Verónica García Yllán, María de Jesús Checa Peña, Severino Rey Nodar
La fibromatosis ligamentoide (fibromatosis invasiva, fibroma desmoide) es una neoplasia mesenquimatosa, intermedia entre los tumores benignos y malignos, de origen fibroso, que de manera infrecuente puede afectar el mesenterio. Se presenta el caso de un paciente masculino con tumoración fibrosa intraabdominal a nivel de raíz de mesenterio. Se realiza una revisión de la literatura.
{"title":"Fibromatosis ligamentoide mesentérica","authors":"Verónica García Yllán, María de Jesús Checa Peña, Severino Rey Nodar","doi":"10.47579/ap.v3.i2.0091","DOIUrl":"https://doi.org/10.47579/ap.v3.i2.0091","url":null,"abstract":"La fibromatosis ligamentoide (fibromatosis invasiva, fibroma desmoide) es una neoplasia mesenquimatosa, intermedia entre los tumores benignos y malignos, de origen fibroso, que de manera infrecuente puede afectar el mesenterio. Se presenta el caso de un paciente masculino con tumoración fibrosa intraabdominal a nivel de raíz de mesenterio. Se realiza una revisión de la literatura.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"74012588","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A. Pérez López, María Victoria López Soto, Reynaldo Álvarez Santana
La linfadenopatía por silicona es una rara complicación en pacientes con implantes mamarios, a menudo es confundida con tumores primarios o secundarios. Se trata de una paciente femenina de 42 años, con implantes mamarios desde hace 12 años, que consulta por presentar dolor y aumento de volumen axilar bilateral. Se realizó ultrasonido, mamografía y punción aspiración con aguja fina, donde se apreciaron: histiocitos y células gigantes fagocitando las partículas de silicona, entremezclada con linfocitos. Hicieron explante de las prótesis y vaciamiento axilar del primer nivel bilateral. El caso reportado evidencia la utilidad de la punción aspiración con aguja fina como herramienta para el diagnóstico diferencial de adenopatías axilares en pacientes con prótesis mamarias.
{"title":"Linfadenitis axilar por silicona. Presentación de un caso diagnosticado por punción aspiración con aguja fina","authors":"A. Pérez López, María Victoria López Soto, Reynaldo Álvarez Santana","doi":"10.47579/ap.v3.i2.0094","DOIUrl":"https://doi.org/10.47579/ap.v3.i2.0094","url":null,"abstract":"La linfadenopatía por silicona es una rara complicación en pacientes con implantes mamarios, a menudo es confundida con tumores primarios o secundarios. Se trata de una paciente femenina de 42 años, con implantes mamarios desde hace 12 años, que consulta por presentar dolor y aumento de volumen axilar bilateral. Se realizó ultrasonido, mamografía y punción aspiración con aguja fina, donde se apreciaron: histiocitos y células gigantes fagocitando las partículas de silicona, entremezclada con linfocitos. Hicieron explante de las prótesis y vaciamiento axilar del primer nivel bilateral. El caso reportado evidencia la utilidad de la punción aspiración con aguja fina como herramienta para el diagnóstico diferencial de adenopatías axilares en pacientes con prótesis mamarias.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"52 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77032353","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
El adenocarcinoma de células caliciformes o goblet cell carcinoma, es una neoplasia poco frecuente, reconocida por la OMS, que característicamente presenta componente mixto: exocrino y endocrino. Es un tumor casi exclusivo del apéndice cecal, pero se han reportado situaciones en las que surge en otros segmentos del tubo digestivo. Se presenta el caso de un hombre de 86 años con adenocarcinoma de células caliciformes extrapendicular en colon derecho. El estudio morfológico del apéndice cecal no demostró neoplasia. Se revisa la literatura referente a esta infrecuente neoplasia.
