Siringocistoadenoma papilífero: reporte de 11 casos del Hospital Clínico de la Universidad de Chile / Papilliferum Syringocystoadenoma: report of 11 cases of Clinical Hospital of the University of Chile

Introducción: El siringocistoadenoma papilífero (SP) es un tumor anexial benigno inusual, de etiología desconocida. Por lo general, se presenta desde el nacimiento, o en la primera infancia como una lesión aislada, frecuentemente asociada a un nevo sebáceo de Jadassohn. Objetivo: Describir y analizar las características demográficas, clínicas e histopatológicas del SP. Materiales y métodos: Revisión retrospectiva de informes histopatológicos del Departamento de Anatomía Patológica del Hospital Clínico de la Universidad de Chile entre los años 2005 y 2016. Se incluyeron todas las muestras que describieron dentro del diagnóstico histológico las palabras "siringocistoadenoma papilífero". Resultados: La muestra está compuesta por 11 pacientes; 9 mujeres y 2 hombres. En 5 pacientes, se desarrolló un SP a partir de un nevo sebáceo y en 6 surgió de novo. El total de la muestra desarrolló el SP durante o posterior a la pubertad. Tanto los pacientes con SP solitario, como aquellos asociados a un nevo sebáceo, presentaron clínica similar con una placa o pápula verrucosa aislada. Los principales diagnósticos diferenciales fueron el nevo sebáceo, nevo verrucoso y verruga. Conclusiones: Este estudio constituye la primera serie de casos de SP en un hospital en Santiago de Chile. La forma clínica más frecuente fue como placa verrucosa solitaria asintomática, con casos aislados como pápula verrucosa y sólo un caso con aspecto de cuerno cutáneo. Se destaca el rol de la extirpación quirúrgica completa, tanto para establecer el diagnóstico, como para ofrecer un tratamiento definitivo, disminuyendo el riesgo de transformación maligna a siringocistoadenocarcinoma papilífero.

Introduction: Syringocystoadenoma papilliferum (SCAP) is an unusual benign adnexal tumor of unknown etiology. Usually it is presented at birth or in early childhood as an isolated lesion associated with nevus sebaceous of Jadassohn. Objective: To describe and analyze the demographic, clinical and histopathological characteristics of SCAP. Materials and methods: A retrospective review of histopathological reports was performed at the Clinical Hospital´s Department of Pathological Anatomy from the University of Chile including biopsies from 2005 to 2016. All the samples that described the histological diagnosis of "syringocystoadenoma papilliferum" were included. Results: The sample consists of 11 patients; 9 women and 2 men. In 5 patients, an SCAP was developed from a sebaceous nevus and in 6 it developed de novo. The total sample developed SCAP during or after puberty. Both the patients with solitary SCAP, and those associated with nevo sebaceous, presented similar clinical signs with an isolated verrucous plaque or papule. The main differential diagnoses were the nevo sebaceous, warty nevus and wart. Conclusions: This study is the first SCAP case series in Santiago, Chile. The most frequent clinical form was an asymptomatic solitary verrucous plaque, with isolated cases as a verrucous papule and a single case as a cutaneous horn. The role of complete surgical resection is highlighted, both to establish the diagnosis and to offer a definitive treatment, reducing the risk of malignant transformation to syringocystadenocarcinoma papilliferum.

Syringocystadenoma papilliferum combined with a tubular apocrine adenoma

Abstract: Syringocystadenoma papilliferum and tubular apocrine adenoma are rare benign sweat gland tumors. Syringocystadenoma papilliferum occurs alone or in association with other tumors. Although it is rare, the association of tubular apocrine adenoma with syringocystadenoma papilliferum developing in a sebaceous nevus on the scalp is well documented. However, the co-existence of these two tumors without the background of a sebaceous nevus has not been frequently reported. Syringocystadenoma papilliferum and tubular apocrine adenoma may have a histopathological overlap, but a few cases of a syringocystadenoma papilliferum combined with a tubular apocrine adenoma have been reported. Herein we describe an unusual case of syringocystadenoma papilliferum co-existing with a tubular apocrine adenoma located on the back of a 14-year-old patient in the absence of a pre-existing sebaceous nevus.

