de su mal pronóstico. Se reporta el primer caso en Colombia de leiomiosarcoma primario de órbita, diagnosticado en un paciente adulto mayor quien consultó por ocho meses de aumento progresivo de la zona frontotemporal izquierda acompañado de disartria, pérdida de visión y proptosis dolorosa ipsilateral. Los estudios imagenológicos eviden ciaron extensa lesión osteolítica con compromiso orbitario y de sistema nervioso central. Se realizó biopsia incisional, con reporte de leiomiosarcoma de alto grado. Tras descartar metástasis, se indicó exéresis completa de la lesión e inicio de radioterapia. A los diez meses de estrecho seguimiento, el paciente presentó una evolución favorable sin recurrencias locales ni metástasis a distancia. (Acta Med Colomb 2021; 46. DOI: https://doi.org/10.36104/amc.2021.1937).

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.

Leiomiosarcoma primario de cornete nasal inferior: reporte de caso clínico y revisión de la literatura / Primary leiomyosarcoma of inferior nasal turbinate: clinical case report and literature review

Los leiomiosarcomas de la cavidad nasal y senos paranasales son neoplasias malignas de baja frecuencia, localmente agresivas. Presentan una alta tendencia a la recurrencia de aproximadamente 55% en nariz y senos paranasales. Están conformados por células musculares lisas. Según diversos autores, aproximadamente el 50% de los pacientes mueren antes del primer año y la supervivencia a los 5 años es del 20%. Anteriormente se pensaba que no poseían potencial metastásico, sin embargo, en series actuales se ha visto que presentan un alto poder metastásico de hasta el 50%. Las metástasis se presentan de forma tardía. El tratamiento recomendado consiste en la resección radical del tumor primario con un amplio margen de tejido normal y la radioterapia es de uso controversial en el manejo.

Leiomyosarcoma of the nasal cavity and paranasal sinuses are malignant, low frequency, locally aggressive neoplasm. They present a high tendency to recurrence of approximately 55% in the nose and paranasal sinuses. They are made up of smooth muscle cells. According to different authors, approximately 50% of patients die before the first year and survival at 5 years is 20%. Previously it was thought that they did not possess metastatic potential, however in current series it has been seen that they have a high metastatic power of up to 50%. Metastases present late. The recommended treatment consists of radical resection of the primary tumor with a wide margin of normal tissue.

Sarcoma gástrico primario: reporte de caso y revisión de la literatura / Primary gastric sarcoma: Case report and literature review

Resumen El cáncer gástrico, patología neoplásica de innegable importancia, corresponde en el 90 % de los casos a un adenocarcinoma. Dentro del 10 % restante, los linfomas y los tumores estromales gastrointestinales (Gastrointestinal Stromal Tumor, GIST) constituyen la mayoría. Sin embargo, los sarcomas no GIST siguen siendo un diagnóstico diferencial posible para tener en cuenta y configuran una patología neoplásica de tratamiento fundamentalmente quirúrgico. En particular, el leiomiosarcoma representa menos del 1 % de los tumores malignos del estómago y la literatura disponible al respecto consiste en reportes de caso o serie de casos. Por su rareza, presentamos este caso clínico y revisamos la literatura relacionada.

Abstract Gastric cancer, a neoplastic pathology of undeniable importance, accounts for 90% of cases to adenocarcinoma. GIST lymphomas and gastrointestinal stromal tumors are the majority of the other 10%. However, non-GIST sarcomas remain a possible differential diagnosis to keep in mind and constitute a neoplastic pathology whose treatment is fundamentally surgical. Leiomyosarcoma represents less than 1% of malignant stomach tumors, and the available literature consists of case reports or case series. Because of its rarity, we present this clinical case and review the literature.