{"title":"Inusual localización de un tumor inusual. Comunicación breve de un caso","authors":"J. Lagos, Adoni Duarte","doi":"10.47579/ap.v3.i2.0093","DOIUrl":"https://doi.org/10.47579/ap.v3.i2.0093","url":null,"abstract":"El adenocarcinoma de células caliciformes o goblet cell carcinoma, es una neoplasia poco frecuente, reconocida por la OMS, que característicamente presenta componente mixto: exocrino y endocrino. Es un tumor casi exclusivo del apéndice cecal, pero se han reportado situaciones en las que surge en otros segmentos del tubo digestivo. Se presenta el caso de un hombre de 86 años con adenocarcinoma de células caliciformes extrapendicular en colon derecho. El estudio morfológico del apéndice cecal no demostró neoplasia. Se revisa la literatura referente a esta infrecuente neoplasia.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"103 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-10-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"79524520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Fermín Lobo, Haimar Hernández Jiménez, Severino Rey
La displasia alveolo-capilar con mal alineamiento de venas pulmonares es una malformación congénita letal en la mayoría de los casos que consiste en el desarrollo vascular anómalo de los pulmones asociado a hipertensión pulmonar severa del recién nacido y desarrollo de insuficiencia cardíaca fatal. Recientemente se han descrito casos de afectación localizada en uno o ambos pulmones asociados a supervivencia a largo plazo en algunos pacientes. Se reporta el caso de un recién nacido y se describen los hallazgos histopatológicos encontrados en la biopsia pulmonar realizada.
{"title":"Displasia alveolo-capilar con mal alineamiento de las venas pulmonares. Reporte de un caso sobreviviente","authors":"Fermín Lobo, Haimar Hernández Jiménez, Severino Rey","doi":"10.47579/ap.v3.i1.0084","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0084","url":null,"abstract":"La displasia alveolo-capilar con mal alineamiento de venas pulmonares es una malformación congénita letal en la mayoría de los casos que consiste en el desarrollo vascular anómalo de los pulmones asociado a hipertensión pulmonar severa del recién nacido y desarrollo de insuficiencia cardíaca fatal. Recientemente se han descrito casos de afectación localizada en uno o ambos pulmones asociados a supervivencia a largo plazo en algunos pacientes. Se reporta el caso de un recién nacido y se describen los hallazgos histopatológicos encontrados en la biopsia pulmonar realizada.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"8 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84515560","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
BACKGROUND: Primary cardiac tumors are extremely rare and encompass less than 1% of all documented cardiac neoplasms with a majority of these being benign. Among the malignant primary cardiac tumors, leiomyosarcoma accounts for only 1% all documented cases and it commonly involves the left atrium. We present a rare case of a primary cardiac leiomyosarcoma originating from the right atrium of a 25-year-old male.CASE: The patient was a 25-year-old Filipino male who presented with progressive dyspnea, orthopnea, and a large, heterogenous, ovoid mass attached at the atrial septum measuring 7.0 cm x 3.16cm in widest dimension on 2D-echocardiography. The patient eventually underwent emergency excision of the right atrial mass. Post-operatively, the patient did not tolerate the procedure and ultimately expired after attempts of cardiopulmonary resuscitation.HISTOPATHOLOGIC FINDINGS: Microscopic sections show a spindle-cell neoplasm with irregular fascicles and whorled configurations interspersed with inflammatory cells in a fibromyxoid stroma. The spindle cells showed pleomorphic, hyperchromatic nuclei, some with prominent nucleoli and eosinophilic cytoplasm. Mitotic figures, areas of necrosis, and hemorrhages are seen. Immunohistochemical studies show reactivity of the neoplastic cells to Smooth Muscle Actin (SMA) and Vimentin. Masson’s Trichrome Stain is also positive. S100, Myogenin, and Desmin show non-reactivity. Proliferation index is at 15-20% using Ki-67 and p53.CONCLUSION: Primary cardiac leiomyosarcoma is an extremely rare tumor occurring in less than 1% of all documented primary malignant cardiac neoplasms. Metastases originating from other sites are more common. It has an aggressive clinical course and a dismal prognosis with features overlapping that of other soft tissue neoplasms. Hence, additional immunohistochemical studies and special stains should be employed to arrive with a definitive diagnosis so that appropriate management and intervention may be offered to affected patients.
