Informe de un caso de liposarcoma paratesticular

Los liposarcomas son neoplasias que se originan de las células mesenquimales, y su localización paratesticular es infrecuente. Se dividen en cuatro subtipos histológicos, siendo el mixoide el más raro. Se muestra el informe de un caso de liposarcoma paratesticular mixoide y una revisión de la literatura, con el objetivo de brindar información sobre esta rara enfermedad. El caso es un paciente masculino de 70 años de edad, que acude a consulta por tumefacción escrotal derecha e indolora de 13 meses de evolución, diagnosticado inicialmente como una hernia inguinal unilateral. Se realizó orquiectomía inguinal radical derecha y ligadura de cordón alto. En anatomía patológica se recibe la pieza quirúrgica de 2500 g. Al estudio histológico se evidencia liposarcoma mixoide. La tomografía computarizada de tórax, abdomen y pelvis no mostró metástasis. La presencia de un liposarcoma debe tenerse en cuenta durante el estudio diagnóstico de masas escrotales, para minimizar la tasa de diagnóstico erróneo y manejo inadecuado.

Liposarcoma are neoplasms that originate from mesenchymal cells, and their paratesticular location is infrequent. They are divided into four histological subtypes, the myxoid being the rarest. The report of a case of myxoid paratesticular liposarcoma and a review of the literature are shown, with the aim of providing information on this rare disease. The case is a 70-years-old male patient who assists the clinic due to painless right scrotal swelling of 13 months evolution, who was initially diagnosed with a unilateral inguinal hernia. Right radical inguinal orchiectomy and high cord ligation were performed. The pathological piece of 2500g is received in pathological anatomy. Myxoid liposarcoma is evidenced at histological study. Chest, abdomen and pelvis computed tomography showed no metastasis. The presence of liposarcoma should be taken into account during the diagnostic study of scrotal masses to minimize the rate of misdiagnosis and inadequate management.

Histopathological Spectrum of Myxoid Soft‑Tissue Neoplasms in a Tertiary Care Center with a Special Focus on Vascular Patterns: A 13‑Year Compilation

Introduction: Myxoid soft‑tissue tumors are a diverse group of tumors which have similar histomorphology but have varied geneticsequence and clinical outcome, hence differentiating and diagnosing them is a challenge for any pathologist. This study describes the varioushistomorphological spectrum and vascular pattern of various myxoid soft‑tissue tumors. Materials and Methods: This was a retrospective and prospective observational study of myxoid soft‑tissue tumors over a period of 13 years. A total of 224 cases with myxoid morphology were included and were examined morphologically with a special focus on the vascular pattern. SPSS v 24 was used for statistical analysis. Results: The predominant lesions were benign in 164 (73.21%) cases, followed by malignant lesions in 43 (19.19%) cases and intermediate lesions 17 (7.58%) cases. Both benign and malignant lesions showed a male preponderance and were seen to arise predominantly from the extremities. The most common benign myxoid lesions in this study were of neural origin with myxoid neurofibroma constituting 65 (29. 01%) cases, followed by schwannoma 38 (16.9%) cases. Myxoid dermatofibrosarcoma protuberans was the most common intermediatelesion. Tumors with adipocytic differentiation were the predominant lesions among the malignant group, i.e myxoid liposarcoma seen in 17 (7.5%) cases. Conclusions: Vascular pattern in the myxoid lesions are subtle yet crucial in arriving at a histo‑morphological diagnosis. Further studies correlating the vascular pattern with the genetic profile of these tumours can help arriving at a histo‑morphological diagnosisof myxoid lesions.

A case of acral fibrochondromyxoid tumor / 中华皮肤科杂志

A 44-year-old male patient presented with a subcutaneous nodule in the left little finger for 3 years. Skin examination showed a subcutaneous nodule with rubber-like hardness but no tenderness on palpation, measuring 0.4 cm × 0.4 cm in size at the dorsal distal aspect of the left little finger, and the movement of the distal interphalangeal joint was unrestricted. Postoperative histopathological examination revealed that the tumor contained abundant stroma consisting of variable fiberous, chondroid and myxoid materials; tumor cells were oval to short spindle-shaped with inconspicuous nucleoli but no mitosis; cells were arranged haphazardly or in small clusters. Immunohistochemical study showed positive staining for vimentin, CD34 and transcription factors ERG and SOX9, but negative staining for S100, P63, broad-spectrum cytokeratin AE1/AE3, epithelial membrane antigen, smooth muscle actin and desmin in tumor cells, and the Ki67 labeling index was below 1%. Finally, the patient was diagnosed with acral fibrochondromyxoid tumor.

