Müllerian adenosarcoma with sarcomatous overgrowth and heterologous elements: A case report and literature review.

Uterine mullerian adenosarcoma (MA) is a rare biphasic tumour that accounts for less than 0.5% of uterine neoplasms. The age range of presentation is wide, with the median age in the 5th decade of life. It usually has a good prognosis; however, it worsens when it presents with sarcomatous overgrowth, heterologous elements or infiltrates the myometrium. We report the case of a 63-year-old woman presenting with abnormal vaginal bleeding and a sensation of solid material coming out of the cervical canal who was diagnosed with mullerian adenosarcoma with sarcomatous overgrowth (MASO) and presence of heterologous elements after performing a mass biopsy and subsequent hysterectomy. We reviewed the literature, focusing especially on the differential diagnoses to be evaluated, as well as the differences in prognosis and treatment according to whether or not they present histologic features of poor prognosis.

[Adenoid cystic carcinoma of the breast, 22 years of experience]. / Carcinoma adenoide quístico de mama, 22 años de experiencia.

INTRODUCTION: Adenoid cystic carcinoma (ACC) is an uncommon neoplasm in the breast (less than 0.1%). ACC is typically triple negative with a favourable prognosis. Its low frequency and indolent course cause difficulties in the agreement of the evaluation and therapeutic consensus, as well as the use of the sentinel node excision in these carcinomas. OBJECTIVE: Our goal is to provide a new series of cases from the Complejo Hospitalario de Navarra, through retrospective review over a period of 22 years. MATERIAL AND METHODS: Every case of breast ACC diagnosed between 1998-2020 is reviewed. We analyze the clinical and pathological characteristics, and compare them with the literature. RESULTS: We identified twelve cases of ACC, from a total of 18,241 patients diagnosed with breast carcinoma, with an average tumor size of 23.8mm and mean of 63.5 years. All of them were in a stage I-II at diagnosis. One case presented a lymph node micrometastases. One of them presented local recurrences and one case presented metastases 5 months after diagnosis (93.5 months of mean follow-up). According to Nottingham Histological Score, eight cases had a grade 1, two grade 2 and two grade 3. According to the classification of Ro et al., three were grade 1, four grade 2 and five grade 3. One case was ER positive (10%). No case presents positivity for BRAF-V600E in immunohistochemistry. CONCLUSION: The management and prognosis ACC of breast, within benignity, remains uncertain, with more studies being needed to understand the clinical evolution and perform adequate therapeutic management.

Carcinoma adenoide quístico de mama, 22 años de experiencia / Adenoid cystic carcinoma of the breast, 22 years of experience

INTRODUCCIÓN: El carcinoma adenoide quístico (CAQ) es una neoplasia infrecuente en la mama (menos del 0,1%). Típicamente es triple negativo con un pronóstico favorable. Su baja frecuencia y su curso indolente provocan dificultades en el convenio de la evaluación y el consenso terapéutico, así como en el uso de la técnica del ganglio centinela en estos carcinomas. OBJETIVO: Aportar una serie nueva de casos procedentes del Complejo Hospitalario de Navarra, mediante la revisión retrospectiva en un periodo de 22 años. MATERIAL Y MÉTODOS: Se revisan todos los casos de CAQ mamario diagnosticados entre 1998 y 2020. Analizamos los datos clínicos y anatomopatológicos y los comparamos con la literatura. RESULTADOS: Identificamos 12 CAQ, de un total de 18.241 pacientes con diagnóstico de carcinoma de mama, con un tamaño tumoral medio de 23,8mm y una edad media de 63,5 años. Todos ellos se encontraban en un estadio I-II al diagnóstico. Un caso presentó una micrometástasis ganglionar. Un caso presentó recidiva local y un caso presentó metástasis a distancia a los 5 meses del diagnóstico (93,5 meses de seguimiento medio). Según el grado histológico de Nottingham, 8 casos tuvieron un grado 1, 2 casos un grado 2 y 2 pacientes un grado 3. Según la clasificación de Ro et al., 3 casos fueron grado 1, 4 casos grado 2 y 5 casos grado 3. Un caso fue RE positivo (10%). Ningún caso presentó positividad para BRAF V600E con inmunohistoquímica. CONCLUSIÓN: El manejo y pronóstico del CAQ mamario, dentro de la benignidad, sigue siendo incierto, siendo necesarios más datos para comprender la evolución clínica y realizar un adecuado manejo terapéutico

INTRODUCTION: Adenoid cystic carcinoma (ACC) is an uncommon neoplasm in the breast (less than 0.1%). ACC is typically triple negative with a favourable prognosis. Its low frequency and indolent course cause difficulties in the agreement of the evaluation and therapeutic consensus, as well as the use of the sentinel node excision in these carcinomas. OBJECTIVE: Our goal is to provide a new series of cases from the Complejo Hospitalario de Navarra, through retrospective review over a period of 22 years. MATERIAL AND METHODS: Every case of breast ACC diagnosed between 1998-2020 is reviewed. We analyze the clinical and pathological characteristics, and compare them with the literature. RESULTS: We identified twelve cases of ACC, from a total of 18,241 patients diagnosed with breast carcinoma, with an average tumor size of 23.8mm and mean of 63.5 years. All of them were in a stage I-II at diagnosis. One case presented a lymph node micrometastases. One of them presented local recurrences and one case presented metastases 5 months after diagnosis (93.5 months of mean follow-up). According to Nottingham Histological Score, eight cases had a grade 1, two grade 2 and two grade 3. According to the classification of Ro et al., three were grade 1, four grade 2 and five grade 3. One case was ER positive (10%). No case presents positivity for BRAF-V600E in immunohistochemistry. CONCLUSION: The management and prognosis ACC of breast, within benignity, remains uncertain, with more studies being needed to understand the clinical evolution and perform adequate therapeutic management