Neoplasia papilar de polaridad inversa: un nuevo subtipo de tumor renal de buen pronóstico / Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis

Introducción: La neoplasia papilar renal de polaridad inversa (NPRPI) ha sido recientemente reconocida como una entidad separada de la clasificación tradicional de los carcinomas papilares de células renales por sus peculiares características histopatológicas, inmunofenotípicas y moleculares, y un comportamiento indolente. Material y métodos: En este trabajo aportamos 6 nuevos casos y realizamos una revisión de la literatura publicada al respecto hasta el momento actual, recopilándose un total de 104 casos. Resultados: Nuestros casos de NPRPI corresponden a 5 hombres y una mujer, con edades comprendidas entre los 47 y los 91 años. En 5 casos la NPRPI resultó un hallazgo incidental en piezas de nefrectomía indicada por la presencia de otro tumor renal y en uno la NPRPI fue el motivo de la intervención quirúrgica. Nuestros casos presentan tamaños entre los 2 y los 13mm, y una histología papilar con revestimiento en monocapa de células eosinófilas con núcleos de bajo grado en localización apical. Inmunohistoquímicamente muestran una constante positividad para GATA3 y negatividad para vimentina. Se identificaron mutaciones en KRAS en el 50% de ellos. Tras un seguimiento comprendido entre uno y 60 meses, 5 de los pacientes seguían vivos sin recurrencia o metástasis y uno falleció a causa de un carcinoma urotelial. Conclusiones: Nuestros casos concuerdan con las características clínicas y patológicas descritas en los publicados hasta el momento. Aportamos la primera serie nacional y corroboramos la existencia de unos criterios diagnósticos definidos y constantes que permiten considerar la NPRPI como una entidad propia distintiva. (AU)

Introduction: Papillary renal cell neoplasm with reverse polarity (PRNRP) has recently been recognized as an entity separate from the traditional classification of papillary renal cell carcinomas, due to its specific histopathological, immunophenotypic and molecular characteristics, as well as its indolent behavior . Material and methods: We provide 6 new cases and a review of the literature published until the present time, which comprises a total number of 104 cases. Results: Our PRNRP cases correspond to 5 men and one woman aged between 47 and 91 years. In 5 of the 6 cases, the PRNRP was an incidental finding in nephrectomy specimens. Nephrectomy had been indicated due to the presence of another renal tumor, except for one case, in which surgical intervention was indicated due to PRNRP. Our cases present mass sizes between 2 and 13mm, as well as papillary histology with a monolayered lining of eosinophilic cells with low-grade nuclei in apical location. Immunohistochemically, they show a constant positivity for GATA3 and negativity for vimentin. KRAS mutations were identified in 50% of our cases. After a follow-up ranging between one and 60 months, 5 of the cases were still alive without recurrences or metastases, and one died from urothelial carcinoma. Conclusions: Our cases agree with the clinical and pathological characteristics described in the PRNRP cases published to date. With the present study, we provide the first series of national cases corroborating the existence of well-defined and constant diagnostic criteria that allow PRNRP to be considered as a distinctive entity. (AU)

Papillary renal cell neoplasm with reverse polarity: A new subtype of renal tumour with favorable prognosis.

INTRODUCTION: Papillary renal cell neoplasm with reverse polarity (PRNRP) has recently been recognized as an entity separate from the traditional classification of papillary renal cell carcinomas, due to its specific histopathological, immunophenotypic and molecular characteristics, as well as its indolent behavior. MATERIAL AND METHODS: We provide 6 new cases and a review of the literature published until the present time, which comprises a total number of 104 cases. RESULTS: Our PRNRP cases correspond to 5 men and one woman aged between 47 and 91 years. In 5 of the 6 cases, the PRNRP was an incidental finding in nephrectomy specimens. Nephrectomy had been indicated due to the presence of another renal tumor, except for one case, in which surgical intervention was indicated due to PRNRP. Our cases present mass sizes between 2 and 13 mm, as well as papillary histology with a monolayered lining of eosinophilic cells with low-grade nuclei in apical location. Immunohistochemically, they show a constant positivity for GATA3 and negativity for vimentin. KRAS mutations were identified in 50% of our cases. After a follow-up ranging between one and 60 months, 5 of the cases were still alive without recurrences or metastases, and one died from urothelial carcinoma. CONCLUSIONS: Our cases agree with the clinical and pathological characteristics described in the PRNRP cases published to date. With the present study, we provide the first series of national cases corroborating the existence of well-defined and constant diagnostic criteria that allow PRNRP to be considered as a distinctive entity.

