Primary scrotal melanoma: it is time to destigmatize genital lesions

Abstract Primary male genital melanomas are very rare; they are associated with high mortality and late detection. Scrotal melanoma is the least common presentation and only 23 cases have been reported. Herein, the authors present a 30-year-old patient with stage IIIC (T4b, N2a, M0) scrotal melanoma in order to report the characteristics, treatment, and outcome, as well as to emphasize the importance of examination of the genitals, education of patients about self-examination and destigmatizing genital lesions to increase the likelihood of earlier detection.

Angiomiofibroblastoma-like, un tumor excepcional: presentación de un caso clínico y diagnóstico diferencial / Angiomyofibroblastoma-like, an exceptional tumor: presentation of a clinical case and differential diagnosis

Presentamos un caso de Angiomiofibroblastoma-like (AML), en el que la ecografía fue importante para determinar la detección, localización y extensión local. Hallazgos: La ecografía demostró una lesión paratesticular sólida hipoecogénica de bordes bien definidos lo que sugirió lesión benigna extratesticular. La histología evidenció una neoplasia mesenquimal benigna tipo mixoide en cuyo diagnóstico diferencial se incluyen el Angiomixoma superficial (AMS), Angiomixoma agresivo (AMA), Angiomiofibroblastoma (AMF) y el AML. En base a estos hallazgos se realizó una revisión de tumores de similares características en la literatura, llegando finalmente al diagnóstico de AML cuya localización paratesticular sólo ha sido descrita en muy pocos casos hasta la fecha. Conclusión: La ecografía es útil para localizar los tumores paratesticulares y determinar su extensión local. No obstante, para llegar al diagnóstico definitivo es necesario realizar un estudio histológico e inmunohistoquímico de la tumoración.

We present a case of a paratesticular Angiomiofibroblastoma-like (AML) tumor in which ultrasound was important to give the specific location and local extension of the lesion. Findings: Ultrasound revealed a hypoechoic paratesticular lesion with well-defined borders, suggesting an extratesticular benign lesion. The histology showed a benign mesenchymal myxoid-type neoplasm. The differential diagnosis included superficial Angiomyxoma (AMS), Aggressive angiomyxoma (AMA), Angiomyofibroblastoma (AMF) and AML. Based on these findings, a review of similar tumors was carried out and ultimately led to the diagnosis of paratesticular AML. This location has been described only in a few cases in the literature. Conclusion: Ultrasound is useful to locate paratesticular tumors and determine their local extension. However, a definitive diagnosis still requires a histological and immunohistochemical study.

Mammary and extramammary Paget's disease

Paget's disease, described by Sir James Paget in 1874, is classified as mammary and extramammary. The mammary type is rare and often associated with intraductal cancer (93-100% of cases). It is more prevalent in postmenopausal women and it appears as an eczematoid, erythematous, moist or crusted lesion, with or without fine scaling, infiltration and inversion of the nipple. It must be distinguished from erosive adenomatosis of the nipple, cutaneous extension of breast carcinoma, psoriasis, atopic dermatitis, contact dermatitis, chronic eczema, lactiferous ducts ectasia, Bowen's disease, basal cell carcinoma, melanoma and intraductal papilloma. Diagnosis is histological and prognosis and treatment depend on the type of underlying breast cancer. Extramammary Paget's disease is considered an adenocarcinoma originating from the skin or skin appendages in areas with apocrine glands. The primary location is the vulvar area, followed by the perianal region, scrotum, penis and axillae. It starts as an erythematous plaque of indolent growth, with well-defined edges, fine scaling, excoriations, exulcerations and lichenification. In most cases it is not associated with cancer, although there are publications linking it to tumors of the vulva, vagina, cervix and corpus uteri, bladder, ovary, gallbladder, liver, breast, colon and rectum. Differential diagnoses are candidiasis, psoriasis and chronic lichen simplex. Histopathology confirms the diagnosis. Before treatment begins, associated malignancies should be investigated. Surgical excision and micrographic surgery are the best treatment options, although recurrences are frequent.

