Tumor nasal de músculo liso de comportamiento maligno incierto: informe de un caso / Nasal smooth muscle tumor of uncertain malignant potential: A case report

Los tumores de músculo liso que no pueden ser clasificados según su histología como leiomiomas o leiomiosarcomas se denominan tumores de músculo liso de comportamiento maligno incierto. La localización nasal de estos tumores es muy infrecuente y la extensión adecuada de la cirugía para tratar estas neoplasias no está bien definida. Se describe el caso clínico de una adolescente de 16 años, que consultó por padecer un tumor de aspecto vascular en la cavidad nasal derecha y que fue tratada con éxito mediante cirugía intranasal. El diagnóstico histológico fue tumor de músculo liso de comportamiento maligno incierto. Por la rareza de estas neoplasias, su infrecuente localización nasal y la falta de evidencia que soporte cuál debe ser la extensión de la cirugía, es relevante la descripción y discusión del caso clínico.

Smooth muscle tumors that cannot be histologically classified as leiomyomas or leiomyosarcomas are defined as smooth muscle tumors of uncertain malignant potential. The location of these tumors in the nose is very rare, and the appropriate surgical extent to manage these neoplasms has not been adequately defined. Here we describe the case of a 16-year-old female adolescent who consulted due to a vascular-like tumor in the right nasal cavity who was successfully treated with intranasal surgery. The histological diagnosis was smooth muscle tumor of uncertain malignant potential. Given that these neoplasms are rare, the uncommon location in the nose, and the lack of evidence indicating the extent of surgery, it is relevant to describe and discuss this clinical case.

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 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.

Tumores ano-rectales del músculo liso / Smooth muscle ano-rectal tumors

Introducción: Los tumores ano-rectales del musculo liso son raros, la relación respecto de los de recto es de 0.1%, presentándose con un rango entre 40-70 años. El objetivo es analizar una serie de pacientes, el tratamiento empleado y actualización bibliográfica. Material y Método: Sobre una base de datos retrospectiva entre enero de 1983 y diciembre de 2018, sobre 421 pacientes operados por cáncer recto-anal, fueron extraídos 6 que correspondieron a tumores del musculo liso. Resultados: Correspondieron al sexo femenino 4, con edades entre 49 y 75 años (57.5 años); 4 de localización rectal, de ellos 3 fueron leiomiosarcoma, y 2 anales (leiomioma). En 2 se obtuvo diagnostico preoperatorio de certeza por punción mediante Tru-Cut. De 3 pacientes con leiomiosarcoma, a 2 se les realizo cirugía radical y al restante biopsia. Los 2 resecados recidivaron a los 6 meses y al año. Los 3 fallecieron entre los 2 y 16 meses por progresión de la enfermedad. La paciente con diagnóstico de leiomioma rectal, operada mediante cirugía radical, se encuentra sin recidiva a 18 meses. Los 2 pacientes resecados localmente por leiomioma de ano, presentaron en el postoperatorio absceso y fistula extraesfinteriana, uno de ellos con incontinencia severa. Ambos fueron re-operados y se encuentran asintomáticos, libres de recidiva a los 36 y 60 meses. Discusión: Los tumores del músculo liso ano-rectal son infrecuentes y presentan síntomas inespecíficos. La biopsia preoperatoria es imperiosa a fin de establecer una adecuada estrategia quirúrgica. Los malignos tienen alto índice de recidiva y mortalidad. (AU)