Usefulness of GATA-3 as a marker of seminal epithelium in prostate biopsies. / Utilidad de GATA-3 como marcador de epitelio seminal en las biopsias de próstata.

OBJECTIVES: The incidental presence of seminal vesicle epithelium in prostate needle biopsies is generally recognisable through routine microscopy. However, the biopsy can sometimes be erroneously interpreted as malignant due to its architectural and cytological characteristics, and immunohistochemistry can be useful for correctly identifying the biopsy. Our objective was to analyse the potential usefulness of GATA-3 as a marker of seminal epithelium. MATERIAL AND METHODS: Through immunohistochemistry with a monoclonal anti-GATA-3 antibody (clone L50-823), we studied seminal vesicle sections from 20 prostatectomy specimens, 12 prostate needle biopsies that contained seminal vesicle tissue and 68 prostate biopsies without seminal vesicle epithelium, 36 of which showed adenocarcinoma. RESULTS: Staining for GATA-3 was intense in the 20 seminal vesicles of the prostatectomy specimens and in the 12 prostate needle biopsies that contained seminal epithelium. In the 60 biopsies without a seminal vesicle, GATA-3 was positive in the prostate basal cells and even in the secretory cells (57 cases), although with less intensity in 55 of the cases. One of the 36 prostatic adenocarcinomas tested positive for GATA-3. CONCLUSIONS: The intense immunohistochemical expression of GATA-3 in the seminal vesicle epithelium can help identify the epithelium in prostate biopsies. This marker is also positive in the basal cells of healthy prostates and, with less intensity, in the secretory cells. Positivity, weak or moderate, is observed on rare occasions in prostatic adenocarcinomas.

Interobserver reproducibility of a grading system for chromophobe renal cell carcinoma.

OBJECTIVES: To evaluate interobserver reproducibility of a grading system proposed by Paner et al. for chromophobe renal cell carcinoma. MATERIAL AND METHODS: After selecting 23 cases of chromophobe renal cell carcinoma from the Xeral-Cíes Hospital, Meixoeiro Hospital and POVISA Hospital from the last 15 years, an informative meeting on the Paner et al. grading system criteria was held. After, the participating pathologists applied the system to each case, evaluating one slide selected. Kappa index for interobserver reproducibility was calculated, and it was classified according to the Landis and Koch scale. RESULTS: The grading distribution was similar for most of the 6 participating observers, with grade 1 predominance. The remaining 2 observers considered a relatively higher proportion of grade 2. Kappa index values ranged from 0.136 to 0.674, with a discrete-moderate reproducibility index predominance (0.21-0.60). Highest Kappa value (0.674) was obtained between the most novel and the most expert interobservers. The lowest Kappa value was obtained among the most veteran pathologists (0.136). CONCLUSIONS: Interobserver reproducibility for chromophobe renal cell carcinoma is discrete-moderate in our institutions when the novel grade proposed by Paner et al. is used. Labeling of grades 1 and 2 is not homogeneous among 6 participating observers. While awaiting a grading consensus on a new classification by the scientific societies, we consider that the routine use of a grading system for chromophobe renal cell carcinoma should not be used.

Livedoid vasculopathy and recurrent thrombosis in a patient with lupus: seronegative antiphospholipid syndrome?