A rare case of vulvar extraskeletal myxoid chondrosarcoma: mimics and diagnostic clues

Only 14 cases of extraskeletal myxoid chondrosarcoma (EMC) of the vulva have been documented in the literature. We report a case of a 63-year-old woman with EMC of the vulva confirmed by both EWSR1 and NR4A3 fluorescence in situ hybridization, the latter of which is a more specific probe for this entity. The unusual location of this tumor of prominent myxoid morphology gave rise to a wide differential diagnosis, which necessitated thorough histologic evaluation and confirmatory ancillary testing in the form of immunohistochemistry and cytogenetic studies. This article aims to review extraskeletal myxoid chondrosarcoma of the vulva and various diagnostic clues to help differentiate it from its histologic mimics. This is the fifth case of vulvar EMC in the literature with confirmation of a NR4A3 gene rearrangement.

Liposarcoma mixoide de omento mayor. Presentación de un caso y revisión de la literatura / Myxoid Liposarcoma of the Greater Omentum. Case Report and Literature Review

RESUMEN El liposarcoma es un tumor maligno de origen mesodérmico derivado del tejido adiposo. Es el más frecuente de los sarcomas de partes blandas en el adulto. La localización más frecuente es en extremidades inferiores; la abdominal es rara. Llega a alcanzar enormes proporciones. El tratamiento es la cirugía con resección completa para el control local de la enfermedad. Se presenta el caso de una paciente que acudió por hallazgo ecográfico de tumor intrabdominal en hipogastrio de origen no precisado, con evolución asintomática. Al examen físico se constató proceso tumoral palpable hacia hipogastrio de aproximadamente 3cm, suave, móvil, no superficial, no doloroso a la palpación. Se realizó laparotomía exploradora electiva, con omentectomía total por tumor en borde libre del omento mayor. La biopsia informó la presencia de liposarcoma mixoide de omento mayor. Debido a la poca frecuencia de este tipo de lesión se decide presentar el caso de esta paciente.

ABSTRACT Liposarcoma is a malignant tumor of mesodermal origin derived from adiposetissue. It is the most frequent of the soft tissue sarcomas in adults. Themost frequent location is in the lower extremities, the abdominal is rare.It reaches enormous proportions. Treatment is surgery with completerresection for local control of the disease. The case of a patient whopresented due to ultrasound finding of an intra-abdominal tumor in thehypogastrium of unspecified origin, with asymptomatic evolution, ispresented. Physical examination revealed a palpable tumor process towardsthe hypogastrium of approximately 3cm, smooth, mobile, non-superficial, notpainful on palpation. Elective exploratory laparotomy was performed, withtotal omentectomy for tumor on the free edge of the greater omentum. Biopsyreported the presence of myxoidliposarcoma of the greater omentum. Due tothe infrequency of this type of injury, it was decided to present the caseof this patient.

Clinicopathologic and molecular characteristics of myxoid angiomatoid fibrous histiocytoma / 中华肿瘤杂志