Extramammary Paget's disease: Analysis of 17 Chinese cases.

Background/Objective: Extramammary Paget’s Disease (EMPD) seems to be more common in Caucasians than Chinese. We report the clinical manifestations, management, and prognostic characteristics in 17 Chinese patients. Methods: Medical records and biopsies of 17 patients who had been treated at a large university hospital in China between March 2005 and January 2012 were reviewed. Results: Of the 17 patients, 14 were men. They had lesions on the scrotum and the penis. Of the three women, two had vulvar and one had inguinal lesions. All patients underwent Mohs micrographic surgery (MMS). Three men had metastasis to the inguinal lymph nodes and underwent an extensive local excision with inguinal lymphadenectomy. Eight patients who had positive excision margins received additional radiation therapy. The mean follow-up duration was 54 months (4-85 months). One patient had two recurrences. Three had metastasis to the inguinal lymph node. One had metastasis to the bone and concomitant prostate cancer. Two patients died of the disease. Conclusion: A striking difference in presentation of EMPD in Chinese compared with Caucasians is the male predominance and location on the penis and scrotum. Mohs micrographic surgery followed by radiotherapy is an effective treatment. Long-term follow-up suggests that the disease has a good prognosis when it does not metastasise.

Male Genitourinary Tumors : 14 Years Experience in Garhwal Region of India.

Genitourinary tumors may show varied clinical presentation and frequency in different regions of the world. The present study was therefore conducted to analyze the frequency, clinical presentation and the histopathological types of the various male genitourinary tumors diagnosed over a period of 14 years in a major teaching institute of north Himalayan region of India. Material and Methods: Retrospective study was undertaken in the Pathology department of the Institute which included all the cases of male genitourinary tumors which were diagnosed on histopathology from the time period between 1 Jan 1997 till 31st Dec. 2010. Results: The study showed that prostate was the most common site for male genitourinary tumors with prostatic adenocarcinoma as the most common histopathological type of tumor. Testicular Non- Hodgkin’s lymphoma constituted about 8% of total tumors in testes which mostly presented in elderly age group. Renal cell carcinoma (66.2%) was the most common tumor in kidney with much lower frequency of Wilm’s tumor (16.9%). Occasional rare tumors such as hemangioma of urinary bladder and schwannoma of penis were also seen over 14 years. Conclusion: The study concludes clinically patients were associated with more severe symptoms as they presented late to the hospital from the remote areas of this Himalayan region leading to late detection of tumors. Prostatic adenocarcinoma was the most common tumor while renal cell carcinoma was most common tumor in kidney. The present study provides valuable information to clinicians and pathologists regarding frequency, clinical presentation and histopathological types of male genitourinary tumors in this region which can be further used to formulate strategies for better management of these tumors.

Paraganglioma of seminal vesicle and chromophobe renal cell carcinoma: a case report and literature review / Paraganglioma da vesícula seminal e carcinoma de células renais cromófobo: um relato de caso e revisão da literatura

CONTEXT: Extra-adrenal paragangliomas are rare tumors that have been reported in many locations, including the kidney, urethra, urinary bladder, prostate, spermatic cord, gallbladder, uterus and vagina. CASE REPORT: This report describes, for the first time to the best of our knowledge, a primary paraganglioma of the seminal vesicle occurring in a 61-year-old male. The patient presented persistent arterial hypertension and a previous diagnosis of chromophobe renal cell carcinoma. It was hypothesized that the seminal vesicle tumor could be a metastasis from the chromophobe renal cell carcinoma. Immunohistochemical characterization revealed expression of synaptophysin and chromogranin in tumor cell nests and peripheral S100 protein expression in sustentacular cells. Succinate dehydrogenase A and B-related (SDHA and SDHB) expression was present in both tumors. CONCLUSIONS: No genetic alterations to the VHL and SDHB genes were detected in either the tumor tissue or tissues adjacent to the tumor, which led us to rule out a hereditary syndrome that could explain the association between paraganglioma and chromophobe renal cell carcinoma in a patient with arterial hypertension.