Introduction: Smooth muscle ano-rectal tumors are rare; the relation with respect to the rectum is 0.1%, in a patient's age range between 40-70 years. The objective is the analysis of a series of patients, the treatment used and bibliographic update. Material and method: On a retrospective, database between January 1983 and December 2018. About 421 patients operated for rectum-anal cancer, of which 6 corresponded to smooth muscle tumors. Results: Four were female, with ages between 49 and 75 years (57.5 years average); 4 were of rectal location, of which 3 were leiomyosarcoma, and 2 anal (leiomyoma). In two, a preoperative diagnosis of certainty was obtained by Tru-Cut. Two out of 3 patients with leiomyosarcoma, underwent radical surgery and the remaining one a biopsy. The two resected relapsed at 6 months and at one year. All 3 died between 2 and 16 months due to disease progression. The patient diagnosed with rectal leiomyoma, operated by radical surgery, is without recurrence at 18 months. The 2 patients resected locally for anus leiomyoma showed abscess and extrasphincteric fistula in the postoperative period, one of them with severe incontinence. Both were re-operated and are asymptomatic, free of recurrence at 36 and 60 months. Discussion: Ano-rectal smooth muscle tumors are uncommon and have nonspecific symptoms. Preoperative biopsy is imperative in order to establish an appropriate surgical strategy. Malignant tumors have a high rate of recurrence and mortality. (AU)

Differential expression, diagnostic and prognostic significance of stathmin-1 and CD147 in various uterine smooth muscle tumors / Expresión diferencial, diagnóstico y significado de pronóstico de stathmin-1 y CD147 en diferentes tumores del músculo liso uterino

SUMMARY: Uterine smooth muscle tumors (USMT) are common, behavior-distinct gynecological tumors; including: leiomyoma (ULM), leiomyosarcoma (ULMS), and smooth muscle tumors of undetermined malignant potential (STUMP). Pre-operative distinction is difficult, thus diagnosis relies on histopathology. Immunohistochemistry (IHC) had been used to help in distinction. We studied two markers (stathmin-1 and CD147) to demonstrate whether they have diagnostic/ prognostic assist. Sixty seven USMT are studied. Age, follow up, and recurrence/metastasis data were collected. Representative slides were stained and Histologic score (HS) calculated as stain intensity (SI) X percentage of positive tumor cells (PP). Results were grouped as low expression (LE) and high expression (HE); then correlated to tumor types, and risk of recurrence/ metastasis. Statistical analysis (P < 0.05); Sensitivity, specificity, positive and negative predictive values and confidence intervals in diagnosing ULMS were calculated. Stathmin-1 HS (p= 0.000) and HE (p=0.002) were different among groups. Same as for CD147 HS and HE (both p=0.000), with a gradient increase from LM to STUMP to ULMS. Sensitivity, specificity, positive and negative predictive values and confidence intervals in diagnosing ULMS were as following: For stathmin-1 HS: 92 %; 20 %; 42 %; and 80 % (CI= 44-96 %). For Stathmin-1 HE: 80 %; 66 %; 60 %; and 84 % (CI=66-94 %). For CD147 HS: 85 %; 22 %; 41 %; and 69 %. For CD147 HE: 58 %; 49 %; 42 %; and 65 % (CI= 45-80 %), respectively. Recurrence / metastasis were documented in 6 cases (4 ULMS; 2 STUMP) with follow up ranging from 6 months to 102 months. 5 tumors had stathmin-1 HE (p=0.099); 2 had CD147 HE (p=0.393) in the primary tumors. STMN1 and CD147 are helpful diagnostic tests for USMT sub-typing, especially for ULMS. Gradient increase of expression from LM, to STUMP, to ULMS may indicate a role in malignant transformation in USMT, and in increased risk of recurrences/metastasis.

