Résumé
Perianal Paget's disease (PPD) is a rare malignant cutaneous tumor. This paper reported a case of PPD complicated by lung adenocarcinoma and anal canal cancer. The patient, a 76-year-old female, had been experiencing recurrent lower abdominal pain and perianal pruritus for the past 5 years. Upon physical examination, a cauliflower-like neoplasm in size of 5 cm×6 cm was observed on the right perianal skin, with local skin ulceration and a small amount of fluid discharge. The left perianal skin was also involved. In thoracoknee position, a hard mass was palpable in the rectal submucosa at 5-6 points 2 cm from the anal verge. Chest CT revealed multiple lesions in both lungs, indication of metastatic tumors. Further evaluation with fluorodeoxyglucose positron emission tomography and computed tomography (FDG-PET/CT) indicated multiple hypermetabolic nodules in the lungs, hypermetabolic lymph nodes throughout the body, early FDG uptake in a small patch of skin on the left hip, and increased FDG uptake in the anorectal region. Histopathological examination confirmed the diagnosis of lung adenocarcinoma. This resulted in the patient being diagnosed with PPD, lung adenocarcinoma, anal canal cancer, and systemic multiple lymph node metastasis. The combination of PPD with gastrointestinal tumors and other metachronous malignant tumors is highly prevalent. Colonoscopy, FDG-PET/CT, histopathology, and immunohistochemistry play crucial roles in early identification of local lymph node and distant involvement, facilitating the evaluation of potential malignant tumors and differential diagnosis. Treating methods for PPD are currently diverse, including postoperative combined or single chemotherapy, radiotherapy, targeted therapy, and photodynamic therapy. As trerapeutical options continue to develop, the extent and efficacy of surgery need to be reassessed.
Sujets)
Femelle , Humains , Sujet âgé , Maladie de Paget extramammaire/anatomopathologie , Fluorodésoxyglucose F18 , Tomographie par émission de positons couplée à la tomodensitométrie , Adénocarcinome pulmonaire/complications , Tumeurs du poumon/complicationsRésumé
Abstract Currently, pagetoid dyskeratosis is believed to involve an accelerated keratinization process, possibly induced by mechanical trauma. It represents, in almost its totality, incidental histological findings of specific cells, except when it occurs in the hands, where it usually occurs simultaneously with skin lesions and local dyschromia. These are large, rounded keratinocytes, with pale cytoplasm and a pyknotic nucleus surrounded by a clear halo, which can be easily mistaken by other skin diseases. Its etiology is not completely elucidated, and the correct identification of this entity can be of great importance in the differential diagnosis of skin disorders and the understanding of the keratinization process of the epidermis.
Sujets)
Tumeurs cutanées , Épithélioma in situ , Maladie de Paget extramammaire , Kératinocytes , ÉpidermeRésumé
@#Extramammary Paget’s disease (EMPD) of the vulva is a rare vulvar neoplasm but commonly arises during the postmenopausal period. Intraepithelial Paget’s disease may persist for prolonged periods without demonstrating invasion but with high rates of recurrence. Appearance of Paget’s disease in a split-thickness skin graft, is associated with an occurrence outside the grafted area. It demonstrates retrodissemination as the pathologic process hypothesized in the spread of the disease within the skin via lymphatics and vessels creating tissue bridges between sites of involvement. We present a case of an 81-year-old female, the patient came in for complaints of vulvar pruritus beginning at the left inguinal area three years prior to her diagnosis. She consulted with a dermatologist and was initially treated with steroids and emollients. Persistence of symptoms and enlargement of the lesion prompted a vulvar punch biopsy which showed Paget’s disease and referral to the Gynecologic Oncology service. Wide local excision with split-thickness skin grafting was performed. However, one year after her surgery, patient noted vulvar pain and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Patient underwent repeat wide local excision with frozen section, and split-thickness skin grafting. With the aid of frozen section, the intraepithelial involvement was noted to spread beyond the grossly apparent lesion. After 6 months post re-excision, patient noted vulvar pruritus and palpable vulvar lesions. Biopsy was done which showed Extramammary Paget’s Disease recurrence. Due to the proximity of the lesion to the sphincter and need for a colostomy, the patient did not consent for re-excision. Imiquimod 5% was chosen as the mode of treatment. The challenges of interventions are to remove or treat disease that may not be visible, without overtreatment and to minimize morbidity from radical surgery. Surgery remains the primary management for EMPD of the vulva. Imiquimod 5% can be used in recurrences. Despite the advances in the knowledge and management of vulvar Paget’s disease the high rate of recurrent disease remains a challenge for optimal management and would require frequent and long-term follow-up.