CONTEXTO: Paragangliomas extra-adrenais são tumores raros que têm sido relatados em muitas localizações, incluindo rim, uretra, bexiga, próstata, cordão espermático, vesícula biliar, útero e vagina. RELATO DE CASO: Este relato descreve, pela primeira vez em nosso conhecimento, um paraganglioma primário da vesícula seminal ocorrendo em um paciente do sexo masculino de 61 anos de idade. O paciente apresentou hipertensão arterial persistente e um diagnóstico prévio de carcinoma de células renais cromófobo (CCRC). Foi pensado que o tumor de vesícula seminal poderia ser uma metástase do CCRC. A caracterização imunoistoquímica revelou expressão de sinaptofisina e cromogranina nos ninhos de células tumorais e expressão de proteína S100 nas células sustentaculares. Expressão de succinato de-hidrogenase A e B relacionada (SDHA e SDHB) estiveram presentes em ambos os tumores CONCLUSÕES: Nenhuma alteração genética dos genes VHL e SDHB foi detectada nos tecidos tumorais e adjacentes ao tumor, o que nos levou a afastar uma síndrome hereditária que poderia explicar a associação entre o paraganglioma e o CCRC em um paciente com hipertensão arterial.

Tumor adenomatoide urológico: presentación clínico-patológica de 14 casos / Urologic adenomatoid tumor: clinico pathological presentation of 14 cases

El Tumor Adenomatoide es la neoplasia paratesticular más frecuente y localizada principalmente en el epidídimo. Material y método: Se presenta una serie clínico-patológica de 14 tumores adenomatoides urológicos diagnosticados entre 1975-2011. Resultados: La edad media de presentación fue de 46 años (rango 32-67 años). La mayoría de los casos se presentó como un nódulo firme indoloro en el epidídimo. No hubo casos de localización intratesticular. Se realizó tumorectomía en 13 casos y además orquiectomía en uno. Los tumores midieron una media de 1.9 cm (rango 0.3-5 cm) y macroscópicamente fueron sólidos, blanquecinos y bien delimitados. A la histología estaban formados por estructuras adenomatoides irregulares rodeadas por tejido fibroso. La inmunotinción para Queratina y Calretinina fueron positivas, lo que apoya el origen mesotelial del tumor. El curso clínico fue benigno en todos los casos, sin presentar recurrencias. Conclusión: Corresponde a una neoplasia benigna urogenital rara de origen mesotelial y que compromete con frecuencia el epidídimo. Se presentaron en hombres de edad media y que en la mayoría fueron tratados con tumorectomía, mostrando un curso clínico benigno...

Adenomatoid Tumor is the most frequent paratesticular neoplasia and it is located mainly in the epididymis. Material and methods: A series of 14 cases of Urological Adenomatoid Tumor diagnosed between 1975 - 2011 is presented. Results: Average age at presentation was 46 years (range 32-67 years). The majority of the cases were presented as a painless and firm nodule in the epididymis. No cases involving the testicular parenchyma were seen. The tumors measured an average of 1.9 cm (range 0.3 - 5 cm) and grossly all cases were solid, whitish and fair/y well demarcated. The histology revealed adenomatoid structures surrounded by fibrous tissue. The immunostains were positive for keratin and calretinin which support‘ the mesothelialial origin of the tumor. All cases showed a benign clinical course and no relapses were found. Conclusion: Correspond to a rare benign urogenital/neoplasia that frequently involves the epidermis. They were presented in middle age men and in the majority of the cases were treated by lumpectomy, showing a bening clinical course...

Primary lymphoma of the spermatic cord: A case report and review of the literature.

Primary lymphomas of the spermatic cord are extremely rare. To date, only 15 cases have been reported in the international literature. Herein, we report a new case of a primary lymphoma of the spermatic cord. A 73-year-old patient presented at the Urology Department, complaining of bilateral painful masses at the inguino-scrotal region. A computed tomography scan revealed spermatic cord tumor. A right inguinal orchidectomy was performed in order to establish a definitive diagnosis. Macroscopically, the tumor was restricted to the spermatic cord area, leaving unaffected the testis and the epididymis. The histopathological and immune-histological evaluation has indicated a diffuse large B-cell lymphoma. Postoperatively, the patient was investigated thoroughly but no further signs of the disease were found.