Livedoid vasculopathy is a rare condition which predominantly affects young women. It is characterized by intense painful purpuric maculae in the legs, ankles and feet, due to thrombosis of the small and medium-sized dermal vessels, in the absence of vasculitis. Livedoid vasculopathy has been frequently associated with hypercoagulable states and antiphospholipid syndrome. We describe a 34-year-old White woman suffering from systemic lupus erythematosus, livedo reticularis, haemolytic anaemia, severe thrombocytopenia and recurrent venous thrombosis who was admitted to the hospital for extremely painful purpuric lesions in her lower limbs. The clinical and histological findings were diagnostic of livedoid vasculopathy. Once the initial sub-therapeutic international normalized ratio levels were corrected, livedoid vasculopathy did not recur. Tests for antiphospholipid antibodies were repeatedly negative. This case, the first reported of livedoid vasculopathy in a patient with seronegative antiphospholipid syndrome and systemic lupus erythematosus, draws attention to livedoid vasculopathy, a thrombotic dermopathy that may be under-diagnosed in patients with antiphospholipid syndrome.

[Squamous cell carcinoma arising on an epidermal inclusion cyst: a case presentation and review of the literature]. / Carcinoma epidermoide desarrollado sobre quiste de inclusión epidérmica cutáneo. Presentación de un nuevo caso y revisión de la literatura.

Epidermal inclusion cysts are very common lesions that very rarely undergo malignant transformation-in the English-language literature we have only found 18 adequately documented cases. We present the case of a man with a 2-month history of a retroauricular skin lesion in which histological study revealed squamous cell carcinoma arising on an epidermal inclusion cyst. Cysts that grow rapidly, reach a large size, ulcerate, develop a fistula, or that do not respond to medical treatment, and those that recur should be excised completely and histological study performed of the whole lesion.

Immunohistochemical analysis of P-Selectin as a possible marker of vitality in human cutaneous wounds.

Immunohistochemical detection of mediators of inflammation, such as P-Selectin, has been proposed to assess vitality of wounds. Forty-five incised cutaneous wounds (24 vital, 14 post-mortem, seven with induced autolysis/putrefaction) were immunostained with antibodies against P-Selectin and CD31. The percentage of stained lumina for P-Selectin out of the total of CD31 positive vessels (P-S/CD31 index) was calculated at both edges of every specimen. In vital samples, the P-S/CD31 index ranged from 10.7% to 71.4% at the wound edge, and was 12.5-58.8% for the opposite margin, with a ratio between both indices of 0.37-1.77 (mean: 0.94). In post-mortem cases, the index ranged from 22.5% to 69.2% at the wound edge, and was 28-89.5% at the opposite margin, with a ratio between both indices of 0.76-1.9 (mean: 0.96). Differences between ratios were not statistically significant and thus precluded any assessment of vitality. The analysis of P-Selectin/CD31 immunoreactivity in skin wounds was not useful for the diagnosis of vitality when evaluating both edges for each specimen. Moreover, P-Selectin has been detected in post-mortem injuries and it is not specific to vital injuries. Microscopic evaluation becomes difficult after autolysis/putrefaction.

[Juvenile xanthogranuloma of the penis]. / Xantogranuloma juvenil de pene.

We report a case of juvenile xanthogranuloma of the penis in a 30 year old patient with clinical suspicion of epidermoid cyst. Histology and ethiopathogenesis are reviewed, with special emphasis on the differential diagnosis with other similar lesions with worst prognosis.

[Soft tissue metastases by micropapillary bladder carcinoma. Metastatic disease]. / Metástasis en partes blandas por un carcinoma micropapilar de vejiga.

Micropapillary transitional cell carcinoma is a rare (incidence of 0.7%) and highly aggressive variant of bladder carcinoma. Morphologically, it is characterized by small tight clusters of neoplastic cell floating in clear spaces resembling lymphatic channels. Its usual presentation is like a high grade and stage carcinoma and most often is associated with a variable component of conventional carcinoma or other variants. The usual sites of bladder cancer metastases are the lymph nodes, lungs, bone and liver. Soft tissues metastases from transitional cell carcinoma of the bladder occur infrequently. We report the cases of a 77-year-old man presenting with an abdominal soft tissue mass a six years after local excision of a micropapillary bladder carcinoma.