背景:原发性心脏肿瘤极为罕见，占所有记录在案的心脏肿瘤的不到1%，其中大多数是良性的。在原发性恶性心脏肿瘤中，平滑肌肉瘤仅占所有文献病例的1%，它通常累及左心房。我们报告一例罕见的原发性心脏平滑肌肉瘤起源于右心房的25岁男性。病例:患者是一名25岁的菲律宾男性，在2d超声心动图上表现为进行性呼吸困难，直立呼吸，房间隔处有一个大的，异质的卵形肿块，最宽尺寸为7.0 cm x 3.16cm。患者最终接受了右心房肿块的紧急切除。术后，患者不能耐受手术，最终在心肺复苏后死亡。组织病理表现:纤维黏液样间质中，显微镜切片显示一个纺锤状细胞肿瘤，具有不规则的束状和轮状结构，其间散布着炎症细胞。梭形细胞呈多形性，细胞核深染，部分核仁突出，胞浆嗜酸性。可见有丝分裂象、坏死区域和出血。免疫组化研究显示肿瘤细胞对平滑肌肌动蛋白(SMA)和Vimentin具有反应性。马森氏三色染色也是阳性的。S100、Myogenin和Desmin无反应。Ki-67和p53的增殖指数为15-20%。结论:原发性心脏平滑肌肉瘤是一种极为罕见的肿瘤，在所有记录的原发性心脏恶性肿瘤中发生率不到1%。从其他部位转移更为常见。它具有侵袭性临床病程和预后差，其特征与其他软组织肿瘤重叠。因此，应采用额外的免疫组织化学研究和特殊染色来获得明确的诊断，以便为受影响的患者提供适当的管理和干预。
{"title":"Incidental finding of a primary cardiac leiomyosarcoma originating from the right atrium of a 25-year old male. A Case Report","authors":"Anna Katrina M. Donato, Felipe, Jr. S. Templo","doi":"10.47579/ap.v3.i1.0085","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0085","url":null,"abstract":"BACKGROUND: Primary cardiac tumors are extremely rare and encompass less than 1% of all documented cardiac neoplasms with a majority of these being benign. Among the malignant primary cardiac tumors, leiomyosarcoma accounts for only 1% all documented cases and it commonly involves the left atrium. We present a rare case of a primary cardiac leiomyosarcoma originating from the right atrium of a 25-year-old male.CASE: The patient was a 25-year-old Filipino male who presented with progressive dyspnea, orthopnea, and a large, heterogenous, ovoid mass attached at the atrial septum measuring 7.0 cm x 3.16cm in widest dimension on 2D-echocardiography. The patient eventually underwent emergency excision of the right atrial mass. Post-operatively, the patient did not tolerate the procedure and ultimately expired after attempts of cardiopulmonary resuscitation.HISTOPATHOLOGIC FINDINGS: Microscopic sections show a spindle-cell neoplasm with irregular fascicles and whorled configurations interspersed with inflammatory cells in a fibromyxoid stroma. The spindle cells showed pleomorphic, hyperchromatic nuclei, some with prominent nucleoli and eosinophilic cytoplasm. Mitotic figures, areas of necrosis, and hemorrhages are seen. Immunohistochemical studies show reactivity of the neoplastic cells to Smooth Muscle Actin (SMA) and Vimentin. Masson’s Trichrome Stain is also positive. S100, Myogenin, and Desmin show non-reactivity. Proliferation index is at 15-20% using Ki-67 and p53.CONCLUSION: Primary cardiac leiomyosarcoma is an extremely rare tumor occurring in less than 1% of all documented primary malignant cardiac neoplasms. Metastases originating from other sites are more common. It has an aggressive clinical course and a dismal prognosis with features overlapping that of other soft tissue neoplasms. Hence, additional immunohistochemical studies and special stains should be employed to arrive with a definitive diagnosis so that appropriate management and intervention may be offered to affected patients.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"169 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78704787","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
The history of discovery of the cardiovascular system (CVS) is a fascinating story full of twists, wrong turns and insightful revelations, contributed to by philosophers and physicians the world over. However, whenever this history is discussed, the contributions described in Ayurveda, the traditional healthcare discipline of India, are overlooked. Ayurveda is one of the oldest traditional healthcare systems in the world with sound theoretical and practical foundations. The ancient masters of Ayurveda discoursed prolifically on all aspects of medicine and surgery, including the structure and function of the heart and circulatory system. In this short article, we discuss the history of discovery of various aspects of the CVS, highlighting the many contributions of the Ayurvedic School of Medicine.
