Mature Cystic Teratoma of the Lung With a Somatic-Like Malignant Component in a Patient With Testicular Germ-Cell Tumor / Teratoma quístico maduro de pulmón con componente maligno de tipo somático en un paciente con un tumor testicular de células germinales

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[Nested variant Transitional Cell Carcinoma. Report of two cases and bibliographic review.] / El carcinoma urotelial variante en nidos(NESTED). Presentación de dos casos y revisión de la literatura.

OBJECTIVE: Nested type transitional cell carcinoma of the bladder is a rare histological variant among bladder tumors. Although clinical presentation is similar to the other bladder tumors, its macroscopic appearance may be equivocally benign, with submucosal growing which is difficult to detect on cystoscopy, so that diagnosis may be delayed. METHODS: We present the characteristics of nested type transitional cell carcinoma and review the differential diagnosis for this entity with possible counterfeiters. RESULTS: In this article, we report two cases of nested type transitional cell carcinoma that presents, in one of them, all three growing patterns. CONCLUSIONS: Microscopically nested transitional cell carcinoma is characterized by a cell distribution forming nests and tubules. They generally present low cytologic atypia simulating a low grade urothelial carcinoma, or benign bladder lesions such as von Brunn nests or nefrogenic adenoma.

El carcinoma urotelial variante en nidos (NESTED). Presentación de dos casos y revisión de la literatura / Nested variant Transitional Cell Carcinoma. Report of two cases and bibliographic review

OBJETIVO: El carcinoma urotelial de vejiga tipo "nested" o en nidos es una variante histológica rara dentro de los tumores uroteliales de vejiga. Aunque las manifestaciones clínicas son semejantes a los demás tumores de vejiga, su apariencia macroscópica puede ser equívocamente benigna, con crecimiento submucoso difícil de detectar en la cistoscopia, lo que puede retrasar el diagnóstico. MÉTODO: presentamos las características del carcinoma urotelial variante tipo nested y revisamos los diagnósticos diferenciales de esta entidad con sus posibles imitadores. RESULTADO: En este trabajo presentamos dos casos de carcinoma urotelial tipo nested que presentan, en uno de los casos, los tres patrones de crecimiento. CONCLUSIONES: Microscópicamente el carcinoma urotelial nested se caracteriza por la distribución celular en forma de nidos y túbulos. Generalmente presentan poca atipia citológica que simula un carcinoma urotelial de bajo grado, o lesiones vesicales benignas como los nidos de von Brunn o los adenomas nefrogénicos

OBJECTIVE: Nested type transitional cell carcinoma of the bladder is a rare histological variant among bladder tumors. Although clinical presentation is similar to the other bladder tumors, its macroscopic appearance may be equivocally benign, with submucosal growing which is difficult to detect on cystoscopy, so that diagnosis may be delayed. METHODS: We present the characteristics of nested type transitional cell carcinoma and review the differential diagnosis for this entity with possible counterfeiters. RESULTS: In this article, we report two cases of nested type transitional cell carcinoma that presents, in one of them, all three growing patterns. CONCLUSIONS: Microscopically nested transitional cell carcinoma is characterized by a cell distribution forming nests and tubules. They generally present low cytologic atypia simulating a low grade urothelial carcinoma, or benign bladder lesions such as von Brunn nests or nefrogenic adenoma

[Adenoma metanephric: Report of 2 cases and bibliographic review.] / Adenoma metanefrico: presentación de 2 casos y revisión de la literatura.

OBJECTIVE: Two cases of metanephric adenoma are presented, a rare benign renal tumor, and a literature review is done under the current WHO classification (2016). METHODS: Standard histopathological study was performed with hematoxylin-eosin and immunohistochemistry to analyze the expression of WT, Vimentin, Racemase, CK7, CD10 and RCC. RESULTS: Neoplasms of 3 and 4.5 cm, histologically, exhibiting tubulopapillary architecture. There was no evidence of significant nuclear atypia and mitotic figures. Immunohistochemical study showed positive immunoreaction for WT1 and Vimentin in tumor cells. CONCLUSIONS: Two new cases of metanephric adenoma are presented and a review of the literature was performed in order to discuss the prognosis and differential diagnosis of metanephric adenoma. This is a rare tumor and its diagnosis lies on its morphology and its immunohistochemical profile.