Linfoma não-Hodgkin de cordão espermático / Non-Hodgkin's lymphoma of the spermatic cord

Non-Hodgkin´s lymphoma of the spermatic cord are rare. There is the registration of 14 (fourteen) cases of spermatic cord lymphoma in the literature, all treated with radical orchiectomy with or without radiotherapy. The adjuvant chemotherapy still is not a consensus, therefore the therapy must be individualized and applied according to the stage of the disease. The present study report a new case of primary non-Hodgkin´s lymphoma of the spermatic cord treated with radical orchiectomy through inguinal via with precocious ligature of the spermatic cord and adjuvant chemotherapy. Presently found with 2 and a half years of follow-up without recidivation clinical evidence, as the image exams show to be normal.

Scrotal neoplasia: would truck drivers be at greater risk?

OBJECTIVE: To analyze how scrotal neoplasias have been managed during the past decade and to question possible factors or professions associated to its presence. MATERIALS AND METHODS: We retrospectively evaluated every case reported from 1995 to 2005 at our hospital. We described the clinical scenario, complementary exams, treatments and outcomes. We also tried to verify if there was any risk, predisposing factors or professions that would explain the cancer origin. RESULTS: Six cases were reviewed. Out of these, three patients were truck drivers. Five of them showed restricted lesions without inguinal lymph nodes enlargement. Histologically, six patients presented squamous carcinoma, with two of them having the verrucous type. The median age of patients was 52 years old (31 to 89). The five patients who are still alive had their lesions completely removed with safety margin and primary closure. CONCLUSIONS: We have noticed that the scrotal carcinoma behavior is similar to that of the penis, where removal of the lesion and study of the regional lymph nodes help to increase the patient survival rate. The outstanding fact was that three out of six patients were truck drivers, raising the hypothesis that such profession, maybe due to the contact or attrition with the diesel exhaust expelled by the engine or to sexual promiscuity, would imply in a larger risk of developing this rare neoplasia.

Laparoscopic resection of metastatic pelvic liposarcoma

We report a pelvic liposarcoma originating from the left spermatic cord that recurred following inadequate excision. In our case, the tumor was resected without performing orchiectomy previously. The patient was managed by laparoscopic resection, before undergoing radical orchiectomy in the left inguinal region. To our knowledge, no case of laparoscopic resection for the recurrent liposarcoma has been described. In addition, the present case serves to demonstrate that radical orchiectomy with wide excision is needed for paratesticular tumor.

Tumor adenomatoide del aparato genital: estudio clinicopatológico e inmunohistoquímico de 9 casos / Adenomatoid tumor of the genital tract: clinical, pathological and immunohistochemical study in 9 cases

Objetivo: Estudio clinico-patológico e inmunohistoquímico de tumores adenomatoides del aparato genital. Material y métodos: Se describen las características histológicas e inmunohistoquímicas de nueve tumores adenomatoides del Centro Médico ABC diagnosticados entre enero del 2000 a mayo del 2004. Resultados: Ocho mujeres y un hombre. Las edades variaron entre 28 y 54 años de edad. Los tumores se localizaron en útero (siete), salpinge (uno) y epidídimo (uno). El tamaño de los tumores varió entre 0.4 y 5.8cm. Se encontraron tres patrones histológicos: adenoide, angiomatoide y sólido. Características histológicas peculiares fueron la disposición de los túbulos neoplásicos alrededor de los fascículos de músculo liso y la localización periférica del patrón angiomatoide y central de los patrones sólido y adenoide en el tumor. Inmunohistoquímicamente todos los tumores mostraron positividad intensa y difusa para calretinina y AE1/AE3. La trombomodulina fue positiva en todos los tumores (focal y débil en el patrón angiomatoide y difusa e intensa en los patrones adenoide y sólido). La CK5/6 fue positiva en siete tumores (difusa en tres y focal en cuatro). Dos tumores fueron negativos para este marcador. Todos los tumores fueron negativos para CD31. Conclusiones: El inmunofenotipo expresado en nuestros casos confirma el origen mesotelial de los tumores adenomatoides.