Sujets)
Femelle , Maladie de Paget extramammaire , Tumeurs de la vulveRésumé
ABSTRACT Paget's disease most commonly affects the breast. Extramammary involvement is rare, and the most commonly affected sites are the vulva, anus, perianal region, and axilla. The disease may progress to invasive adenocarcinoma or synchronous cancers. Due to the lack of distinctive features and nonspecific presenting symptoms, Paget's disease may be misdiagnosed as other conditions, thus delaying the correct diagnosis. We report a case of extramammary Paget's disease in the perianal region that initially presented as an irregular, circumferential, scaling lesion with eczematous eruptions. Immunology and immunohistochemistry confirmed the diagnosis. Although surgery is the standard treatment, the patient opted for pelvic radiotherapy associated with radiosensitizing chemotherapy.
RESUMO A doença de Paget acomete mais comumente a mama. Os focos extramamários são raros e os locais mais habitualmente acometidos são vulva, ânus, região perianal e axila. A patologia envolve a evolução da doença para adenocarcinoma invasivo ou neoplasias sincrônicas. Devido à singularidade e sua aparência inespecífica, a doença pode ser confundida com outras comorbidades, retardando o diagnóstico. O objetivo foi relatar um caso de doença de Paget extramamária na região perianal, a qual inicialmente apresentou lesão circunferencial com erupção eczematosa, descamativa e irregular. A confirmação diagnóstica foi por meio de exames imuno-histoquímico e imunológico. O tratamento padrão da doença é cirúrgico, porém optou-se por radioterapia pélvica associada à quimioterapia radiossensibilizante.
Sujets)
Humains , Femelle , Tumeurs de l'anus , Maladie de Paget extramammaire/traitement médicamenteux , Radiothérapie , Tumeurs cutanées , Maladie de Paget extramammaire/diagnostic , Maladie de Paget extramammaire/anatomopathologieRésumé
Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.
Sujets)
Humains , Femelle , Sujet âgé , Tumeurs de la vulve/anatomopathologie , Maladie de Paget extramammaire/anatomopathologie , Vulvectomie , Imiquimod/usage thérapeutique , Récidive tumorale locale/anatomopathologie , Antinéoplasiques/usage thérapeutique , Tumeurs de la vulve/thérapie , Résultat thérapeutique , Maladie de Paget extramammaire/thérapie ,Résumé
Pagetoid Bowen disease is a histological variant of Bowen disease which demonstrates large pale staining cells (pagetoid cells). It requires differential diagnosis from other cutaneous malignancies with similar patterns, such as extramammary Paget's disease (EMPD) and Pagetoid melanoma in situ. Herein, we report a case of Pagetoid Bowen disease which was initially misdiagnosed as ectopic EMPD.
Sujets)
Maladie de Bowen , Diagnostic différentiel , Immunohistochimie , Mélanome , Maladie de Paget extramammaireRésumé
Primary cutaneous mucinous carcinoma (PCMC) is an uncommon tumor of the sweat gland origin. The occurrence of PCMC is mostly in middle-aged and older patients, with a slight male predominance. Most cases of PCMC arise on the head, with a preference for eyelids. The histogenesis of PCMC, whether eccrine or apocrine, remains controversial. We report a rare case of PCMC with secondary extramammary Paget’s disease in the groin of a 75-year-old man, which favored an apocrine origin. Furthermore, based on a review of the literature, we provide several histologic clues that can be used to differentiate PCMC from metastatic mucinous carcinoma.