RESUMEN: Los tumores del músculo liso uterino (USMT, por sus siglas en inglés) son tumores ginecológicos comunes y de comportamiento distinto; incluyendo: leiomioma (ULM), leiomiosarcoma (ULMS) y tumores de músculo liso de potencial maligno indeterminado (STUMP). La distinción preoperatoria es difícil, por lo que el diagnóstico se basa en la histopatología. La inmunohistoquímica (IHQ) se había utilizado para ayudar en la distinción. Estudiamos dos marcadores (stathmin-1 y CD147) para demostrar si había efecto diagnóstico / pronóstico. Se estudiaron 67 USMT. Se recopilaron los datos de edad, seguimiento y recurrencia / metástasis. Las muestras representativas se tiñeron y la puntuación histológica (HS) se calculó como la intensidad de la tinción (IS) x porcentaje de células tumorales positivas (PP). Los resultados se agruparon como expresión baja (EB) y expresión alta (EA); luego se correlacionaeon con los tipos de tumores y el riesgo de recurrencia / metástasis. Análisis estadístico (P <0,05); se calcularon la sensibilidad, la especificidad, los valores predictivos positivos y negativos y los intervalos de confianza en el diagnóstico de ULMS. Stathmin-1 HS (p = 0,000) y HE (p = 0,002) fueron diferentes entre los grupos. Igual que para CD147 HS y HE (ambos p = 0,000), con un aumento de gradiente de LM a STUMP a ULMS. La sensibilidad, la especificidad, los valores predictivos positivos y negativos y los intervalos de confianza en el diagnóstico de ULMS fueron los siguientes: Para stathmin-1 HS: 92 %; 20 %; 42 %; y 80 % (IC = 44-96 %). Para Stathmin-1 HE: 80 %; 66 %; 60 %; y 84 % (IC = 66-94 %). Para CD147 HS: 85 %; 22 %; 41 %; y el 69 %. Para CD147 HE: 58 %; 49 %; 42 %; y 65 % (IC = 45-80 %), respectivamente. La recurrencia / metástasis se documentaron en 6 casos (4 ULMS; 2 STUMP) con un seguimiento que osciló entre 6 meses y 102 meses. Cinco tumores tenían stathmin-1 HE (p = 0,099); dos tenían CD147 HE (p = 0,393) en los tumores primarios. STMN1 y CD147 son pruebas de diagnóstico útiles para la subclasificación de USMT, especialmente para ULMS. El aumento en el gradiente de la expresión de LM, a STUMP, a ULMS puede indicar un papel en la transformación maligna en USMT y en un mayor riesgo de recurrencias / metástasis.

Leiomiosarcoma rectal: reporte de un caso / Rectal leiomyosarcoma: a case report

Introducción: Los leiomiosarcomas (LMS) son tumores raros del tubo digestivo, corresponden a neoplasias malignas originadas de células de músculo liso, representando sólo el 0,1% del total. La localización rectal se da en el 11% de los LMS, aunque representan menos del 1% de los tumores malignos colón- icos. En nuestro medio también es una patología de baja frecuencia. Objetivo: Exponer método diagnóstico, tratamiento, y resultado quirúrgico en paciente con LMS rectal. Caso Clínico: Paciente mujer, 53 años, atendida en el Hospital Padre Hurtado, gran tabáquica, que se presentó con un cuadro de 1 año de evolución de dolor perianal, defecación laboriosa y 3 episodios de rectorragia. Al examen físico presentaba al tacto rectal a 4 cm del margen anal una masa de ±2 cm de superficie irregular y de consistencia pétrea. Se realizó colonoscopía objetivando la masa tumoral y tomando biopsia la que se informó como LMS rectal, incluyendo resultados de inmunohistoquímica. Siguiendo con el estudio se realizó TAC y RNM. Se decide la intervención quirúrgica realizándose una resección anterior baja, sin incidentes durante la cirugía y con un buen postoperatorio. Conclusión: El leiomiosarcoma es de extrema baja frecuencia en nuestro país, con sólo pocos casos reportados. La confirmación diagnóstica es con biopsia e inmunohistoquímica. La cirugía sugerida en la literatura es la resección anterior baja, la que se realiza en este caso con buenos resultados. (AU)