OBJECTIVE: Describe the histological and immunohistochemical features of nine genital tract adenomatoid tumors. MATERIAL AND METHODS: Nine cases of adenomatoid tumors were collected from the files of the Pathology department at a private hospital (ABC Hospital). Tumors were studied from a histological and inmunohistochemical perspective. RESULTS: Eight women and one man were studied. Age range was 28-54 yrs. Tumors were located in the uterus (seven),fallopian tube (one) and epididymis (one). Tumor size ranged from 0.4 to 5.8 cm. We observed three histological patterns: adenoid, angiomatoid and solid. Arrangement of the neoplastic tubules around fascicles of smooth muscle; angiomatoidpattern with a peripheral location, and solid and adenoidpatterns with a central location in the tumor were some of the observed histological features. Immunohistochemically all tumors exhibited strong and diffuse positivity for calretinin and AE1/AE3. Thrombomodulin was positive in all tumors (focal and weak in angiomatoid pattern and diffuse and strong in adenoid and solid patterns). The CK5/6 antibody was positive in seven tumors (diffuse in three and focal in four). Two tumors were negative for this marker. All tumors were negative for CD31. CONCLUSIONS: The immunopheno type of the adenomatoid tumors in our series confirms their mesothelial origin.

Leiomyosarcoma of the scrotum.

A case of leiomyosarcoma of the scrotum is reported for its rarity. The tumor measured 6.2 x 4 x 4 cm and presented as a gradually increasing painless mass of 1 month duration. Light microscopy showed the typical interlacing fascicles of neoplastic cells with mitotic count of 1-2 / 10 HPF in different areas. Immunoperoxidase stains of vimentin & desmin and electron microscopy confirmed its muscle origin.

Bizarre Leiomyoma of the Scrotum

Scrotal leiomyomas with atypical bizarre nuclei are rare, which might be misdiagnosed as malignant tumor. We describe a case of scrotal bizarre leiomyoma in a 65-yr-old man. The tumor was a 1 cm-sized, well circumscribed, oval mass arising from the tunica dartos muscle. Histologically, it was formed by whorling bundles of fusiform cells with occasional atypical, pleomorphic nuclei and pseudoinclusions. Mitosis was not found. Although morphologically atypical, scrotal bizarre leiomyomas take on a biologic behavior not different from that of conventional leiomyoma, they should be distinguished from leiomyosarcoma to avoid unnecessary treatment.

Liposarcoma with Meningothelial-like Whorls. Report of Four Cases Showing Diverse Histologic Findings and Behavior

We report the clinicopathologic findings of four cases of liposarcoma with meningothelial-like whorls. Two cases occurred in the retroperitoneum and the remaining cases in the anterior mediastinum and scrotum. The whorls varied in terms of amount and morphology and the type tissue surrounding the whorls also varied in every case. One of the retroperitoneal cases with large areas of whorl coalescence recurred in the abdominal wall as an inflammatory malignant fibrous histiocytoma one year after primary resection of the tumor, and a metastasis to the cervical spines was detected twenty months later. The other retroperitoneal tumor recurred locally two years after the resection of the tumor and the amount and cellularity of the whorls as well as p53 reactivity and Ki-67 labeling index were higher in the recurrent tumor. However, coalescence of the whorls was not present in the recurrent tumor in contrast to the primary tumor. The anterior mediastinal and scrotal cases have demonstrated neither local recurrence nor distant metastasis although the follow-up period has been less than one year. The cells comprising whorls showed positive reactions for CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor in addition to vimentin and alpha-smooth muscle actin. Our results indicate that liposarcoma with meningothelial-like whorls is a heterogeneous group that shows wide variations in histologic findings and biologic behavior. The phenotypic transformation of the whorls to higher grade in two retroperitoneal tumors, which showed recurrence within two years of follow up, supports that a whorl is a sign of dedifferentiation. Although we demonstrate the expressions of several markers, such as CD10, CD56, CD99, factor XIII, and low-affinity nerve growth factor receptor, in the spindle cells of the whorls for the first time, the lineage of the whorls still cannot be addressed due to the fact that these markers are lineage nonspecific.