Objective@#To investigate the molecular genetic and clinicopathologic characteristics, immunophenotypes, diagnostic and differential diagnostic features of myxoid angiomatoid fibrous histiocytoma (MAFH).@*Methods@#Three cases of MAFH were collected from the archives of Zhejiang Provincial People′s Hospital between January 2015 to August 2018. The clinical and radiologic features, histomorphology, immunohistochemistry, molecular genetics and prognosis were analyzed.@*Results@#Patients consisted of 2 women and 1 man aged 37 years, 46 years, and 57 years, respectively. The clinical manifestations of 3 patients were presented as a painless, slowly-enlarged mass with a duration ranging of 2 weeks, 2 months and 50 years. These tumors were located at the deep somatic soft tissue of extremities or limbs (right hip, left forearm, left wrist, respectively) and 2 were preoperatively considered as ganglion cyst or giant cell tumor of tendon sheath by imaging examinations. The diameter of circumscribed mass lesion was ranged from 3.0 to 7.5 cm, which exhibited a gray white to tan and gelatinous cut surface. Extensive hemorrhage and cystic changes were observed in 2 cases. Under low magnification, all tumors showed a dense fibrous pseudo-capsule with a peritumoral lymphoplasmacytic cuff and a multi-nodular growth pattern. Blood-filled cystic spaces were observed in 2 tumors. The myxoid stroma occupied 60.0%, 80.0% and 90.0% area of the entire tumor, respectively. Within the myxoid areas, tumor cells were oval to stellate and arranged in cord-like, microcystic and reticular growth patterns. Transitions of myxoid tumor components to more solid areas with typical histology of angiomatoid fibrous histiocytoma (AFH) were observed at least focally in all the three cases. The tumor cells exhibited minimal atypia and scarce mitoses (1 to 2/50 HPF) without necrosis, and prominently focal intracytoplasmic vacuoles were identified in one case. The results of immunohistochemistry staining showed that, 2/3 cases focally expressed desmin, 2/3 focally expressed epithelial membrane antigen (EMA), and 1/3 focally expressed CD99. The positive index of Ki67 was approximately 1% to 5%. Fluorescence in situ hybridization analysis showed that EWSR1 gene rearrangement occurred in all of the three cases. During the period of follow-up, one case showed local recurrence at 15 months, one case showed postoperative recurrence at 24 months, and the recurrent tumor slowly grew for 120 months until the second resection, without recurrence at the following 2 months. The left case showed a disease-free survival at 32 months.@*Conclusions@#MAFH is a rare subtype of AFH with a low-grade behavior and may lead to diagnostic confusions. Carefully searching for the typical AFH histomorphology and combining with EWSR1 gene rearrangement test can help to distinguish MAFH from other mimickers.

Myxoid Liposarcoma: A Rare Soft Tissue Tumour in the Breast

@#Liposarcoma is one of the most common mesenchymal tumour in adults but it is rare to occur in the breast. Our case was a 50 year old single nulliparous woman who presented with a right breast mass for one year duration. The mass was progressively increasing in size in the last few months. Breast examination showed a huge mass measuring 5 x 8 x 6 cm occupying the entire right breast. Mammogram showed a large homogenous soft tissue mass occupying the entire right breast with foci of calcification. A trucut biopsy showed a cellular tumour which was thought to be an invasive carcinoma. The patient underwent right modified radical mastectomy with axillary clearance. Macroscopy showed a well circumscribed lobulated solid haemorrhagic yellowish tumour mass measuring 180 x 110 x 50 mm. Microscopically the tumour was heterogenous comprising cellular round nonlipogenic mesenchymal cells and loose myxoid areas containing small cells. The typical arborizing ‘chicken wire’ capillaries were observed. Vacuolated lipoblasts were seen. All eleven axillary lymph nodes sampled showed no metastasis. A diagnosis of a myxoid liposarcoma was made. To raise the suspicion of a possible mesenchymal tumour, it is very important for clinicians to relay the clinical and radiological findings to the pathologist to avoid misdiagnosis in a trucut biopsy.

Leg myxoid liposarcoma: correlation between image and anatomopathological tests / Lipossarcoma mixoide da perna: correlação entre os exames de imagem e anatomopatológico

ABSTRACT A 41-year-old male patient refers to a painful tumor on the lateral side of the right leg. Ultrasonography visualized a predominantly hypoechoic well-delineated large heterogeneous mass in the subcutaneous tissue, showing an internal hyperechoic area. Magnetic resonance imaging (MRI) demonstrated an oval and lobulated tumor with well-defined margins at the anterolateral part of the leg, with contrast enhancement. The anatomopathological evaluation revealed a neoplasm with an extensive chondromyxoid matrix. The immunohistochemical study demonstrated positive vimentin (V9 clone), positive S-100 protein and negative desmin (D33 clone), smooth muscle actin (1A4), CD68 (KP1 clone) and caldesmon. The set of findings is compatible with myxoid liposarcoma.

RESUMEN Paciente masculino de 41 años de edad, reporta tumor doloroso en la parte lateral de la pierna derecha. La ultrasonografía demostró una masa voluminosa, hipoecogénica, heterogénea y bien delimitada en el tejido subcutáneo, con área hiperecogénica en su interior. La resonancia magnética (RM) reveló un tumor ovalado y lobulado, con márgenes bien definidas en la parte anterolateral de la pierna, presentando realce de contraste. El estudio anatomopatológico constató neoplasia con abundante matriz condromixoide. La inmunohistoquímica presentó vimentina (clon V9) y proteína S-100 positivas, y desmina (clon D33), actina de músculo liso (1A4), CD68 (clon KP1) y caldesmón negativos. El conjunto de hallazgos es compatible con liposarcoma mixoide.