Sujets)
Sujet âgé , Humains , Mâle , Adénocarcinome mucineux , Paupières , Aine , Tête , Mucines , Maladie de Paget extramammaire , Glandes sudoriparesRésumé
Ectopic extramammary Paget's disease (EMPD) is a rare variant of EMPD that develops in non-apocrine regions. We present a 70-year-old man in whom ectopic EMPD affected the lower back. Initially, erythematous scaly plaques with several papules on the back were suggestive of Bowen's disease. However, the biopsy specimen and immunohistochemical study results were consistent with EMPD features. The lesion was resected with a 1-cm safety margin and covered with a full thickness local skin graft. Ectopic EMPD is so rare that only few cases of EMPD in a non-apocrine region have been reported. We considered this case of EMPD on the back to be interesting since there has been no previous case reported in this location in Korea.
Sujets)
Sujet âgé , Humains , Biopsie , Maladie de Bowen , Corée , Maladie de Paget extramammaire , Peau , TransplantsRésumé
No abstract available.
Sujets)
Canal anal , Maladie de Paget extramammaire , Radiothérapie , VulveRésumé
Pigmented extramammary Paget's disease (PEMPD) is an uncommon intraepithelial adenocarcinoma and a rare variant of Paget's disease, characterized as a superficial pigmented scaly macule clinically and an increased number of melanocytes scattered between the Paget's cells histologically. So it may be confused clinically and histologically with melanocytic tumors, dermatitis and other dermatoses. Different therapeutic attitudes are required in this case of adenocarcinoma in situ as opposed to melanoma and dermatitis. Condyloma acuminatum (CA) is a common sexually transmitted disease caused by human papilloma virus infection, which is also called as genital warts. In this article, we first reported a case of a 65-year-old Chinese man who had pigmented extramammary Paget's disease complicated with CA. This patient presented with verrucous papules on the scrotum for 3.5 years, infiltrative erythema with itch on the mons pubis for 3 years, and scrotum and penis involved gradually for 4 months. Physical examination showed a 8 cm×10 cm dark red patch on the upper part of the scrotum, penis and mons pubis, as well as few maculopapules and nodules. Histopathologic examination of the lesion on the scrotum revealed a focus of Paget's disease, characterized by the presence of large round cells with abundant pale or granular/dusty cytoplasm, pleomorphic vesicular nuclei and prominent nucleoli (Paget's cells), while the histology of the verrucous lesion was consistent with CA. Immunohistochemistry was performed, which showed diffuse positive staining with CK, CEA, PAS, CK20, EMA, CK7, and Ki-67 (40%), HER2 in Paget's cells and negative with P53, P16, CK5/6, S100, MelanA, HMB45, estrogen receptor, progesterone receptor, and gross cystic disease flid protein 15 (GCDFP15). Human papillomavirus-11 (HPV-11) was positive by genotyping using gene amplification in the lesion of scrotum. According to clinical features and laboratory findings, a diagnosis of PEMPD complicated with CA was made. Local excision of the lesion was performed and sent for histological examination, with all margins clear of tumor. Both aforementioned diseases often occur in the vulva. Even so, it has been rarely reported coexisting of the above two diseases, of which the clinical significance and association are also unclear. In this article, we also reviewed the literature relating to PEMPD, and on this basis, the profile of this disease is discussed including its pathogenesis, clinical manifestation, diagnosis, treatment and advances. Due to PEMPD occasionally accompanied with an underlying carcinoma, it's essential to make an accurate diagnosis. Besides, review of the literature reveals that pigmented variant of Paget's disease could be initially misdiagnosed as melanocytic tumors and other dermatoses unless the entity is considered in the differential diagnosis and additional confirmatory studies are performed.