Introduction: Leiomyosarcomas (LMS) are rare digestive tract tumors. It is a malignant tumor originated from smooth muscle cells, representing only 0.1% of the total. Rectal location is given in 11% of LMS, however, they represent less than 1% of all malignant colon tumors. It is a very infrequent tumor in our medium. Objective: Show the diagnostic method, treatment, and surgical result. Case Presentation: A 53-year-old female, treated in Padre Hurtado Hospital, heavy smoker, presented with a 1-year history of perianal pain, difficulty during defecation, and three episodes of rectal bleeding. Digital rectal examination showed a mass 4 cm from the anal margin of approximately 2 cm, of irregular surface, and very hard consistency. A colonoscopy was performed, objectivating the tumoral mass and taking a biopsy which was informed as a rectal LMS, including immunohistochemistry results. Continuing with the study, a CT-Scan and MRI were performed. Treatment of choice was surgery; a low anterior resection was carried out with no incidents during the surgery and a favorable post-operatory. LMS is extremely infrequent in our country, with only a few cases reported. Conclusion: Diagnostic confirmation is made through biopsy and immunohistochemistry. Surgery suggested by literature is low anterior resection, which was carried out in this case with good results. (AU)

Leiomiosarcoma primario vaginal en el embarazo. A propósito de un caso / Primary vaginal leiomyosarcoma in pregnancy. About A case

RESUMEN Los sarcomas primarios de vagina son extremadamente raros especialmente durante la gestación. Se presenta el caso de una gestante de 25+2 semanas que acude a urgencias con clínica de sangrado vaginal objetivándose a la exploración una formación nodular dependiente de pared vaginal derecha. Dada la clínica se reseca en quirófano con el hallazgo histopatológico de leiomiosarcoma. A la gestante se le realiza sin incidencias una cesárea en la semana 34 de gestación, previa maduración pulmonar fetal, completándose en el puerperio inmediato el estudio de extensión sin encontrarse ningún hallazgo relevante. Finalmente, se somete a la paciente a una histerectomía y salpinguectomía bilateral con preservación ovárica. El seguimiento y los controles sucesivos de la gestante en los meses posteriores muestran ausencia de enfermedad.

ABSTRACT Primary sarcomas of the vagina are extremely rare, especially during pregnancy. We report a case of a 25-week-pregnant who came to emergency room complaining of vaginal bleeding. Pelvic examination revealed a globular mass located on the right vaginal wall. Given the persistent bleeding, resection of the mass was performed. The specimen was sent to pathology and confirmed to be a leiomyosarcoma. At 34-weeks of pregnancy after fetal lung maturation a cesarean section is done. It was completed the extension study during immediate postpartum without any relevant finding. Finally, she had a totally hysterectomy and bilateral salpingectomy with ovarian preservation. Clinical follow-up over the following months shows no evidence of disease.

Atypical Leiomyomas of the Uterus: A Clinicopathologic Study of 54 Cases and an Immunohistochemical Analysis of Ki-67 and p53 / Leiomiomas atípicos del útero: un estudio clínico-patológico de 54 casos y un análisis inmunohistoquímico de Ki-67 y p53

ABSTRACT Objective: Uterine atypical leiomyomas (ALs) are extremely rare and occur in an age group almost one decade earlier than that for leiomyosarcomas. According to the literature, extensive clinicopathologic studies on ALs were limited to only two studies (2, 8). Atypical leiomyomas of the uterus are well-defined neoplasms with smooth muscle cells. The aim of this study was to investigate clinicopathologic findings in 54 cases diagnosed with ALs as well as Ki-67 and p53 expressions immunohistochemically. Methods: Fifty-four cases diagnosed between 2000 and 2013 were included. The histological and clinical features of the cases were reviewed, and their medical records were examined. Ki-67 and p53 were performed on all cases immunohistochemically. Results: The average age of the patients was 41.8 years. The average clinical follow-up period was 57 months. Hysterectomy was performed in 31 patients, and myomectomy in 21 patients, while resection of the mass was performed in two patients due to the intraligamentary mass. The average size of the neoplasms was 6.2 cm. Severe cellular atypia was noticed in 25 patients. While the number of mitoses was 1/10 high power field in 30 patients, it was 4/10 high power field in six patients. Ki67 was found to be positive in 50 patients at the rate of 1-5% immunohistochemically, while p53 demonstrated staining at the ratio of 10-15% staining in four patients. Conclusion: The differentiation of ALs from leiomyosarcomas is crucial. The recurrence rate after follow-up is 2%. In our opinion, the patients diagnosed with 'AL with limited experience' before should be correctly diagnosed as AL. We recommend that Ki-67 and p53 can be used as adjuvant markers immunohistochemically in the patients where a problem in differential diagnosis from leiomyosarcoma exists.