RESUMO Paciente do sexo masculino, 41 anos de idade, relata tumor doloroso na face lateral da perna direita. A ultrassonografia demonstrou massa volumosa, hipoecogênica, heterogênea e bem delineada no tecido subcutâneo, com área hiperecogênica em seu interior. A ressonância magnética (RM) revelou um tumor ovalado e lobulado, com margens bem definidas na parte anterolateral da perna, apresentando realce pelo contraste. O estudo anatomopatológico constatou neoplasia com extensa matriz condromixoide. O estudo imuno-histoquímico apresentou vimentina (clone V9) e proteína S-100 positivas e desmina (clone D33), actina de músculo liso (1A4), CD68 (clone KP1) e caldesmon negativos. O conjunto de achados é compatível com lipossarcoma mixoide.

A case of laparoscopic resection of myxoid liposarcoma of the greater omentum

@#Primary omental liposarcoma is a rare clinical entity with less than 20 cases being reported in the literature. Laparotomy has been the traditional approach for resection, with no reports of laparoscopic resection. A 39-year-old lady presented at the Sarawak General Hospital, Kuching, Malaysia with a history of a progressive, painless left upper quadrant abdominal swelling for a year. CT scan showed a well-defined heterogeneously enhancing cystic mass measuring 7.5x7.5x8.1cm with a poor plane with the adjacent greater curvature of stomach and transverse colon. Upper and lower endoscopy was normal. The tumour was completely dissected from the adjacent transverse colon and removed laparoscopically. Histopathological examination of the resected specimen revealed a myxoid liposarcoma. She had an uneventful recovery and was discharged well on the third postoperative day. She subsequently underwent adjuvant chemotherapy and was well at 1-year follow-up with PET CT showing no evidence of recurrence or metastases.

A Case of Solitary Cutaneous Myxoma / 대한피부과학회지

No abstract available.

Myxoid Liposarcoma: A Single Institute Experience / 대한정형외과학회잡지

PURPOSE: This study assessed the treatment outcomes of myxoid liposarcoma in the extremities and investigate the prognostic factors. MATERIALS AND METHODS: A total of 91 patients with myxoid liposarcoma (83 primary, 8 recurrent) between 2001 and 2015 were reviewed retrospectively. The local recurrence and metastasis after treatment were examined. The survival rates and prognostic factors affecting the survival were investigated. The mean follow-up was 84 months (range, 5–196 months). RESULTS: The overall survival rates at 5-yr and 10-yr were 82% and 74%, respectively. The tumor size (p=0.04), round cell component (p<0.0001), grade (p=0.0002), and local recurrence (p=0.006) affected survival in primary patients. Extrapulmonary metastases were observed in 75.0% (18/24) of metastatic patients and the mean post metastasis survival was 26 months (range, 2–72 months). CONCLUSION: Myxoid liposarcoma developed mainly at the lower extremities. The tumor size, grade, component of round cells, and local recurrence were associated with the prognosis. The unique feature of extrapulmonary metastasis in myxoid liposarcoma should be noted in the treatment and follow-up.

Pathological significance of NY-ESO-1 expression in the diagnosis of myxoid liposarcoma / 中华病理学杂志

Objective@#To detect the expression of New York esophageal squamous cell carcinoma antigen 1 (NY-ESO-1) in common types of mesenchymal myxoid tumors, and to investigate its significance in the diagnosis and differential diagnosis of myxoid liposarcoma.@*Methods@#A total of 43 formalin-fixed paraffin-embedded samples of mesenchymal myxoid tumors from the Affiliated Hospital of Qingdao University and Qingdao Municipal Hospital ranging between 2010 and 2017 were selected. NY-ESO-1 expression was detected by immunohistochemical staining. DDIT3 gene status was detected by fluorescence in situ hybridization (FISH). NY-ESO-1 mRNA was detected by reverse transcription-PCR (RT-PCR).@*Results@#Histopathology and FISH results confirmed that there were 11 cases of myxoid liposarcoma and 32 other types (including 7 cases of well-differentiated liposarcoma, 1 dedifferentiated liposarcoma, 3 lipomas, 2 lipoblastomas and 19 non-adipocytic tumors). Immunohistochemical staining showed that the positive expression propotion of NY-ESO-1 in myxoid liposarcoma was 11/11, and the positive location was the cytoplasm and nucleus of lipoblast cells. The expression intensity is higher in regions with round cell differentiation. Among the 32 cases of other mesenchymal myxoid tumors, only one well-differentiated liposarcoma showed positive immunoreactivity for NY-ESO-1. RT-PCR confirmed that 7 cases of myxoid liposarcoma (7/11) and one well-differentiated liposarcoma (1/7) had NY-ESO-1 mRNA expression.@*Conclusions@#NY-ESO-1 is positively expressed in myxoid liposarcoma. It can be served as a useful marker for the diagnosis and differential diagnosis of myxoid liposarcoma.

