Testicular plasmacytoma as presentation of multiple myeloma: case report and review of the literature.

OBJECTIVE: We present the case of a patient with testicular plasmacytoma as initial presentation of multiple myeloma, and we carry out a literature review of this uncommon pathology. METHODS: 63 year-old male who consulted for a testicular mass for three months. After clinical and diagnostic studies he underwent radical orchiectomy. RESULTS: Pathologic study of the specimen revealed the presence of round cells, some with plasmocytic aspect. Immunohistochemical studies gave the final diagnosis of plasmacytoma. Studies on disease extension showed rounded lytic lesions spread over the vault of the skull bones. Bone marrow studies, as well as bone biopsy showed infiltration by plasma cell neoplasia in more than 90%, consistent with the diagnosis of multiple myeloma. The patient received treatment, developing disease progression and subsequently died from the disease. CONCLUSIONS: Solitary plasmacytoma represents only 6% of all plasma cell neoplasms. Testicular presentation is an unusual event, representing 2% of cases. Although this is usually an autopsy finding, it may constitute the first manifestation of multiple myeloma or exceptionally be the unique location of a plasma cell neoplasm. To date there are few reports published in the literature. This case constitutes a contribution for the knowledge of testicular plasmacytoma.

Plasmocitoma testicular como presentación inicial de mieloma multiple: presentación de un caso y revisión de la literatura / Testicular plasmacytoma as presentation of multiple myeloma: case report and review of the literature

OBJETIVO: Presentamos el caso de un paciente con diagnóstico de plasmocitoma testicular como presentación inicial de mieloma múltiple y realizamos la revisión de la literatura en relación a lo infrecuente de dicha patología. MÉTODOS: Paciente de sexo masculino de 63 años portador de un tumor testicular, de 3 meses de evolución, de crecimiento progresivo. Una vez evaluado clínicamente y con estudios de ayuda diagnóstica fue sometido a orquiectomía radical. RESULTADO: La anatomía patológica reveló la presencia de células redondas, algunas de aspecto plasmocítico; el estudio inmunohistoquímico concluyó que era Plasmocitoma. Estudios de extensión de enfermedad mostraron lesiones líticas redondeadas diseminadas en los huesos de la bóveda del cráneo. Los estudios de médula ósea, así como la biopsia de hueso demostraron infiltración por neoplasia de células plasmáticas en más del 90%, consistente con el diagnóstico de Mieloma Múltiple. Paciente recibe el tratamiento respectivo, presentando progresión de enfermedad y posteriormente fallece. CONCLUSIONES: El plasmocitoma solitario es una lesión poco frecuente que representa sólo el 6% de todas las neoplasias de células plasmáticas. El compromiso testicular por esta enfermedad constituye un evento inusual, estimado en el 2% de los casos. Aunque éste es generalmente un hallazgo de autopsia, carente de expresión clínica, en raras ocasiones, como en el presente caso, puede constituir la primera manifestación de un mieloma múltiple o excepcionalmente ser la única localización de una neoplasia de células plasmáticas. Hasta la fecha son pocos los reportes publicados en la literatura y éste constituye un aporte más al conocimiento del mismo(AU)

OBJECTIVE: We present the case of a patient with testicular plasmacytoma as initial presentation of multiple myeloma, and we carry out a literature review of this uncommon pathology. METHODS: 63 year-old male who consulted for a testicular mass for three months. After clinical and diagnostic studies he underwent radical orchiectomy. RESULTS: Pathologic study of the specimen revealed the presence of round cells, some with plasmocytic aspect. Immunohistochemical studies gave the final diagnosis of plasmacytoma. Studies on disease extension showed rounded lytic lesions spread over the vault of the skull bones. Bone marrow studies, as well as bone biopsy showed infiltration by plasma cell neoplasia in more than 90%, consistent with the diagnosis of multiple myeloma.The patient received treatment, developing disease progression and subsequently died from the disease. CONCLUSIONS: Solitary plasmacytoma represents only 6% of all plasma cell neoplasms. Testicular presentation is an unusual event, representing 2% of cases. Although this is usually an autopsy finding, it may constitute the first manifestation of multiple myeloma or exceptionally be the unique location of a plasma cell neoplasm. To date there are few reports published in the literature. This case constitutes a contribution for the knowledge of testicular plasmacytoma(AU)

Epithelioid angiomyolipoma: a rare variant of renal angiomyolipoma.