{"title":"From the Hridaya to the heart. Contributions by the ancient Indian masters of Ayurveda to the discovery of the cardiovascular system","authors":"Aditya D. Kulkarni, Singaravel Saranya","doi":"10.47579/ap.v3.i1.0083","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0083","url":null,"abstract":"The history of discovery of the cardiovascular system (CVS) is a fascinating story full of twists, wrong turns and insightful revelations, contributed to by philosophers and physicians the world over. However, whenever this history is discussed, the contributions described in Ayurveda, the traditional healthcare discipline of India, are overlooked.\u0000Ayurveda is one of the oldest traditional healthcare systems in the world with sound theoretical and practical foundations. The ancient masters of Ayurveda discoursed prolifically on all aspects of medicine and surgery, including the structure and function of the heart and circulatory system.\u0000In this short article, we discuss the history of discovery of various aspects of the CVS, highlighting the many contributions of the Ayurvedic School of Medicine.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90620917","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Manuel. F Molina Centelles, Liliana F. Castillo Paredes, Judith Pérez Rojas
Las lesiones pseudotumorales biliopancreáticas son lesiones infrecuentes formadoras de masas que pueden conllevar una clínica obstructiva y simular una neoplasia. El diagnóstico definitivo se realiza tras el estudio anatomopatológico, generalmente de la pieza quirúrgica. La etiología más frecuente es la inflamatoria autoinmune, encontrándose también lesiones sólido-quísticas. En este sentido, describimos tres casos clínico-patológicos con un diagnóstico de sospecha de adenocarcinoma en los que el estudio anatomopatológico informó tres patologías benignas pancreáticas. El objetivo es contribuir a su evidencia científica y el correcto diagnóstico, evaluación y seguimiento de las patologías descritas a pesar de su infrecuencia.
{"title":"Tres escenarios diferentes de lesiones pseudotumorales biliopancreáticas","authors":"Manuel. F Molina Centelles, Liliana F. Castillo Paredes, Judith Pérez Rojas","doi":"10.47579/ap.v3.i1.0088","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0088","url":null,"abstract":"Las lesiones pseudotumorales biliopancreáticas son lesiones infrecuentes formadoras de masas que pueden conllevar una clínica obstructiva y simular una neoplasia. El diagnóstico definitivo se realiza tras el estudio anatomopatológico, generalmente de la pieza quirúrgica. La etiología más frecuente es la inflamatoria autoinmune, encontrándose también lesiones sólido-quísticas. En este sentido, describimos tres casos clínico-patológicos con un diagnóstico de sospecha de adenocarcinoma en los que el estudio anatomopatológico informó tres patologías benignas pancreáticas. El objetivo es contribuir a su evidencia científica y el correcto diagnóstico, evaluación y seguimiento de las patologías descritas a pesar de su infrecuencia.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"23 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"72398140","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aneurysms of the membranous septum are extremely rare malformations that are usually asymptomatic. However, they have the potential for varied complications including sudden cardiac death. Reported here is a case of sudden death in a 25-year-old woman. A medico-legal autopsy revealed a large aneurysm of the membranous septum. It is speculated that the infiltration of fibrous tissue from the aneurysm into the atrioventricular conduction axis may have led to the observed mortality.