Angiomiofibroblastoma-like, un tumor excepcional: presentación de un caso clínico y diagnóstico diferencial / Angiomyofibroblastoma-like, an exceptional tumor: presentation of a clinical case and differential diagnosis

Presentamos un caso de Angiomiofibroblastoma-like (AML), en el que la ecografía fue importante para determinar la detección, localización y extensión local. Hallazgos: La ecografía demostró una lesión paratesticular sólida hipoecogénica de bordes bien definidos lo que sugirió lesión benigna extratesticular. La histología evidenció una neoplasia mesenquimal benigna tipo mixoide en cuyo diagnóstico diferencial se incluyen el Angiomixoma superficial (AMS), Angiomixoma agresivo (AMA), Angiomiofibroblastoma (AMF) y el AML. En base a estos hallazgos se realizó una revisión de tumores de similares características en la literatura, llegando finalmente al diagnóstico de AML cuya localización paratesticular sólo ha sido descrita en muy pocos casos hasta la fecha. Conclusión: La ecografía es útil para localizar los tumores paratesticulares y determinar su extensión local. No obstante, para llegar al diagnóstico definitivo es necesario realizar un estudio histológico e inmunohistoquímico de la tumoración.

We present a case of a paratesticular Angiomiofibroblastoma-like (AML) tumor in which ultrasound was important to give the specific location and local extension of the lesion. Findings: Ultrasound revealed a hypoechoic paratesticular lesion with well-defined borders, suggesting an extratesticular benign lesion. The histology showed a benign mesenchymal myxoid-type neoplasm. The differential diagnosis included superficial Angiomyxoma (AMS), Aggressive angiomyxoma (AMA), Angiomyofibroblastoma (AMF) and AML. Based on these findings, a review of similar tumors was carried out and ultimately led to the diagnosis of paratesticular AML. This location has been described only in a few cases in the literature. Conclusion: Ultrasound is useful to locate paratesticular tumors and determine their local extension. However, a definitive diagnosis still requires a histological and immunohistochemical study.

A Case Report Of Myxoid Neurothekeoma Of The Scalp - A Rare Soft Tissue Tumour

Neurothekeoma is a benign soft tissue tumour of neural origin. It is mostly seen in young females and commonly involves the head and neck region. We present a case of a 47 year old male, who presented with a nodule on his scalp. It was clinically diagnosed to be a case of sebaceous cyst however histologically proven to be a myxoid neurothekeoma. The histologic variants include myxoid, cellular and mixed tumors. Myxoid neurothekeoma is a cutaneous neoplasm of nerve sheath origin. This case report presents the rarity of the lesion that needs to be differentiated from lesions of similar histological features.

Two Cases Report of Myxoid Degeneration of the Auricular Cartilage / 대한이비인후과학회지

Myxoid degeneration (MD) in the cartilage results from the accumulation of hyaluronic acid in the stroma. However, it is rarely found in the auricular cartilage, with only one published report to date. This article describes two histologically confirmed cases of MD of the auricle that was excised with favorable aesthetic results. Two men presented with auricular masses, with no history of trauma or tumors in the auricle. Laterally protruding masses were located around the helix and antihelix, which were similar in appearance to the normal auricular cartilage. We made an aesthetic skin incision under local anesthesia, and carved the mass from the normal cartilage for pathological and cosmetic reasons. Both excised masses showed MD of the auricular cartilage. We report these two cases with a review of the literature.