Sujets)
Sujet âgé , Humains , Mâle , Condylomes acuminés/complications , Diagnostic différentiel , Immunohistochimie , Mélanome , Maladie de Paget extramammaire/anatomopathologie , Pénis , Scrotum/anatomopathologieRésumé
La Enfermedad de Paget extramamaria de la vulva es una neoplasia poco frecuente que representa solo el 1% de los cánceres de vulva. Clínicamente, se caracteriza por una placa eritematosa, bien definida, con erosiones y ulceraciones. El síntoma más frecuente es el prurito. Esta clínica tan inespecífica puede retrasar el diagnóstico, además de inducir a tratamientos inadecuados. El diagnóstico se realiza con estudio histopatológico e inmunohistoquímica, la cual orienta si es primario de piel o metástasis de otro cáncer, usualmente genitourinario o gastrointestinal subyacente. Actualmente no hay guías clínicas de tratamiento, pero dentro de las opciones la más aceptada es la cirugía de Mohs. Otros tratamientos incluyen uso de imiquimod, láser CO2, quimioterapia tópica o local, radioterapia o terapia fotodinámica. Cuando se trata de una enfermedad de Paget extramamaria primaria, el pronóstico es excelente y la mayoría de los pacientes fallece por otra condición. En cuanto a la enfermedad invasora, tiene una mortalidad de 13-18% con sobrevida a 5 años de 72% (1)
Sujets)
Humains , Femelle , Sujet âgé , Tumeurs de la vulve/diagnostic , Tumeurs de la vulve/anatomopathologie , Maladie de Paget extramammaire/diagnostic , Maladie de Paget extramammaire/anatomopathologieRésumé
Clear cell papulosis (CCP) is a rare skin condition characterized by asymptomatic multiple hypopigmented maculopapules distributed primarily on the lower aspect of the abdomen or along the milk lines in young children. The characteristic histological feature of CCP is the presence of clear cells scattered among basal keratinocytes. We describe the case of a 3-year-old girl with clear cell papulosis. Immunohistochemical staining results were variable based on the different kinds of CEA antibodies. Immunostaining specification of CEA glycoproteins may provide a clue for the differential diagnosis of clear cell papulosis with other diseases such as primary extramammary Paget's disease.
Sujets)
Enfant , Enfant d'âge préscolaire , Femelle , Humains , Abdomen , Anticorps , Antigène carcinoembryonnaire , Diagnostic différentiel , Glycoprotéines , Immunohistochimie , Kératinocytes , Lait , Maladie de Paget extramammaire , PeauRésumé
Clear cell papulosis (CCP) is a rare skin condition characterized by asymptomatic multiple hypopigmented maculopapules distributed primarily on the lower aspect of the abdomen or along the milk lines in young children. The characteristic histological feature of CCP is the presence of clear cells scattered among basal keratinocytes. We describe the case of a 3-year-old girl with clear cell papulosis. Immunohistochemical staining results were variable based on the different kinds of CEA antibodies. Immunostaining specification of CEA glycoproteins may provide a clue for the differential diagnosis of clear cell papulosis with other diseases such as primary extramammary Paget's disease.
Sujets)
Enfant , Enfant d'âge préscolaire , Femelle , Humains , Abdomen , Anticorps , Antigène carcinoembryonnaire , Diagnostic différentiel , Glycoprotéines , Immunohistochimie , Kératinocytes , Lait , Maladie de Paget extramammaire , PeauRésumé
Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. A 65-year-old female and 78-year-old male patients visited our institution because of an unhealed perianal skin lesion despite treatment for a long period with topical agents. PPD was diagnosed by skin biopsies in both cases, and the patients underwent surgical treatment. Clinical manifestations, preoperative work-ups, and surgical treatments including different reconstruction methods are described in detail. As only sporadic PPD cases have been reported and no standard treatment has been established, we hope that our experience could contribute to improving the diagnosis and treatment of PPD patients.
Sujets)
Sujet âgé , Femelle , Humains , Mâle , Biopsie , Diagnostic , Espoir , Maladie de Paget extramammaire , PeauRésumé
Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. A 65-year-old female and 78-year-old male patients visited our institution because of an unhealed perianal skin lesion despite treatment for a long period with topical agents. PPD was diagnosed by skin biopsies in both cases, and the patients underwent surgical treatment. Clinical manifestations, preoperative work-ups, and surgical treatments including different reconstruction methods are described in detail. As only sporadic PPD cases have been reported and no standard treatment has been established, we hope that our experience could contribute to improving the diagnosis and treatment of PPD patients.