[Conjunctival inclusion cyst after strabismus surgery by hang-back recession]. / Quiste conjuntival de inclusión tras cirugía de estrabismo según técnica de retroinserción en asa.

CASE REPORT: A 24-year-old woman, with a history of infantile esotropia and DVD operated on in infancy, had strabismus surgery performed by us. Four months later she presented with a cystic lesion that recurred after drainage and medical treatment. Complete excision of the cystic lesion was therefore performed. DISCUSSION: The epithelial cells implanted on the sclera at the time of the most recent surgery may have been the origin of inclusion cyst which developed after the strabismus surgery. This suggests a possible relationship with the scleral suture as the mechanism of cyst formation, independent of the muscle position. Complete excision is the recommended treatment for large cysts.

The pathology of the heart conduction system in congenital heart block.

The incidence of congenital complete heart block is estimated in 1 of 2500-20,000 births. Many cases are isolated (found in an otherwise normal heart) and the pathology of the heart conduction system is variable. We report a 51-year-old man with the diagnosis of complete heart block, with a permanent pacemaker. No family history of rhythm disturbances was available. The patient presented and endocarditis after replacement of the pacemaker battery. The prognosis was poor and the patient died three months later. Autopsy examination showed signs of shock, of septic origin. The heart was hypertrophic (450g) and the left ventricle thickened. Histopathological examination of the heart conduction system showed that the sinus and atrioventricular nodes were normal, but the His bundle was interrupted and replaced by fibrous tissue. No inflammatory signs were present. Loss of conducting fibres and their replacement by fibrous tissue is the most common pathological process in complete heart block. In this case His bundle was mostly affected, different to Lev's disease where the process is more distal (branching atrioventricular bundle) and to Lenegre's disease, which shows a diffuse damage in the conducting system.

[Lymphoepithelial cyst of the thyroid gland: a case report and review of the literature]. / Quiste linfoepitelial intratiroideo: caso clínico y revisión de la literatura.

We report a case of lymphoepithelial cyst of the thyroid gland in a 31 year-old-man with chronic lymphocytic thyroiditis and history of multinodular goiter. The lesion was resected and the histopathologic examination showed that the cystic mass was lined by squamous and focally columnar epithelium, an it was surrounded by follicular lymphoid tissue. To our knowledge only twenty cases of this lesion have been reported in the medical literature. An association with chronic thyroiditis has been noted in 10 cases.

[Expression of cytokeratin 5 and calretinin in clear-cell renal cell carcinoma]. / Expresión de citoqueratina 5 y calretinina en el carcinoma renal de células claras.

PURPOSE: Cytokeratin 5 (CK5) and calretinin have been useful in different studies as immunohistochemical markers suggestive of mesothelioma, and their expression is analyzed for the histological differential diagnosis with adenocarcinomas, specially when confronting with metastatic tumors of unknown origin. We have analyzed the expression of CK5 and calretinin in clear cell renal cell carcinoma. METHODS: A series of 63 clear cell renal cell carcinomas was studied. 46 of these cases were embedded in two tissue arrays, and a second group, of 17 cases, was constituted by conventional paraffin blocks from high-grade tumors (grade 4 of Fuhrman). Immunohistochemical staining was performed with monoclonal antibodies against CK5 and calretinin, following the labeled sptreptavidin-biotin technique. RESULTS: No positivity for calretinin was observed in any case, while CK5 was focally expressed, in an isolated group of cells, in 1 of the 63 cases (1,59%) which corresponded to a high-grade carcinoma (grade 4 of Fuhrman). CONCLUSIONS: Expression of calretinin was not observed in clear cell renal cell carcinoma and positivity for CK5 occurred only in one case, in a very small proportion of tumor cells. Therefore, in practice, although the positivity for these markers cannot completely exclude renal cell carcinoma, this result is very rare in this tumor and other diagnostic posibilities should be considered.