Liposarcoma mixoide del muslo: a propósito de un caso / Myxoid liposarcoma of the thigh: a case report

Fundamento: los tumores malignos de partes blandas no son lesiones frecuentes, sin embargo, el liposarcoma es que el incide con mayor frecuencia entre estas enfermedades. Objetivo: mostrar un paciente con un lipoma mixoide del muslo. Caso clínico: paciente de 49 años de edad, blanco, masculino con antecedentes de salud anterior, el cual hace alrededor seis años comenzó con una bolita en la cara interna del muslo derecho, en los últimos seis meses ha crecido muy rápido. El paciente en todo momento refirió ausencia de dolor y justifica la demora en asistir al médico por tener miedo. A la exploración física del muslo derecho se detectó una masa de bordes irregulares de consistencia mixta, adherida a planos profundos por debajo de la fascia, fija pero no adherida al hueso no móvil, aunque no en relación a estar adherida al hueso, de 30 centímetros de largo, por 20 centímetros de ancho y 15 centímetros de profundidad, localizada en la cara interna del muslo derecho. Mediante la palpación se constató aumento de volumen y de la temperatura local del muslo. Se realizaron exámenes imaginológicos dentro de los que se incluyeron, ultrasonido diagnóstico de alta resolución, tomografía axial computarizada, imagen de resonancia magnética y arteriografía. Al tener en cuenta todos los elementos anteriores el paciente es llevado al salón de operaciones donde un equipo multidisciplinario compuesto por ortopédicos, angiólogos, cirujanos generales y anestesiólogos realizó la exceresis completa de la tumoración, a través de un margen de seguridad. Conclusiones: el liposarcoma mixoide es el tumor maligno de partes blandas más frecuente, así como su localización en el esqueleto apendicular, en particular en el muslo.

Background: malignant soft tissue tumors are not common, but among them liposarcoma has the higher incidence. Objective: to show a patient with a myxoid liposarcoma of the thigh. Case report: a 49-year-old white man, with record number 207009. The patient was taken to the Outpatient Orthopedic Department because of a soft tissue mass found in the right thigh for about six years, which in the last six months grew quickly. There was no pain and the patient refused medical assistance because of fear. On palpation a big soft tissue mass was detected with irregular margins, mix consistency, location under the fascia, deep-seated tumor although not fixed to the bone, and 30 centimeters long, 20 centimeters width and 15 centimeters deep found on the right thigh. Imaging exams like ultrasound, computed axial tomography, magnetic resonance imaging and arteriography were performed. The patient underwent surgery and a wide resection was carried out by a team of orthopedics, angiologists, general surgeons and anesthesiologists. Tumor resection was done by a safe margin. Conclusions: myxoid liposarcoma is the most common soft tumor as well as its location at the appendicular skeleton, like the thigh.

Liposarcoma en lactante: Reporte de un caso / Liposarcoma in an infant: Case report

Liposarcomas, tumores adiposos propios en edad adulta, excepcional en niños, reportándose solo el 6% de los casos en la 2da década de la vida, dos tercios son lipomas, 30% lipoblastomas y 2% liposarcomas. Lactante menor de 10 meses con aumento de volumen progresivo en región gemelar derecha de 3 meses de evolución, masa palpable de 10,5 x 19 cm, renitente, no dolorosa, bordes definidos, circulación colateral, funcionalidad normal. Ecosonogramas: no concluyente. Biopsia insicional: fibrolipomas adultos diferenciados. Biopsia excisional: tumor lipomatoso gigante con patrón mixoide consono con lipoma. Inmunohistoquimico: liposarcoma de bajo grado, positivo para proteína s-100.

Liposarcoma, fatty tumors described in adults, rare in children, reporting only 6% of cases in the 2nd decade of life, two-thirds are lipomas, 30% lipoblastomas and 2% liposarcoma. A 10 month old infant with progressive increase of the right gastrocnemius region with a 3 months evolution, with palpable mass of 10.5 x 19 cm, painless, defined borders, collateral circulation and normal functionality. Ecosonograms: inconclusive. Incisional biopsy reports: differentiated adult fibrolipomas. Excisional biopsy reports: giant lipomatous tumor with myxoid pattern consistent with lipoma. Immunohistochemical reports: low-grade liposarcoma positive for s-100 protein.

