Uterine Inflammatory Myofibroblastic Neoplasms With Aggressive Behavior, Including an Epithelioid Inflammatory Myofibroblastic Sarcoma: A Clinicopathologic Study of 9 Cases.

The experience with uterine inflammatory myofibroblastic neoplasms with an unfavorable outcome is limited. We present the clinicopathologic features of 9 such cases, including 8 inflammatory myofibroblastic tumors (IMTs) and 1 epithelioid inflammatory myofibroblastic sarcoma (EIMS). Median patient age for the IMT group was 50.5 years; the patient with EIMS was 43 years old. Patients presented with abnormal uterine bleeding, presumed fibroids, pelvic pain, arthralgia and low-grade fever, as well as an incidental finding. Median tumor size for the IMTs was 8.5 cm. The borders were either infiltrative or well-circumscribed. Histologically, IMTs were purely fascicular or myxoid or showed predominance of one or the other pattern. Seven tumors were spindled, and 1 was both spindled and epithelioid. Tumors had variable nuclear atypia, ranging from grade 1 to 3. All tumors had an inflammatory infiltrate-predominantly lymphocytic, majority had necrosis (62.5%) and none had lymphovascular invasion. 7/8 (87.5%) tumors were positive for ALK-1 by immunohistochemistry (IHC). One tumor was negative for ALK-1 by IHC but was positive for ALK fusion by fluorescence in situ hybridization and had TNS1-ALK fusion by next-generation sequencing (NGS). Three other tumors with NGS testing showed one of the following ALK-fusion partners: FN1, DCTN1, and IGFBP5. The EIMS had infiltrative borders, myxoid and hyalinized patterns, epithelioid cells, and no lymphovascular invasion. This tumor was ALK-1 positive by IHC, had ALK rearrangement by fluorescence in situ hybridization and RANBP2-ALK fusion by NGS. Extrauterine disease at time of diagnosis was noted in 2/8 (25%) of IMTs, and in the single case of EIMS. Seven patients had surgery as primary treatment, 1 patient had neoadjuvant chemotherapy and 1 patient declined treatment. Patients with recurrence were treated with a combination of chemotherapy, targeted therapy, radiotherapy or hormonal therapy. Most patients (71.4%) recurred within 24 months (mos). Two thirds of patients were alive with disease at last follow up (mean 43.6 mos). The patient with EIMS was alive with disease at 22 mos. IMT referral cases were initially diagnosed as smooth muscle tumors in 87.5% of cases; while the EIMS was diagnosed as high-grade endometrial stromal sarcoma. Lack of consideration of IMT in the differential diagnosis of smooth muscle tumors with myxoid features can result in misdiagnosis and under-utilization of targeted therapy in these patients.

Hibernoma cardiaco embolizante y fallo respiratorio agudo / Embolizing cardiac hibernoma and acute respiratory failure

Los hibernomas cardiacos son muy infrecuentes, y generalmente son infra-diagnosticados pre-mortem debido a su similitud clínico-radiológica con otras lesiones intracardiacas ocupantes de espacio. Además, los hibernomas cardiacos pueden ser asintomáticos y pueden escapar a la detección en los estudios radiológicos. Presentamos el caso de una mujer de 81 años quien falleció como consecuencia de embolismo tumoral pulmonar. Reportamos este caso con el fin de familiarizar a residentes y patólogos con las neoplasias intracardiacas. Se discute su dificultad diagnóstica, además de la embolia tumoral pulmonar como complicación de las neoplasias intracardiacas

Not only ae cardiac hibernomas rare, ante-mortem diagnosis is poor, due to the clinical and radiological similarity with other intracardiac masses. Furthermore, cardiac hibernomas can be asymptomatic and thus escape detection with imaging studies. We present a case of an 81-year-old woman who died as a result of pulmonary tumor embolism. This unusual case highlights the necessity for clinicians and pathologists to be familiar with intra-cardiac neoplasms. We discuss its diagnostic difficulty and the complication of pulmonary tumor embolism

[Embolizing cardiac hibernoma and acute respiratory failure]. / Hibernoma cardiaco embolizante y fallo respiratorio agudo.

Not only ae cardiac hibernomas rare, ante-mortem diagnosis is poor, due to the clinical and radiological similarity with other intracardiac masses. Furthermore, cardiac hibernomas can be asymptomatic and thus escape detection with imaging studies. We present a case of an 81-year-old woman who died as a result of pulmonary tumor embolism. This unusual case highlights the necessity for clinicians and pathologists to be familiar with intra-cardiac neoplasms. We discuss its diagnostic difficulty and the complication of pulmonary tumor embolism.

Salpingitis y ooforitis xantogranulomatosa: presentación de 2 casos / Xanthogranulomatous salpingitis and oophoritis: a report of 2 cases

La salpingitis xantogranulomatosa es un proceso inflamatorio poco frecuente, caracterizado por un infiltrado de la mucosa por tejido inflamatorio de granulación conteniendo histiocitos de citoplasma espumoso. Este tipo de inflamación es excepcional a nivel del tracto genital femenino y clásicamente se ha asociado a infecciones bacterianas crónicas, inmunosupresión u otras causas de inflamación crónica. Pero también se ha visto en relación con el carcinoma endometrial, la endometriosis, cuerpos extraños y enfermedad inflamatoria pélvica crónica. El hecho de que acompañe o no a endometriosis genital ha llevado a la diferenciación de 2 tipos de entidades: la salpingitis xantogranulomatosa, sin endometriosis acompañante, y la salpingiosis pseudoxantomatosa. Presentamos 2 casos con su histología e inmunohistoquímica, y se ha revisado la literatura sobre este tipo de inflamaciones crónicas (AU)

Xanthogranulomatous salpingitis is an uncommon inflammatory process, characterized by an infiltration of the mucous by inflammatory granulation tissue with foamy histiocytes. This kind of inflammation is exceptional in the female genital tract; classically, it has been associated with chronic bacterial infections, immunosuppression and other causes of chronic inflammation. However, it has also been found associated with endometrial carcinoma, endometriosis, foreign bodies and chronic pelvic inflammatory disease. As this inflammation may or may not be accompanied by genital endometriosis, 2 entities have been differentiated: xanthogranulomatous salpingitis without endometriosis and pseudoxanthomatous salpingitis. We present 2 cases of this type of chronic inflammation, including immunohistochemistry findings, and we review the pertinent literature (AU)