[Expression of CD10 and renal cell carcinoma marker in clear cell renal cell carcinoma: analysis on tissue arrays]. / Expresión de CD10 y del marcador de carcinoma renal en el carcinoma renal de células claras: análisis en matrices tisulares.

OBJECTIVES: CD10 and renal cell carcinoma (RCC) marker antibodies react against proteins of the epithelium of the renal proximal tubule, being expressed by renal cell carcinomas. The frequence and pattern of expression of both markers are analysed in a series of clear cell renal cell carcinomas. METHOD: Two tissue arrays were used, which were composed of cylinders obtained with a 16G needle from 40 paraffin blocks that corresponded to clear cell renal cell carcinomas. The labeled streptavidin-biotin technique was performed (LSAB2, Dako) using CD10 and RCC monoclonal antibodies (Novocastra), testing different antigen retrieval methods for RCC. Immunoreactivity was evaluated as + (isolated cells or focal staining); ++ (moderate) and +++ (extense). RESULTS: Thirty cases (75%) were positive for CD10: 12 +; 5 ++ and 13 +++. The best antigen retrieval method for RCC was a double enzyme digestion (trypsin + protease). Twenty cases (50%) were positive for RCC: 7 +; 5 ++ and 8 +++. Four cases out of the 20 immunoreactive for RCC were negative for CD10. The 16 remaining cases also expressed CD10. CONCLUSIONS: CD10 and RCC are often expressed by clear cell renal cell carcinomas, and they may be useful markers to suggest a renal origin of carcinomas. RCC is less sensitive than CD10. Staining for both of them is usually focal, and thus sensitivity of these techniques decreases when small samples are investigated, such as tissue arrays. The antigen retrieval method is essential for RCC immunohistochemical detection, obtaining the best results with the use of proteolytic enzymes.

[Verrucous carcinoma of the penis arising from a lichen planus. A true preneoplastic lesion?]. / Carcinoma verrucoso de pene desarrollado sobre un liquen plano. Una auténtica lesión preneoplásica?

INTRODUCTION: Up to 30% of squamous cell carcinomas of the penis arise from a lichen sclerosus, but very few reports in which lichen planus was the preexisting lesion have been published. We report a male with verrucous carcinoma of penis that developed in an area of lichen planus. CLINICAL CASE: A 53-year-old male presented with an exophytic tumor on the glans penis that had been present for about 6 months. The lesion developed in a previously biopsied area of lichen planus hypertrophicus. After excision, histological diagnosis was verrucous carcinoma. COMMENT: The development of any subtype of squamous cell carcinoma of penis in a lichen planus can be coincidental, or a neoplastic transformation of lichen planus can take place.

Carcinoma verrucoso de pene desarrollado sobre un liquen plano. ¿una auténtica lesión preneoplásica? / Verrucous carcinoma of the penis arising from a lichen planus. A true preneoplastic lesion?

Introducción: Hasta un 30% de carcinomas epidermoides de pene se desarrollan sobre un liquen escleroso, mientras que hay muy pocos casos publicados en los que la lesión preexistente sea un liquen plano. Se presenta un carcinoma verrucoso de pene desarrollado sobre un área de liquen plano. Caso clínico: Un varón de 53 años consultó por presentar un tumor exofítico en glande de 6 meses de evolución, que había crecido sobre una lesión biopsiada 2 años antes, y diagnosticada como liquen plano hipertrófico. Tras su extirpación, el diagnóstico histológico fue de carcinoma verrucoso. Comentario: el desarrollo de cualquier variante de carcinoma epidermoide de pene sobre un liquen plano puede ser meramente casual, o por el contrario podría ser una transformación neoplásica del mismo

Introduction: up to 30% of squamous cell carcinomas of the penis arise from a lichen sclerosus, but very few reports in which lichen planus was the preexisting lesion have been published. We report a male with verrucous carcinoma of penis that developed in an area of lichen planus. Clinical case: a 53-year-old male presented with an exophytic tumor on the glans penis that had been present for about 6 months. The lesion developed in a previously biopsied area of lichen planus hypertrophicus. After excision, histological diagnosis was verrucous carcinoma. Comment: the development of any subtype of squamous cell carcinoma of penis in a lichen planus can be coincidental, or a neoplastic transformation of lichen planus can take place

[Spermatic cord mass as a manifestation of systemic vasculitis: a case report and review of the literature]. / Masa en el cordón espermático como primera manifestación de una vasculitis sistémica: presentacion de un caso y revisión de la literatura.