{"title":"Aneurysm of the membranous septum. A potential cause of sudden death","authors":"R. Kothari, Pranita Zare, P. Vaideeswar","doi":"10.47579/ap.v3.i1.0082","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0082","url":null,"abstract":"Aneurysms of the membranous septum are extremely rare malformations that are usually asymptomatic. However, they have the potential for varied complications including sudden cardiac death. Reported here is a case of sudden death in a 25-year-old woman. A medico-legal autopsy revealed a large aneurysm of the membranous septum. It is speculated that the infiltration of fibrous tissue from the aneurysm into the atrioventricular conduction axis may have led to the observed mortality.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"1 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75526161","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Severino Rey, Juan Esteban Huerta Brunel, Inmaculada Concepción Pérez Caballero, C. Cárdenas
Las neoplasias torácicas pueden ser benignas y malignas, y afectar a cada uno de los componentes del tórax, siendo las más frecuentes las metástasis derivadas de las propias neoplasias primarias y de las neoplasias extratorácicas. Las imágenes constituyen un aspecto integral en la evaluación de pacientes con neoplasias en el tórax que por lo general provocan quejas indolentes o inespecíficas. La evaluación comienza con una radiografía de tórax y puede ser complementada por técnicas de imágenes más avanzadas con tomografía computarizada, resonancia magnética y Tomografía por Emisión de Positrones con Fluorodesoxiglucosa. El radiólogo es responsable de distinguir los procesos neoplásicos de las complicaciones asociadas y secundarias al tratamiento. El análisis radiográfico de las anomalías torácicas constituye un desafío dada la superposición de numerosas estructuras anatómicas de diversas morfologías y densidades radiológicas.
{"title":"Características radiológicas de las lesiones del tórax","authors":"Severino Rey, Juan Esteban Huerta Brunel, Inmaculada Concepción Pérez Caballero, C. Cárdenas","doi":"10.47579/ap.v3.i1.0087","DOIUrl":"https://doi.org/10.47579/ap.v3.i1.0087","url":null,"abstract":"Las neoplasias torácicas pueden ser benignas y malignas, y afectar a cada uno de los componentes del tórax, siendo las más frecuentes las metástasis derivadas de las propias neoplasias primarias y de las neoplasias extratorácicas. Las imágenes constituyen un aspecto integral en la evaluación de pacientes con neoplasias en el tórax que por lo general provocan quejas indolentes o inespecíficas. La evaluación comienza con una radiografía de tórax y puede ser complementada por técnicas de imágenes más avanzadas con tomografía computarizada, resonancia magnética y Tomografía por Emisión de Positrones con Fluorodesoxiglucosa. El radiólogo es responsable de distinguir los procesos neoplásicos de las complicaciones asociadas y secundarias al tratamiento. El análisis radiográfico de las anomalías torácicas constituye un desafío dada la superposición de numerosas estructuras anatómicas de diversas morfologías y densidades radiológicas.","PeriodicalId":87886,"journal":{"name":"Archivos peruanos de patologia y clinica","volume":"19 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2022-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"78309784","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Glenn Nathaniel S.D. Valloso, F. Templo, A. D. de Luna