Syringocystadenoma papilliferum in an unusual location

Abstract: Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumor of the apocrine or eccrine sweat glands. Most patients present a solitary lesion in the head and neck region. Presentation outside the head and neck region is even more uncommon. We present a case of Syringocystadenoma papilliferum with papulonodular lesion located on the vulva of an infant girl. This case illustrates the atypical location of this rare disease and adds to the differential diagnosis of lesions on the vulva.

Giant multicystic cystadenoma of Cowper's gland: a case report

Main findings We report what to our knowledge is the first case of a giant multicystic cystadenoma of the Cowper's glands. An otherwise healthy 41-year-old man presented with acute urinary retention. Physical examination showed a perineal mass. Different imaging techniques demonstrated a multicystic tumor and en bloc excision was performed. Histological evaluation showed that the tumor arised from the bulbourethral glands; immunohistochemistry proved positive staining for high molecular weight cytokeratin. Case hypothesis Cystic tumors in the pelvis can arise from different structures. Malignancy should be ruled out. Surgical excision can be diagnostic and curative. Future implications When evaluating a pelvic cystic tumor, Cowper's glands cystadenoma may be a differential diagnosis and must be considered. Similar to prostate cystadenomas, en bloc excision is the optimal treatment. .

Histopathological Study of Ovarian Tumors.

The ovarian tumors show wide variation in the clinical and morphological features. The incidence also varies in different parts of the world. This is a histopathological study of 100 ovarian tumors over a period of 5 years in our institute. The ovarian tumors were classified according to WHO Classification. The aim of this work was to study the incidence and gross and microscopic features of different benign and malignant ovarian tumors. Benign tumors were more common than malignant tumors. Among benign tumors serous cystadenoma (41.89%) was the most common followed by mucinous cystadenoma (29.72%) and mature cystic teratoma (16.21%). Among malignant ovarian tumors, the most common type was serous cystadenocarcinoma (38.46%) followed by dysgerminoma (11.53%) and clear cell carcinoma (11.53%). Majority of the ovarian tumors were surface epithelial tumors (77%) followed by germ cell tumors (16%) and sex cord stromal tumors (6%).

Aspectos dermatoscópicos do siringocistoadenoma papilífero associado a nevo sebáceo / Dermoscopic aspects of syringocystadenoma papilliferum associated with nevus sebaceus

O siringocistoadenoma papilífero é uma neoplasia anexial benigna rara, com frequente diferenciação apócrina. Localiza-se preferencialmente no couro cabeludo e está associado ao nevo sebáceo em 40 por cento dos casos. Apesar da variabilidade clínica, a histologia é característica. Há relatos da dermatoscopia de tumores anexiais, como poroma écrino, hidradenoma e angio-histiocitoma; porém, até o momento, não há descrição da dermatoscopia do siringocistoadenoma. Apresentamos aspectos dermatoscópicos de um caso de siringocistoadenoma associado a nevo sebáceo, visualizando-se padrão vascular polimorfo e vasos em ferradura.

Syringocystadenoma papilliferum is a rare benign adnexal tumor that frequently shows apocrine differentiation. It usually develops on the scalp and is associated with a nevus sebaceus in 40 percent of cases. Although the clinical presentation may differ, its histology is characteristic. Reports have been made of dermoscopy used in cases of adnexal tumors such as eccrine poromas, hidradenomas and angiohistiocytomas; however, up to the present moment there have been no reports of dermoscopy in a case of syringocystadenoma. This paper describes the dermoscopic features found in a case of syringocystadenoma associated with a nevus sebaceus, revealing a polymorphous vascular pattern including a horseshoe-shaped arrangement of vessels.

Acinar Cell Cystadenoma (Acinar Cystic Transformation) of the Pancreas: the Radiologic-Pathologic Features

Acinar cystic transformation of the pancreas is also known as acinar cell cystadenoma (ACC), and this is an extremely rare benign lesion that was first described in April 2002. We report here on a case of a previously asymptomatic patient with pancreatic ACC and this was diagnosed by computed tomography (CT) and magnetic resonance imaging (MRI). To the best of our knowledge, there is no previous report concerning the CT or MRI features of ACC in the medical literature. We present here the CT, MRI and pathological findings of pancreatic ACC.