Seminoma extragonadal metastásico a SNC: a propósito de un caso / Metastasic extragonadal seminoma ASNC: a case presentation

Los tumores germinales extragonadales representan menos del 10 por ciento de estos tumores en el varón , cuya localización más frecuente es mediastínica y retroperitoneal. Las mt cerebrales ocurren aproximadamente 2 por ciento a 8 por ciento de los pacientes con tumores de testículo, e invariablemente son asociados con recaídas a otros sitios, más comunes pulmón o como un evento terminal. Nosotros presentamos a continuación un caso: se trata de paciente masculino de 31 años, quien inicia EA, en mayo 95, caracterizado por dolor en el hemitórax izquierdo y región external, leve intensidad, sin irradiación, concomitante presente episodio de pérdida de conocimiento, seguido de hemoptisis; motivo por el cual se inician los estudios correpondientes y en la TAC de tórax se evidencia "extensa lesión neoproliferativa de mediatino antero-superior y lateral izquierdo"; por lo que es referido al IOLR el 17-11-95, ingresando por admisión el 05-12-95. Antecedentes de importancia: pérdida de 10 kg. en 2 meses, hábitos tabáquicos acentuados. El examen de ingreso mostraba un paciente en RsCsGs, una adenopatía de 1 cm en la región yugular superior izquierda y otra de 1 cm en la región yugular superior derecha, las fosas supraclaviculares y las axilas eran negativas, los Rs Res Ptes en As hemotórax y disminuidos en As bases sin agregados, quistes de epidimo derecho, Ptiriasis simple. Es evaluado por el Servicio de Cirugía de Tórax, en enero 96 deciden hospitalizarlo para tratamiento quirúrgico, el 23-1-96 se le practica Esternotomía media y el tumor es considerado irresecable, por su relación con los grandes vasos, se tomó biopsia. Evaluado en conferencia del Sercicio de MIO 02-2-96 se decide, solicitar marcadores tumorales, Inmunohistoquímica e iniciar quimioterapia con esquema BEP. Los marcadores fueron negativos, y se realizó inmunohistoquímica, pero la revisión de láminas reportó "Tu. Germinal, Seminoma". El 11-2-96 presentó Sx. Compresión Vena Cava Superior, iniciándose el tratamiento en día 12-2-96. mejorando notablemente y evolucionando satisfactoriamente. El día 27-2-96 presenta neutropenia febril, instaurándose tratamiento, a pesar de no existir foco de infección, con Cefoperazona-Sulbactam, Amikacina y Fluconazol, 24 horas después el paciente presenta alteración del estado de coincidencia, Glasgow 3, y rigidez de nuca, falleciendo posteriormente

A rare paratesticular pleomorphic rhabdomyosarcoma.

A paratesticular pleomorphic rhabdomyosarcoma occurring in a 81-year old man is reported. The unusual clinical presentation and pathological features and the differential diagnosis are discussed. Difficulties encountered in diagnosis are emphasised.

Fibrohistiocitoma maligno do cordäo espermático / Malignant fibrous histiocytoma of the spermatic cord

Os autores relatam um caso de fibrohistiocitoma maligno do cordäo espermático em um homem de 65 anos, com história de um nódulo em regiäo inguinal direita indolor e endurecido com 4 meses de evoluçäo, tratado com dois eventos cirúrgicos. O primeiro com ressecçäo da massa tumoral e 6 meses após com nova exploraçäo cirúrgica por haver recidiva. O tratamento complementar consistiu em orquiectomia direita com hemiescrotectomia direita e esvaziamento inguino-crural direito até o anel inguinal. Foi planejado e executado curso de telecobaltoterapia com 5000 em 5 semanas, ficando o paciente desde a conclusäo da radioterapia em 12/10/93 livre de doença