RESUMEN Objetivo: Los leiomiomas atípicos uterinos (LA) son extremadamente raros y se presentan en un grupo de edad casi una década antes que los leiomiosarcomas. De acuerdo con la literatura, los extensos estudios clínico-patológicos en los LA se limitaron a sólo dos estudios (2, 8). Los leiomiomas atípicos del útero son neoplasmas bien definidos con células de músculo liso. El objetivo de este estudio fue investigar los hallazgos clínico-patológicos en 54 casos diagnosticados con LA, así como las expresiones Ki-67 y p53, de forma inmunohistoquímica. Métodos: Se incluyeron cincuenta y cuatro casos diagnosticados entre 2000 y 2013. Se revisaron las características histológicas y clínicas de los casos y se examinaron sus historias clínicas. Ki-67 y p53 se realizaron en todos los casos de forma inmunohistoquímica. Resultados: La edad promedio de los pacientes fue de 41.8 años. El período promedio de seguimiento clínico fue de 57 meses. Se realizaron histerectomías a 31 pacientes, y miomectomías a 21 pacientes, en tanto que a dos pacientes se les realizó resección de la masa debido a la situación intraligamentosa. El tamaño promedio de los neoplasmas fue de 6.2 cm. Se observó atipia celular severa en 25 pacientes. El número de mitosis fue de 1/10 campos de gran aumento en 30 pacientes, en contraste con el número de mitosis de 4/10 campos de gran aumento en seis pacientes. Se encontró que Ki67 fue positivo en 50 pacientes a razón de 1-5% inmunohistoquímicamente, mientras que p53 mostró tinción a razón de 10-15% de tinción en cuatro pacientes. Conclusión: La diferenciación de LA entre los leiomiosarcomas es crucial. La tasa de recurrencia después del seguimiento es de 2%. En nuestra opinión, los pacientes diagnosticados con 'LA con experiencia limitada ' antes, deben ser diagnosticados correctamente como LA. Recomendamos que Ki-67 y p53 sean usados como marcadores adyuvantes inmunohistoquímicamente en los pacientes con un diagnóstico diferencial de leiomiosarcoma.

Cutaneous leiomyosarcoma on the face

Abstract: Leiomyosarcoma is a rare skin tumor, most common in white men in the fifth to eighth decades of life. Primary tumors are classified in dermal or subcutaneous, that differ by clinical and prognostic features. They may appear on any site of the body, but are rare on the face. A 54-year-old female was admitted with a 5cm exophytic nodular lesion of 8 months duration on the right cheek, site of previous chronic radiodermatitis. Histopathology revealed spindle-shaped cell neoplasia, positive for smooth muscle actin on immunohistochemistry. Cutaneous leiomyosarcomas on the face are rare and may occur in previously irradiated areas. Immunohistochemistry is mandatory for an accurate diagnosis. Its similarity with other tumors may complicate the diagnosis, with delay expansion of the tumor.

Periorbital leiomyosarcoma treated by means of conventional surgery and frontal myocutaneous flap

Abstract Cutaneous leiomyosarcoma is a rare neoplasia, and its periorbital presentation is rather uncommon. We present a case of a male patient who was surgically treated, with the reconstruction performed with a frontal myocutaneous flap. The patient has been followed up for one year with adequate oncologic control and good aesthetic outcome.