Adenoma metanefrico: presentación de 2 casos y revisión de la literatura / Adenoma metanephric: Report of 2 cases and bibliographic review

OBJETIVO: Presentamos 2 casos de adenoma metanéfrico, un tumor renal benigno poco frecuente y realizamos una revisión de la literatura bajo la clasificación actual de la OMS (2016). MÉTODOS: Se realizaron cortes de bloques de parafina para tinción con HE y técnicas inmunohistoquímicas para analizar la expresión de WT, Vimentina, Racemasa, CK7, CD10 y RCC. RESULTADOS: Las neoplasias de 3 y 4,5 cm, histológicamente mostraban arquitectura tubular y papilar. No se evidenció atipia nuclear significativa ni figuras de mitosis. El estudio inmunohistoquímico demostró inmunorreacción positiva de las células tumorales para WT1 y Vimentina. CONCLUSIONES: Presentamos 2 nuevos casos de adenoma metanéfrico y realizamos revisión de la literatura que hay al respecto para discutir la evolución y el diagnóstico diferencial del adenoma metanéfrico. Este es un tumor poco frecuente y su diagnóstico reside en su morfología y perfil inmunohistoquímico

OBJECTIVE: Two cases of metanephric adenoma are presented, a rare benign renal tumor, and a literature review is done under the current WHO classification (2016). METHODS: Standard histopathological study was performed with hematoxylin-eosin and immunohistochemistry to analyze the expression of WT, Vimentin, Racemase, CK7, CD10 and RCC. RESULTS: Neoplasms of 3 and 4.5 cm, histologically, exhibiting tubulopapillary architecture. There was no evidence of significant nuclear atypia and mitotic figures. Immunohistochemical study showed positive immunoreaction for WT1 and Vimentin in tumor cells.CONCLUSIONS: Two new cases of metanephric adenoma are presented and a review of the literature was performed in order to discuss the prognosis and differential diagnosis of metanephric adenoma. This is a rare tumor and its diagnosis lies on its morphology and its immunohistochemical profile

Expression of dog1 in low-grade fibromyxoid sarcoma: A study of 19 cases and review of the literature.

DOG1 is a highly-sensitive marker often included in the immunohistochemical panel for the diagnosis of gastrointestinal stromal tumors (GISTs). Recent research has shown that DOG1 may also be expressed by low-grade fibromyxoid sarcomas (LGFMSs); this may give rise to diagnostic error when the sarcoma is located in the abdominal cavity. This paper reports on immnohistochemical expression of DOG1 in 19 LGFMSs using two different monoclonal antibodies: K9 (Leica, Novocastra Laboratories, Newcastle upon Tyne, UK) and SP31 (Thermo Scientific, Freemont, USA). All LGFMSs displayed the standard histological pattern of alternating myxoid and fibrous areas, low cellularity and bland spindle-cell morphology. Positive staining for MUC4 was observed in 18/19 cases (94.7%), while there was rearrangement of the FUS gene in 14/19 (73.7%) cases and of the EWR1 gene in 2/19 (10.5%). The sarcoma staining negative for MUC4 displayed FUS gene rearrangement. Whole-section immunohistochemistry revealed positive staining for DOG1 in 8/19 cases (42.1%), though only with clone K9. Cytoplasmic as well as membrane staining was observed in all cases; staining was focal (10-30%) and of varying intensity (1+ to 2+). In conclusion, DOG1 clone K9 exhibited low sensitivity (42.1%) for the diagnosis of LGFMS, although higher than clone SP31. Since the two clones display similar sensitivity and specificity for GIST diagnosis, SP31 would appear to be more specific for this purpose, since no reaction was observed here with LGFMS, a GIST-mimicking lesion.

Complicaciones de la enfermedad renal quística adquirida: síndrome de Wünderlich y carcinoma papilar / Major compications of acquired renal cystic disease: Wünderlich syndrome and papilar carcinoma

OBJECTIVO: Presentamos el caso de un paciente con enfermedad renal quística adquirida en hemodiálisis por enfermedad renal terminal que desarrolló dos de las complicaciones más graves asociadas a esta entidad; un carcinoma renal y hemorragia renal espontánea. MÉTODOS: Nuestro caso se trata de un paciente con enfermedad renal quística adquirida (ERQA), monorreno e intervenido por carcinoma renal de células claras 4 años antes, que desarrolló un síndrome de Wünderlich (SW). RESULTADOS: En el estudio anatomopatológico de la pieza de nefrectomía se objetivó un carcinoma renal papilar en el contexto de un riñón poliquístico tras intervención de urgencia por SW. CONCLUSIONES: La hemorragia renal es una complicación grave de la ERQA. Los pacientes sometidos a diálisis deben ser vigilados de forma activa por el riesgo de desarrollar ERQA y las complicaciones asociadas