Myofibroblastic sarcoma (MS) is a rare malignant tumor of myofibroblast. Myofibroblasts were primarily described by Gabbiani et al. by means of electron microscopy. These spindle cells have features common with smooth muscle cells and fibroblast. Vasudev et al. first reported the pathologic characteristics of MS in 1978. Mentzel et al. coined the term “low-grade MS” after immunohistochemical and ultrastructural profiling in a series of cases. This study was undertaken to analyze the clinicopathologic, histopathologic, and immunohistochemical characteristics of MS cases arising in the heart. This is a descriptive study performed at the Philippine Heart Center. All twelve cases diagnosed as cardiac MS and other cardiac spindle cell tumors included in the Philippine Heart Center Pathology Cardiac Registry from 2000 to 2018 were reviewed. Six of the twelve cases were later proven or reclassified as MS. The six patients with MS were composed of three males (50%) and three females (50%) with a mean age of 57.5 and SD of 9.91. Four of the cases were from the left atrium (68%), one was from the right atrium (16%), and one was biatrial (16%). The tumor size ranged from 3.0 to 8.7 cm with a mean size of 5.57 cm and SD of 1.60. All six cases were treated surgically; four by excision (68%), one by debulking (16%), and one by both excision and debulking (16%). Histologically, the six tumors were predominantly composed of pleomorphic to monomorphic spindle cells arranged in syncytium, storiform, fascicles, and dense cellular sheets, some are intersecting at angles. The individual tumor cells are spindled with elongated nuclei with blunted ends, granular to open chromatin pattern, inconspicuous nucleoli, and scanty to moderate amount of eosinophilic to amphophilic cytoplasm. Cytoplasmic membrane are indistinct. Lymphoplasmacytic infiltrates are present with a mean of 28.67 per 10 high power fields, range of 7-91, and SD of 33.07. Mitosis are present with a mean of 12.17 per 10 high power fields, range of 3-32, and SD of 10.93. Necrosis of less than 50% is present in 4 of 6 cases (67%). Two cases (33%) showed no necrosis. Trichrome stain showed collagen deposits in five of six cases (83%). Immunohistochemically, all six cases of MS showed reactivity for vimentin and smooth muscle actin (100%). Three of six cases were reactive to calponin (50%). All six cases show non-reactivity against h-caldesmon (0%). Two of the six cases were reactive with desmin (33%). Two of the six cases were reactive with CD99 (33%). Only one of six cases were reactive to pancytokeratin (17%). No significant differences between proliferation indices using Ki-67 and p53 were observed. The application of the panel of immunohistochemical stains can aid in the diagnosis of MS in particular h-caldesmon which when negative rules-out smooth muscle tumors. MS consistently expresses vimentin and smooth muscle actin. Expression of calponin, desmin, and CD99 is variable. Prognostic value of proli
肌成纤维细胞肉瘤(MS)是一种罕见的肌成纤维细胞恶性肿瘤。肌成纤维细胞最初是由Gabbiani等人通过电子显微镜描述的。梭形细胞具有平滑肌细胞和成纤维细胞的共同特征。Vasudev等人于1978年首次报道了多发性硬化症的病理特征。Mentzel等人在对一系列病例进行免疫组织化学和超微结构分析后，创造了“低级别MS”一词。本研究旨在分析发生于心脏的多发性硬化症的临床病理、组织病理和免疫组织化学特征。这是在菲律宾心脏中心进行的一项描述性研究。我们回顾了菲律宾心脏中心病理心脏登记处2000年至2018年诊断为心脏MS和其他心脏梭形细胞肿瘤的所有12例病例。12例中有6例后来被证实或重新分类为多发性硬化症，6例多发性硬化症患者男3例(50%)，女3例(50%)，平均年龄57.5岁，SD为9.91。左心房4例(68%)，右心房1例(16%)，双心房1例(16%)。肿瘤大小3.0 ~ 8.7 cm，平均5.57 cm, SD为1.60。6例均行手术治疗;4例切除(68%)，1例减体积(16%)，1例切除和减体积(16%)。组织学上，6例肿瘤主要由多形性至单形性梭形细胞组成，排列成合胞体、故事状、束状和致密的细胞片，部分呈一定角度相交。单个肿瘤细胞呈纺锤形，细胞核细长，末端钝化，颗粒状至开放的染色质模式，核仁不明显，嗜酸性至嗜两性细胞质稀少至中等。细胞质膜不清。淋巴浆细胞浸润存在，每10个高倍视野平均28.67个，范围7-91,SD 33.07。有丝分裂存在，每10个高倍场平均有12.17个，范围3-32,SD为10.93。6例中有4例(67%)坏死小于50%。2例(33%)未见坏死。三色染色显示6例中有5例(83%)胶原沉积。免疫组化结果显示，6例MS均对vimentin和平滑肌肌动蛋白具有反应性(100%)。6例中有3例对钙钙蛋白有反应(50%)。所有6例对h-caldesmon均无反应(0%)。6例中2例对desmin有反应(33%)。6例中有2例CD99反应性(33%)。6例中只有1例对全细胞角蛋白有反应(17%)。Ki-67与p53的增殖指标差异无统计学意义。免疫组化染色组的应用有助于MS的诊断，特别是h-caldesmon，当阴性时可排除平滑肌肿瘤。MS一致表达波形蛋白和平滑肌肌动蛋白。calponin, desmin和CD99的表达是可变的。以Ki-67和p53为预后指标的增殖指标的预后价值尚未得到证实。
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