Thyroid leiomyosarcoma: presentation of two cases and review of the literature / Leiomiossarcoma da tireoide: apresentação de dois casos e revisão da literatura

Abstract Introduction: Leiomyosarcoma is a tumor which is rarely seen in the thyroid gland. The diagnosis may be difficult and the treatment is controversial. Objective: The objective of the study is to review the literature about a rare malignant disease of the thyroid gland which has high mortality. Methods: Two cases of thyroid leiomyosarcoma are presented and the previous 23 cases in the current literature are reviewed. Results: A total of 25 cases of thyroid leiomyosarcoma are reviewed; the most common complaint was rapidly growing anterior neck mass, and ten of the 25 patients had distant metastasis at the initial admission. Fifteen of the 25 patients died with the disease in the first 12 months after the diagnosis. Conclusion: The differential diagnosis of thyroid leiomyosarcoma is important and should be performed with other malignancies of the gland, especially with anaplastic carcinoma. The prognosis is poor and there is no consensus regarding the treatment.

Resumo Introdução: Leiomiossarcoma é um tumor raramente observado na glândula tireoide. O diagnóstico pode ser difícil e o tratamento é controverso. Objetivo: O objetivo do estudo foi revisar a literatura sobre um tumor raro da glândula tireoide que possui alto índice de mortalidade. Método: Dois casos de leiomiossarcoma da tireoide são apresentados, e os 23 casos anteriores relatados na literatura atual foram revisados. Resultados: Um total de 25 casos de leiomiossarcoma da tireoide foi revisado. A queixa mais comum foi o rápido crescimento de um tumor cervical anterior; 10 dos 25 pacientes apresentavam metástases distantes no momento da admissão. Quinze dos 25 pacientes foram a óbito nos primeiros 12 meses após o diagnóstico. Conclusão: O diagnóstico diferencial de leiomiossarcoma da tireoide é importante e deve ser feito com outras doenças malignas da glândula, especialmente carcinoma anaplásico. O prognóstico é ruim e não há consenso em relação ao tratamento.

High-grade Primary Renal Leiomyosarcoma

Objective: To investigate the clinical characteristics, prognosis, survival and diagnosis of high-grade primary renal leiomyosarcoma. Materials and Methods: From January 2003 to April 2013, 10 cases of high-grade primary renal leiomyosarcoma were retrospectively reviewed. We analyzed clinical manifestations, treatment and prognosis of our group and correlated to the literature. Results: Ten cases (five male and five female patients; age range 43­77 years, mean=57±std d:12.3 ) were enrolled. The mean diameter of the tumor masses was 9.35±4.5 cm (range 3-18 cm). 40% of the patients were asymptomatic while the major symptom of 60% patients was lumbar pain. Nephrectomy was performed in 90% of patients. Partial nephrectomy surgery was preferred for only one patient. Pleomorphism and necrosis with high-grade, pink spindle cell cytoplasm were viewed in all patients. All patients were high-grade, pink spindle cell cytoplasm and pleomorfism and necrosis were observed in all. In an immunohistochemical examination, vimentin was seen in 100%, desmin in 90% and smooth muscle actin in 80% of the patients. CD117 was negative in all patients. All of the cases were followed-up, and the time of survival varied from 6 to 68 months (mean 23.9±std d:20.1). No patient received adjuvant CTx and/or RTx. Conclusion: High-grade primary renal leiomyosarcomas (LMSs) are rare and highly malignant and the prognosis is poor. Early diagnosis and radical nephrectomy can prolong the patient's life. Surgery is the main treatment modality for renal (leiomyosarcoma) LMS

Unusual Malignant Solid Neoplasms of the Kidney: Cross-Sectional Imaging Findings

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.