OBJECTIVE: We report a case of acquired renal cystic disease associated with renal dialysis and end-stage renal disease. The patient suffered the two major complications related with acquired renal cystic disease; hemorrhage and renal carcinoma. METHODS: Our case is a patient with acquired renal cystic disease, single kidney after surgery for renal clear cell carcinoma four years earlier, who developed a Wünderlich syndrome (WS). RESULTS: The histological study of the nephrectomy specimen showed a renal papillary carcinoma in the context of acquired renal cystic disease after surgery for a WS. CONCLUSIONS: Renal hemorrhage is a serious complication that can lead to a fatal outcome. Patients undergoing dialysis should be monitored actively due to the risk of developing acquired renal cystic disease and associated complications

[Major complications of acquired renal cystic disease: Wünderlich syndrome and papilar carcinoma.] / Complicaciones de la enfermedad renal quística adquirida: síndrome de Wünderlich y carcinoma papilar.

OBJETIVE: We report a case of acquired renal cystic disease associated with renal dialysis and endstage renal disease. The patient suffered the two major complications related with acquired renal cystic disease; hemorrhage and renal carcinoma. METHODS: Our case is a patient with acquired renal cystic disease, single kidney after surgery for renal clear cell carcinoma four years earlier, who developed a Wünderlich syndrome (WS). RESULTS: The histological study of the nephrectomy specimen showed a renal papillary carcinoma in the context of acquired renal cystic disease after surgery for a WS. CONCLUSIONS: Renal hemorrhage is a serious complication that can lead to a fatal outcome. Patients undergoing dialysis should be monitored actively due to the risk of developing acquired renal cystic disease and associated complications.

Tumor fibroso calcificante de intestino delgado / Calcifying fibrous tumor of the small bowel

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[Subclinical acute intermittent porphyria. An uncommon cause of chronic hepatitis]. / Porfiria aguda intermitente subclínica. Etiología inusual de hepatitis crónica.

Acute porphyria is a term that encompasses a group of hereditary disorders involving defects in heme metabolism, characterized by acute episodes of abdominal pain, acute hypertension, tachycardia and neuropsychiatric disorders, sometimes leading to convulsions, ascending paralysis and coma. Misdiagnosis or delayed diagnosis can seriously worsen prognosis. We report the case of a woman with subclinical acute intermittent porphyria and chronic hepatitis incidentally diagnosed due to transaminase elevation on laboratory analysis.

Porfiria aguda intermitente subclínica. Etiología inusual de hepatitis crónica / Subclinical acute intermittent porphyria. An uncommon cause of chronic hepatitis

Resumen Las porfirias agudas (PA) son trastornos hereditarios en la síntesis del grupo hemo quese caracterizan por la aparición de episodios agudos de dolor abdominal, crisis de hipertensiónarterial, taquicardia y trastornos neuropsiquiátricos, llegando incluso a provocar convulsiones,parálisis ascendente o coma. El retraso o error en el diagnóstico puede empeorar gravemente elpronóstico. Presentamos el caso de una paciente con porfiria aguda intermitente (PAI) subclínicay hepatitis crónica diagnosticada de forma casual por una elevación de transaminasas en elestudio analítico (AU)

Abstract Acute porphyria is a term that encompasses a group of hereditary disorders involvingdefects in heme metabolism, characterized by acute episodes of abdominal pain, acutehypertension, tachycardia and neuropsychiatric disorders, sometimes leading to convulsions,ascending paralysis and coma. Misdiagnosis or delayed diagnosis can seriously worsen prognosis.We report the case of a woman with subclinical acute intermittent porphyria and chronichepatitis incidentally diagnosed due to transaminase elevation on laboratory analysis (AU)

Gastrointestinal stromal tumors: morphological, immunohistochemical and molecular changes associated with kinase inhibitor therapy.