OBJECTIVE: We present a case of primary renal epithelioid angiomyolipoma, its association with tuberous sclerosis and review the literature. METHODS: We present the case of a 12 year-old male with past medical history of tuberous sclerosis, characterized by developmental delay, tonic and clonic seizures, and cutaneous abnormalities. He presented with macroscopic hematuria and abdominal pain. CT scan of the abdomen showed the presence of a left renal tumor. He underwent left radical nephrectomy. Pathologic study of the specimen showed primary renal epithelioid angiomyolipoma, corroborated by immunohistochemistry staining. Review of the literature was performed for this rare variant and its malignant potential. RESULTS: The presence of this epithelial variant is rare and must be taken into account because of its malignant potential and, thus, with different prognosis and follow up, compared to classical angiomyolipoma. CONCLUSIONS: Renal angiomyolipoma is an uncommon benign tumor, representing a challenge for clinical and pathological diagnosis. Despite the big size they can reach, as well as bilaterality, multiplicity of lesions and/or lymphatic regional involvement, its malignant potential has not been established. Nevertheless, the epithelioid variant has been described recently, a rare entity with aggressive behavior, difficult histological characterization and poor prognosis.

Angiomiolipoma epiteloide: una variante del angiomiolipoma renal / Epithelioid angiomyolipoma: a rare variant of renal angiomyolipoma

OBJETIVO: Presentar un caso de angiomiolipoma (AML) variante epitelioide, primario renal, su asociación con Esclerosis Tuberosa (ET) y revisión de la literatura.MÉTODOS: Presentamos el caso de un paciente varón de 12 años con antecedente de retardo en el desarrollo psicomotor, crisis epilépticas tónico clónicas y estigmas cutáneos, todo esto compatible con ET. Debuta con hematuria macroscópica y dolor abdominal, encontrándose en la tomografía tumor que compromete dos tercios superiores del riñón izquierdo. Fue sometido a nefrectomía radical izquierda. La anatomía patológica, corroborada con estudios de inmunohistoquímica informó la presencia de AML, variante epiteliode.Se reviso la bibliográfica existente sobre esta variante poco común y su comportamiento maligno.RESULTADOS: La presencia de la variante epitelial es poco frecuente pero debe tenerse en cuenta por su comportamiento maligno y por lo tanto diferente pronóstico y seguimiento comparado al AML clásico.CONCLUSIONES: El AML renal es un tumor benigno, poco común, que representa un reto para el diagnóstico clínico e histopatológico. A pesar del gran tamaño que puede alcanzar, la bilateralidad, la multiplicidad de las lesiones y/o el compromiso linfático regional, no se ha demostrado su potencial maligno. Sin embargo, en los últimos años se ha descrito la variante epitelioide, entidad rara de comportamiento agresivo, difícil caracterización histológica y pobre pronóstico(AU)

OBJECTIVE: We present a case of primary renal epithelioid angiomyolipoma, its association with tuberous sclerosis and review the literature.METHODS: We present the case of a 12 year-old male with past medical history of tuberous sclerosis, characterized by developmental delay, tonic and clonic seizures, and cutaneous abnormalities. He presented with macroscopic hematuria and abdominal pain. CT scan of the abdomen showed the presence of a left renal tumor. He underwent left radical nephrectomy. Pathologic study of the specimen showed primary renal epithelioid angiomyolipoma, corroborated by immunohistochemistry staining. Review of the literature was performed for this rare variant and its malignant potential.RESULTS: The presence of this epithelial variant is rare and must be taken into account because of its malignant potential and, thus, with different prognosis and follow up, compared to classical angiomyolipoma.CONCLUSIONS: Renal angiomyolipoma is an uncommon benign tumor, representing a challenge for clinical and pathological diagnosis. Despite the big size they can reach, as well as bilaterality, multiplicity of lesions and/or lymphatic regional involvement, its malignant potential has not been established. Nevertheless, the epithelioid variant has been described recently, a rare entity with aggressive behavior, difficult histological characterization and poor prognosis(AU)

Micetomas, presentación de dos casos con estudio clínico y anátomo-patológico / Mycetomas, report of two cass with clinical and pathologic study

Se presentan dos casos de micetoma de miembro inferior, enfermedad de baja prevalencia en nuestro país, cuyo reconocimiento y definición etiológica tiene implicancias terapéuticas decisivas.