Neoplasias quísticas pancreáticas / Cystic neoplasms of the pancreas

BACKGROUND: Cystic neoplasms of the pancreas represent between 5% and 10% of all pancreatic tumors. Due to their malignant potential, surgery is indicated. Because of the uncommon nature of this pathology, the aim of this study was to present a series of six patients who were operated on for a cystic tumor and to analyze the morphological characteristics, surgical experience and follow-up. METHODS: This was a retrospective study. Clinical files were studied, as well as surgical and pathological registries from patients diagnosed with cystic neoplasms of the pancreas. Five females and one male were included with a mean age of 54 +/- 15 years. The main symptom was abdominal pain. RESULTS: Clinical course and CT scan results were the criteria for suspecting the diagnosis. In 4/6 patients, the tumor was located within the body and tail of the pancreas. The most frequent intervention was distal pancreatectomy. There was no mediate operative mortality in a 30-day period. Histopathological diagnoses were three serum cystadenomas, two mucinous cystadenomas and one cystadenocarcinoma. After a 4-year follow-up, no tumor recurrences have been reported in the surviving patients. CONCLUSIONS: The natural history of cystic neoplasms of the pancreas is generally benign, but complications may occur by direct invasion of neighboring structures. Clinical course, tomography, pancreatography and punction are fundamental for its treatment.

Thirteen Cases of Intrahepatic Biliary Cystadenoma and Cystadenocarcinoma: A Single Center Experience / 대한소화기학회지

BACKGROUND/AIMS: Biliary cystadenoma (BCA) and biliary cystadenocarcinoma (BCACa) are rare cystic neoplasms that usually arise from the liver. We reviewed the clinicopathologic and radiologic findings of 13 cases of intrahepatic biliary cystic neoplasms. METHODS: Seven patients with BCA and 6 patients with BCACa which were pathologically proven within past 10 years were included in this retrospective study. RESULTS: BCA (7 of 7) was more common in female compared to BCACa (4 of 6). Mean age at diagnosis was 53.4 years (BCA) and 58.5 years (BCACa). Abdominal pain (54%) was the most common presenting symptom. Eleven patients (61.5%) exhibited normal liver function profiles and 5 patients (38%) showed elevated levels of serum CA19-9 levels (mean 894.2 U/mL, range: 78.7-2,080). Mean size of tumor was 11.7 cm (range: 5-15). Most frequent radiologic finding was a single cystic mass with septation. BCACa tended to have intracystic solid portion. The cut surface revealed a unilocular or multilocular cystic mass with mucinous contents. Complete surgical excision was done in 12 patients. After the complete resection, recurrence was observed in 1 case of BCACa. CONCLUSIONS: The possibility of biliary cystic neoplasm should be suspected when an intrahepatic cystic lesion with multiseptation or solid portion is noted on imaging study. In addition, complete excision for definite diagnosis and treatment need to be performed.

Image-directed percutaneous FNAC of ovarian neoplasms.

Fine needle aspiration of ovarian neoplasms is a relatively less frequented area of diagnostic aspiration cytology. Hence, an attempt has been made in the present study to evaluate the current status of Image-directed percutaneous needle aspiration in ovarian neoplasms and to assess its value and limitations in the diagnosis and management of ovarian neoplasms. The present study involves 105 cases of ovarian neoplasms, which were assessed by ultrasound-guided FNAC. Only those cases with adequate material on aspiration and availability of cellblock or biopsy material following FNAC for correlative histopathological study were included in the study. The diagnostic accuracy of ovarian neoplasms in the present study was 89.85%, with a false negative rate of 4.76%. Considering the rapidity and reliability of the procedure with its added advantages like cost-effectiveness and increased patient acceptability, it can be concluded that image-guided FNAC holds a key position in the diagnosis and management of ovarian neoplasms.