Leiomiosarcoma: un raro tumor renal / Leiomyosarcoma: a rare renal tumor

Introducción: El leiomiosarcoma renal es un tumor raro que constituye el 0,12 por ciento de todas las neoplasias renales malignas; su presentación clínica es muy variable. Caso clínico: Se reporta el caso de un hombre de 68 años de edad con lumbalgia derecha, en una tomografía contrastada de abdomen y pelvis se detecta una gran masa tumoral dependiente del polo superior renal derecho. Se realizó una nefrectomía radical y la resección incidental de una lesión pulmonar basal derecha, el diagnóstico histopatológico y de inmuno-histoquímica fue de un leiomiosarcoma renal maligno de alto grado metastásico a pulmón.

Introduction: Renal leiomyosarcoma is a rare tumor constituting 0.12% of all malignant renal neoplasms; the clinical presentation is highly variable. Clinical case: We report the case of a 68 year old man who presented with right back pain; CT scan (computed tomography) showed a large mass located at upper pole of the right kidney. We performed a radical nephrectomy and resection of a right basal lung node incidentally found, the histopathological and immunohistochemical diagnosis was a renal high-grade leiomyosarcoma with metastases to the lung.

Metastatic pattern of uterine leiomyosarcoma: retrospective analysis of the predictors and outcome in 113 patients / 부인종양

OBJECTIVE: To describe metastatic pattern of uterine leiomyosarcomas (ULMS) and correlate it with clinical and histopathologic parameters. METHODS: We included 113 women (mean age, 53 years; range, 29 to 72 years) with histopathology-confirmed ULMS from 2000 to 2012. Distribution of metastases was noted from imaging by two radiologists in consensus. Predictors of development of metastases were analyzed with univariate and multivariate analysis. Impact of various clinical and histopathologic parameters on survival was compared using Log-rank test and Cox proportional hazard regression model. RESULTS: Distant metastases were seen in 81.4% (92/113) of the patients after median interval of 7 months (interquartile range, 1 to 21). Lung was most common site of metastases (74%) followed by peritoneum (41%), bones (33%), and liver (27%). Local tumor recurrence was noted in 57 patients (50%), 51 of whom had distant metastases. Statistically significant correlation was noted between local recurrence and peritoneal metastases (p<0.001) and between lung and other common sites of hematogeneous metastases (p<0.05). Age, serosal involvement, local recurrence, and the International Federation of Gynecology and Obstetrics (FIGO) stage were predictive factors for metastases. At the time of reporting, 65% (74/113) of the patients have died; median survival was 45 months. Stage, local recurrence, and age were poor prognostic factors. CONCLUSION: ULMS metastasizes most frequently to lung, peritoneum, bone, and liver. Local recurrence was associated with peritoneal spread and lung metastases with other sites of hematogeneous metastases. Age, FIGO stage and local recurrence predicted metastatic disease and advanced stage, older age and local recurrence predicted poor outcome.

A case of collision tumor or transdifferentiation between malignant melanoma and leiomyosarcoma.

A 73-year-old woman was referred to the hospital due to a pigmented, asymptomatic nevus on her right arm that had changed in size and color. The histopathological examination showed a superficial spreading malignant melanoma, Clark level III, 2.26 mm in thickness. Two years later, the patient presented a 10 cm rapidly growing mass in her right axilla. The mass in the axilla measured 12.5 ΄ 9 ΄ cm. It revealed a lymph node metastases with a tumor growth composed of two different contiguous morphological and immunohistochemical components, respectively, melanosomes and leiomyosarcoma. The combination of a melanocytic nevus with other tumor of epidermal or adnexal origin has been described before, but still the co-existence of two different neoplasms within a lesion is still uncommon. The most common combination is basal cell carcinoma and melanocytic nevus or one of them together with a seborrheic keratosis. There have also been occasional reports of rhabdomyosarcomatous differentiation. However, mesenchymal differentiation, and in this case leiomysarcoma, with formation of heterologous elements in melanocytic tumor is very rare. Another plausible explanation may be that malignant melanoma cells could have transdifferentiated into a leiomyosarcomatoid phenotype with resulting metastases of either type. Malignant melanomas have shown a wide variety of cytological changes and can mimic carcinomas, lymphomas, and sarcomas. Spindle cell melanomas commonly simulate spindle cell carcinomas. It has also been documented that desmoplastic melanomas can change into fibroblastic, Schwannian, and myofibroblastic differentiation.