Recurrent or metastatic GISTs are currently treated with kinase inhibitors since they achieves disease control in 70-85% of patients but this response depend on KIT and PDGFRA gene mutation status. We review the morfological and molecular findings associated to kinase inhibitors administration in GISTs based on the literature on Medline and authors' own experience. The initial response to kinase inhibitors (imatinib mesylate, Gleevec, Novartis) usually is partial and depend on the mutational KIT or PDGFRA state. Amongst patients wih KIT mutations, the best results are achived in those harboring exon 11 (85%) and exon 9 (45%) mutations. GISTs harboring PDGFRA gene mutations generally respond favorably except those involving the Asp842Val mutation. In the absence of KIT/PDGFRA gene mutations, partial response or disease stabilization is reported in 23% and 50% of patients, respectively, and disease progression in 19%. Histological examination of tumors displaying an initial response to imatinib reveals a highly-variable reduction in the number of tumor cells, a decline in the proliferative index, myxohyaline or sclerohyaline stroma, and a varying degree of bleeding and edema, necrosis and cystification. 72% of patients with initial good response to imatinib, display metastases or new nodule growth within an existing clinically-quiescent tumor after 12-36 months of treatment. This secondary resistance is characterized by a number of well-defined morphological and molecular changes. Histologically, the new growths display increased mitotic activity, pleomorphism, an epithelioid or mixed phenotype and persistent KIT expression although more rarely, dedifferentiation and loss of KIT expression (Fig. 4), as well as trans-differentiation into a rhabdomyosarcoma or epithelial phenotype has been reported. Molecularly, 46-67% of patients present additional KIT mutations, generally in the kinase domain (exons 13, 14 and 17) but also in the ATP-binding domain (exons 15,16) of the same allele. Secondary PDGFRA mutations are very rare. Secondary mutations have not been observed in GISTs not harboring KIT/PDGFRA mutations, or in tumors displaying an unusual morphology or loss of CD117 expression. A number of studies highlight the presence of different resistance mutations within different new tumor nodules, as well as the simultaneous development of distinct resistant tumor subclones within a single lesion (acquired polyclonal resistance). Secondary mutation in genes other than KIT/PDGFRA has only been reported in BRAF (Val600Glu).

Avances en los tumores del estroma gastrointestinal / A review of the recent advances in gastrointestinal stromal tumours

Los tumores del estroma gastrointestinal (GIST) son las neoplasias mesenquimales más comunes del tubo digestivo y representan uno de los mejores modelos de tratamiento farmacológico dirigido a dianas moleculares específicas. En general, el diagnóstico morfológico no plantea mayores problemas debido a que el cuadro histológico (celularidad fusiformes y/o epiteliode) e inmunohistoquímico (expresión de CD117, CD34 y, más eventualmente, de actina de músculo liso, desmina e incluso proteína S-100) suele ser bastante característico. No obstante, en el 5–10% de los casos la histología y los resultados inmunohistoquímicos no son los esperados y el diagnóstico debe descansar en la demostración mediante técnicas de biología molecular de mutaciones en KIT o PDGFRA, ya que el diagnóstico correcto es requisito necesario para la aplicación de la terapia específica. En el presente artículo llevamos a cabo una revisión sobre los avances acontecidos en 5 aspectos fundamentales sobre la biología y diagnóstico de estas neoplasias: nuevos marcadores inmunohistoquímico, factores pronósticos, biología molecular, síndromes clínicos asociados y respuesta tisular a los inhibidores de tirosin quinasa(AU)

Gastrointestinal stromal tumours (GIST) are the most common mesenchymal neoplasms of the intestinal tract and are one of the best models for treatment with molecular target therapy. Morphological diagnosis does not usually present many problems due to the presence fusiform and/or epitheliod cells together with the characteristic immunohistochemical expression of CD117, CD34 and, less frequently, smooth muscle actin, desmin and even S-100 protein. However, unexpected histological and immunohistochemical results are found in 5 to 10% of cases. In such unusual cases, molecular biology is needed to demonstrate c-KIT or PDGFRA mutations in order to make a correct diagnosis, which is a necessary prerequisite for molecular target therapy. The present article reviews recent advances in five fundamental biological and diagnostic aspects of GIST: new immunohistochemical markers, prognostic factors, molecular biology, associated clinical syndromes and tissue response to tyrosine kinase inhibitors(AU)

Interferon-induced cutaneous sarcoidosis in melanoma.

Interferon-induced cutaneous sarcoidosis in the adjuvant treatment of melanoma is a rare side effect. We present the case of a patient who developed two histologically confirmed subcutaneous sarcoid nodules 15 months after starting adjuvant therapy with interferon for lymph node metastatic melanoma in which the primary tumor was not known. The extension study, coinciding with occurrence of the nodules, showed no systemic sarcoidosis. This therefore represents the second reported case of interferon-induced cutaneous sarcoidosis in melanoma therapy. As computed axial tomography-PET or other imaging techniques are unable to differentiate between the radiological signs of melanoma metastasis and sarcoidosis, histological evaluation of the granulomatous lesions is essential with a view to avoiding unnecessary treatments.