Tumor sólido-cístico de pâncreas. Relato de caso / Solid cystic tumor of pancreas. Case report

O tumor sólido cístico de pâncreas é uma entidade rara que acomete indivíduos jovens do sexo feminino. A sintomatologia é discreta. O tumor é de tamanho variável (2 a 10 cm) e coberto por uma cápsula fibrosa. Ao contrário do adenocarcinoma de células ductais do pâncreas, que é o tumor mais comum, este é de crescimento lento, raramente é acompanhado de metástases e tem bom prognóstico. Relato de um caso e revisäo da doença.

Pseudomixoma peritoneal y mucocele apendicular secundario a cistadenoma y cistadenocarcinoma del apéndice cecal. Informe de dos pacientes / Peritoneal pseudomyxoma and appendicular mucocele due to cystadenoma and cystadenocarcinoma of the cecal appendix: Report of two patients

Objetivo. Informar nuestra experiencia con dos enfermos de mucocele del apéndice cecal, secundario uno a cistadenocarcinoma y el otro a cistadenoma mucinosos. Sede Hospital General de Zona, segundo nivel de atención médica. Descripción de los casos. Se trató de dos pacientes del sexo femenino, de 54 años de edad, a quienes se les practicó cirugía urgente por abdomen agudo secundario a apendicitis aguda complicada. Las dos presentaron apéndice cecal con gran dilatación, perforación y múltiples adherencias interasa debido a la reacción plastica asociada: en ambas se realizó hemicolectomía derecha con ileostomía debido a que el ciego y colon ascendente se encontraban involucrados. La evolución fue defierente en cada enferma, una de las pacientes requirió de dos reintervenciones quirúrgicas por sepsis residual. El estudio histopalológico informó en una paciente cistadenoma mucinoso y en la otra cistadenocarcinoma también mucinoso. Discusión. El mucocele apendicular es una entidad poco frecuente, está asociado a la formación de quistes de retención, que se generan a partir de neoplasias productoras de mucina como los que se presentan en este informe, las complicaciones incluyen: pseudomixoma y la presencia de gran reacción peritoneal con múltiples adherencias. El tratamiento consiste generalmente en la apendicectomía, sin embargo, en algunos pacientes debe asociarse la hemicolectomía con ileostomía por invasión directa al ciego y colon. Conclusión. Se trata de un problema de aparación espóradica que en muchas ocasiones resulta grave por dificulatades técnicas y manejo quirrúgico

Ovarian serous cystadenoma associated with Sertoli-Leydig cell tumor: a case report

We Describe a case of ovarian serous cystadenoma having Sertoli-Leydig cell tumor, well differentiated, in the cystic septum. Well differentiated Sertoli-Leydig cell tumor coexisting with other tumor, including serous tumor, has not yet been described. In all cases of Sertoli-Leydig cell tumor with heterologous components or other tumors, the androblastomatous components are intermediately or poorly differentiated. The present case revealed a well differentiated Sertoli-Leydig cell tumor arising in a septum of serous cystadenoma, as a circumscribed nodule. With these findings, we discuss the possibility of this Sertoli-Leydig cell tumor, considered a mural nodule, which is well established in cystic common epithelial tumors of the ovary.

Multilocular cystadenoma of the liver with mural nodules.

Hepatobiliary cystadenoma, characterised by the interposition of a mesenchymal stroma beneath the epithelium and the connective tissue layer, is a tumor described exclusively in women. These tumors have a potential for malignant transformation. Complete surgical excision is mandatory. We report a case cystadenoma with mesenchymal stroma (CMS), with mural nodules in the cyst wall, similar to those described in ovarian mucinous cystadenoma.

Anaesthetic management in removal of a huge ovarian cyst.

A patient with a huge ovarian cyst weighing 38 kg was operated successfully with utmost care to avoid all possible intra and postoperative complications. The same has been presented and the salient features of management have been discussed.

Cistadenoma seroso del ligamento ancho de bajo potencial maligno / Serous cystadenoma in the broad ligament of low malignant potential

Se presenta un caso de cistadenoma seroso papilar de bajo potencial maligno localizado en el ligamento ancho, en una mujer de 43 años de edad. La localización de este tumor no es usual y sólo 12 casos han sido reportados en la literatura internacional