Leiomiossarcoma cutâneo no tronco / Cutaneous leiomyosarcoma on the trunk

O Leiomiossarcoma cutâneo é um tumor maligno que representa 7 por cento dos sarcomas dos tecidos moles, afetando, mais frequentemente, homens entre os 50-70 anos. A localização, no tronco, é atípica, constituindo 10 a 15 por cento dos casos. A radioterapia e o traumatismo prévio têm sido referidos como fatores de risco. Descrevemos um homem de 57 anos, com tumor eritemato-violáceo, indolor, de consistência dura, localizado na região pré-esternal, com cerca de oito meses de evolução. A histopatologia evidenciou tumor maligno de células fusiformes, com núcleos em forma de "charuto", pleomórficos, com alto índice mitótico, ocupando toda a espessura da derme. Estas células expressaram actina do músculo liso, desmina e vimentina e foram negativas para proteína S-100 e pancitoqueratina. Foi efetuada a exérese cirúrgica radical do tumor.

Cutaneous leiomyosarcoma is a relatively uncommon tumor that accounts for 7 percent of all soft tissue sarcomas. It occurs more frequently in males between 50-70 years and only 10-15 percent of cases are located on the trunk. Radiotherapy and previous trauma have been implied as risk factors. We report the case of a 57 year-old male with an eight-month history of a hard painless erythematous-violaceous tumor on the presternal region. Histopathology evidenced a malignant spindle cell tumor, "cigar" shaped, with pleomorphic nuclei and a high mitotic index that occupied the entire dermal thickness. Immunohistochemical staining of the tumor cells was positive for smooth muscle actin, desmin and vimentin and negative for S-100 protein and pan-cytokeratin, which supported the diagnosis of dermal leiomyosarcoma. Radical surgery was performed to remove the tumor.

[Diagnostic value of immunohistochemical markers in uterine smooth muscle tumors]

Uterine smooth muscle tumors are the most common human neoplasms. They are divided clinically as benign and malignant but there is another group of lesions which is difficult to place in these two categories, so-called [STUMP] and differentiation of these tumors on the basis of H and E staining is impossible. The aim of this study was to find a set of distinguishing objective biomarkers and a survey of immunohistochemical [IHC] markers comparing these three groups of tumors. Twenty one [21] cases in each group of the above mentioned tumors were randomly selected [63 cases in total] from pathobiology laboratories of Kermanshah and Shiraz cities and studied by IHC for expression of ER, PR, C-kit, Bcl-2, P16, and P53. The frequency of IHC markers expression in leiomyosarcoma [LMS] were as follow: ER [5.26%], PR [15.52%], P53 [26.31%], Bcl-2 [15.78%], C-kit [15.52%] and P16 [15.42%]. In the STUMP cases: PR [23.80%], Bcl-2 [4.76%] and P16 [9.52%], ER, C-kit and P53 were not expressed. In Leiomyoma group, only Bcl-2 was expressed [14.28%] and the others had no expression. P53 and P16 expression between the two groups of Leiomyoma and LMS, and also STUMP and LMS were significantly different [P<0.05]. P53 and P16 are reliable markers for differentiation between STUMP and LMS and also Leiomyoma from LMS and have a high diagnostic specificity. STUMP is used to identify cases in which clinicopathologic findings for final diagnosis as benign or malignant is not adequate. In this study a high similarity of STUMP group of immunohistochemical profile with benign cases was seen