Válvula aórtica bivalva con cambios de degeneración mixoide asociados / Bivalve aortic valve with associated myxoid degeneration changes

Resumen:En la actualidad con el uso generalizado de los antibióticos, la fiebre reumática ha perdido el primer puesto como causa de afección de las válvulas del corazón, cediéndolo a otras patologías como el debilitamiento del tejido de la válvula ocasionado por la degeneración mixomatosa, el acumulación de calcio en las válvulas denomina degeneración cálcica, o el hecho mismo de nacer con una válvula de forma irregular como lo es válvula aortica bivalva. Precisamente, esto da pie a la presentación del caso de un joven masculino conocido sano de 16 años de edad el cual muere de forma súbita mientraspractica el ciclismo de forma recreativa.

Summary:Today with the widespread use of antibiotics, rheumatic fever has lost first place as a cause of disease of the heart valves, ceding it to other pathologies such as the weakening of the tissue of the valve caused by myxomatous degeneration, the accumulation of calcium valves called calcific degeneration, or being born with a valve same irregular shape as bicuspid aortic valve is. Precisely, this leads to the presentation of the case of a healthy young male known 16-year- old who suddenly dies while practicing cycling recreationally.

Clinicopathologic analysis of primary pulmonary myxoid sarcoma with EWSR1 translocation / 中国癌症杂志

Background and purpose: Primary pulmonary myxoid sarcoma (PPMS) is a very rare lung tumor that has recently been shown to harbor EWSR1-CREB1 translocation. This study aimed to investigate the clinicopatho-logical characteristics and differential diagnosis of PPMS. Methods: The clinical and pathological features of 6 cases of PPMS with EWSR1 gene rearrangement were reviewed. Immunohistochemistry and fluorescence in situ hybridization (FISH) study were performed. Results: Six patients were enrolled in this study, including 4 male patients and 2 female patients with an age range of 23 to 64 years (median age, 44 years). All tumors involved pulmonary parenchyma, with a predominant endobronchial component in 1 and adjacent to bronchus in 5 patients. Microscopically, the tumor was lobulated and composed of cords of polygonal, spindle cells within myxoid stroma. Tumors were immunoreactive for only Vimentin and weakly focal for epithelial membrane antigen (EMA). All tumors were shown to harbor EWSR1 gene rearrangement by FISH. Follow-up of all patients showed that 1 patient developed a pleura and bone metastasis but was still alive and 5 were disease-free after 4-29 months. Conclusion: PPMS is an extremely rare sarcoma with low-grade malignant potential. This is characterized by distinct histological features and EWSR1 gene rearrangement. It is important that understanding tumor spectrum and genetic feature can contribute to diagnosis and differential diagnosis of PPMS.

Myxoid synovial sarcoma: a report of 2 cases and literature review / 临床与实验病理学杂志

Purpose To investigate the clinicopathological features,diagnosis and differential diagnosis of myxoid synovial sarcoma (MSS).Methods Clinicopathological changes and immunophenotype were retrospectively evaluated in two MSS cases collected from Fujian Provincial Hospital,conbined with genetic mutation analysis.The relevant literatures were reviewed to explore its clinical and pathological features of this tumor.Restilts The two cases,one man and one woman,aged 71 and 15years,respectively.Tumor was located in the left down abdomen in case 1,and left frontal temporal lobe in case 2.Histopathologically,at low magnification in case 1,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In some areas of hypocellularity,the tumor cells were arranged in fascicular,story-form,sheet arrangements with mucoid degeneration.In hypercellularity area,the tumor cells were arranged in fascicular,fish bone-liked arrangenents.At low magnification in case 2,the tumor was nodular,which was made up of areas of hypercellularity and hypocellularity.In hypocellularity area,the tumor cells were arranged in net-like,sheet arrangements,and fascicular,sheet arrangements in case 2.In some area,the tumor cells were epithelioid with cluster distribution,without infringing brain tissue.Immunohistochemically,the tumor cells were diffusely positive for BCL-2,vimentin,and α-SMA and EMA were partially positive,while CD34,CD57,S-100,CD117,PLAP were negative.However,in case 2,only BCL-2 was positive,and MyoD1,GFAP,Olig-2,EMA,Syn,CD99,CgA,S-100,Myogenin,STAT6,CD34,desmin and α-SMA were negative.Molecular detection SYT-SSX fusion gene was detected in both cases.Conclusion MSS is a rare malignancy of soft tissue.The diagnosis of MSS depends on molecular pathology.The clinical and pathological findings are different from mucinous fibrosarcoma and solitary fibrous tumor.The treatment is surgical resection,combined with radiotherapy,with poor prognosis.