We report a case of necrotizing granulomatous vasculitis in the spermatic cord in a 35-year-old man with an antecedent of brain stroke 3 years before. The clinical manifestation was as a painless left scrotal mass. The diagnosis was established by histological examination of the spermatic cord. We discuss the physical findings, radiological features and pathological findings, reviewing the literature for previous similar cases.

Hobnail haemangioma associated with the menstrual cycle.

We report the third case in the literature of a hobnail haemangioma with cyclic changes throughout the menstrual cycle in a 36-year-old woman, suggesting a hormonal basis for the variations in the tumour. This patient was under oral contraceptive treatment due to ovarian endometriosis, making the clinical diagnosis difficult.

[Expression of the cerbB-2 (HER-2/neu) oncoprotein in prostatic adenocarcinoma]. / Estudio de la expresión de cerbB-2 en el adenocarcinoma de próstata.

OBJECTIVES: Our aim is to determine the expression of the cerbB-2 oncoprotein in prostate cancers using an immunohistochemistry staining and to compare these results with several clinical and histological prognostic factors. METHODS: An immunohistochemical staining using the cerbB-2 monoclonal antibody (Dako) was performed in 32 radical prostatectomy specimens diagnosed of adenocarcinoma. The intensity of cerbB-2 expression was evaluated with a scale that variated from 0 (no staining) to 3+ (strong complete membrane staining) according to published guidelines. Association of cerbB-2 index immunoreactivity with clinical and histological prognostic factors was examined. RESULTS: Definite positive membranous staining was detected in 14 of 32 neoplastic cases (44%). Such overexpression was correlated with higher Gleason grade (p=0.04) and higher stage of disease (p=0.038). CONCLUSIONS: 1) This study shows that 44% of all prostate cancer express the cerbB-2 oncoprotein with immunohistochemical technique. 2) These findings suggest that is necessary to standardize the immunohistochemical staining procedure with cerbB-2 in prostate adenocarcinoma. 3) The level of cerbB-2 expression was correlated with Gleason grade and clinical stage.

[Expression of CDX2 in urinary bladder and urethra lesions]. / Expresión de CDX2 en lesiones de vejiga urinaria y uretra.

BACKGROUND: CDX1 and CDX2 are transcription factors involved in the development and maintenance of the intestinal epithelial cell. Expression of CDX2 has been reported in normal and metaplastic intestinal epithelium, and in those adenocarcinomas with that cellular origin. We have analyzed the expression of this marker in reactive and tumoral lesions arising in urinary bladder, urethra and urachus. METHOD: CDX2 was investigated through immunohistochemistry on paraffin-embedded tissue, using the labelled streptavidin-biotin method (LSAB2, Dako) with a monoclonal antibody (CDX2-88, BioGenex). RESULTS: Expression of CDX2 was observed in intestinal-type cistitis glandularis, intestinal metaplasia of urinary bladder, bladder adenocarcinoma, mucinous urothelial-type carcinoma of prostatic urethra and urachal mucinous carcinoma. CDX2 was not detected in normal urothelium and prostatic glandular epithelium, Von Brunn nests, typical-type cistitis glandularis, glandular adenosis and transitional carcinoma. CONCLUSIONS: Lesions, both benign and malignant, with enteric-cell morphological features show positivity for CDX2. Expression of this marker is not organ-specific but is just related to a cellular phenotype. Reactivity for CDX2 in an adenocarcinoma can be consistent with an origin in urinary tract or urachus.