Anatomic Subtype Differences in Extramammary Paget Disease: A Meta-Analysis.

Importance: Extramammary Paget disease (EMPD) is a rare, highly recurrent cutaneous malignant neoplasm of unclear origin. EMPD arises most commonly on the vulvar and penoscrotal skin. It is not presently known how anatomic subtype of EMPD affects disease presentation and management. Objective: To compare demographic and tumor characteristics and treatment approaches for different EMPD subtypes. Recommendations for diagnosis and treatment are presented. Data Sources: MEDLINE, Embase, Web of Science Core Collection, and Cochrane Reviews CENTRAL from December 1, 1990, to October 24, 2022. Study Selection: Articles were excluded if they were not in English, reported fewer than 3 patients, did not specify information by anatomic subtype, or contained no case-level data. Metastatic cases on presentation were also excluded. Data Extraction and Synthesis: Abstracts of 1295 eligible articles were independently reviewed by 5 coauthors, and 135 articles retained. Reporting was in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guidelines. The analysis was cunducted in August 2019 and updated in November 2022. Findings: Most vulvar EMPD cases were asymptomatic, and diagnosis was relatively delayed (mean, 25.1 months). Although most vulvar EMPD cases were intraepidermal (1247/1773 [70.3%]), radical surgeries were still performed in almost one-third of cases. Despite this aggressive surgical approach, 481 of 1423 (34%) recurred, commonly confined to the skin and mucosa (177/198 [89.4%]). By contrast, 152 of 1101 penoscrotal EMPD cases (14%) recurred, but more than one-third of these recurrences were regional or associated with distant metastases (54 of 152 [35.5%]). Perianal EMPD cases recurred in one-third of cases (74/218 [33.9%]), with one-third of these recurrences being regional or associated with distant metastasis (20 of 74 [27.0%]). Perianal EMPD also had the highest rate of invasive disease (50% of cases). Conclusions and Relevance: The diagnosis and treatment of EMPD should differ based on anatomic subtypes. Considerations for updated practice may include less morbid treatments for vulvar EMPD, which is primarily epidermal, and close surveillance for local recurrence in vulvar EMPD and metastatic recurrence in perianal EMPD. Recurrences in penoscrotal subtype were less common, and selective surveillance in this subtype may be considered. Limitations of this study include the lack of replication cohorts and the exclusion of studies that did not stratify outcomes by anatomic subtype.

Image intensifier-guided transperineal prostate biopsy for patients without a rectum: novel technique.

INTRODUCTION: It is challenging to perform prostate biopsy in men with suspicion of prostate malignancy without a rectum to facilitate prostate biopsy. Nevertheless, such patients are presenting at an earlier stage, due to increased PSA testing in association with improved MRI imaging. We describe a novel technique for prostate biopsy in two such cases. TECHNIQUE: The patient is under General Anaesthesia and in lithotomy position. The patient is catheterised and a measured volume of contrast is inserted to the catheter balloon. By using anatomical surface landmarks, placing traction on the catheter to bring the balloon to the level of the bladder neck and using fluoroscopy, the distance to the apical prostate was estimated. This facilitates image intensifier guided trans-perineal prostate biopsy. OUTCOMES: A 67-year-old patient, with a history of panproctocolectomy for ulcerative colitis, presented with increasing PSA and a suspicious prostate on MP-MRI. The prostate couldn't be visualised on transperineal ultrasound and the patient was offered transperineal biopsy using image intensifier guidance. Several biopsy cores were taken and prostate cancer was diagnosed. The second patient was a 68-year-old who presented similarly, but with a history of panproctocolectomy for Crohn's disease. Using the above technique, biopsies were taken with low-risk prostate cancer diagnosed. Subsequently, due to rising PSA levels, a repeat set of prostate biopsies was taken 13 months later in an identical manner, upstaging his disease. There were no post-operative complications after any of the procedures. CONCLUSION: We review the literature and discuss several techniques available to sample the prostate in this patient cohort. We conclude that we have identified a safe and effective technique, which utilises commonly available equipment, to biopsy the prostate in the post proctectomy patient.

Transitioning from transrectal to transperineal prostate biopsy using a freehand cognitive approach.

OBJECTIVES: To report a single-centre experience of a complete transition from transrectal (TR) to transperineal (TP) prostate biopsy under local anaesthesia using a freehand cognitive coaxial approach and without use of antibiotic prophylaxis. PATIENTS AND METHODS: Analysis was performed of a prospective database of patients undergoing prostate biopsy performed by four surgeons between 1 June 2018 and 31 May 2022. Outcomes of interest were complications, cancer detection rate, inter-operator reliability, and tolerability. RESULTS: Overall, 1915 patients underwent 2337 separate prostate biopsy sessions. Only 2.4% patients in the TP group received antibiotic prophylaxis, while 100% received antibiotics in the TR group. The complication rate was significantly lower in the TP group compared to the TR group (0.3% vs 5.0%, P < 0.001). In contrast to the TR group, there were no cases of urosepsis or admissions to intensive care in the TP group. The total cancer detection rate by TP biopsy was 70% and the overall pathology detection rate was 88.4%. There was no difference in cancer or pathology detection between operators. A stable level of cancer detection was reached early on for both Prostate Imaging-Reporting and Data System 4 and 5 lesions. All cases performed were performed successfully without need for early termination. CONCLUSION: Implementing a complete transition from TR to TP biopsy can result in a significant reduction in complications and hospital re-admissions. A cognitive freehand coaxial technique is well tolerated by patients and achieves a high cancer detection rate.

Aggressive Angiomyxoma of the Pelvis: 35-Year Experience.

BACKGROUND: Aggressive angiomyxoma is a very rare mesenchymal tumor most commonly found in the pelvic and perineal regions. Although many are estrogen and progesterone hormone receptor positive, the pathogenesis is unknown. Due to its rarity, there is a paucity of literature relating to this pathology. This article presents a case series on the management of aggressive angiomyxoma of the pelvis. OBJECTIVE: To present a 35-year experience managing aggressive angiomyxoma of the pelvis. DESIGN: This was a retrospective single-system analysis. SETTINGS: This study was conducted at a quaternary referral academic health care system. PATIENTS: All patients treated for aggressive angiomyxoma of the pelvis. INTERVENTIONS: All patients underwent surgical or medical management of their disease. MAIN OUTCOME MEASURES: The primary outcomes were disease recurrence and mortality. Secondary outcomes included risk factors for recurrence. RESULTS: A total of 32 patients (94% women) were identified with a median follow-up of 65 months. Thirty patients (94%) underwent operative resection and 2 patients were treated solely with medical management. Fifteen achieved an R0 resection (negative microscopic margins) at the index operation, of which 4 (27%) experienced tumor recurrence. There were no mortalities. No risk factors for disease recurrence were identified. LIMITATIONS: Limitations to our study include its nonrandomized retrospective nature, single health care system experience, and small patient sample size. CONCLUSIONS: Aggressive angiomyxoma is a rare, slow-growing tumor with locally invasive features and a high potential for recurrence even after resection with negative margins. Imaging modalities such as CT or MRI should be obtained to aid in diagnosis and surgical planning. Workup should be paired with preoperative biopsy and testing for hormone receptor status, which can increase diagnostic accuracy and guide medical treatment. Close posttreatment surveillance is imperative to detect recurrence. See Video Abstract . ANGIOMIXOMA AGRESIVO DE PELVIS EXPERIENCIA DE AOS: ANTECEDENTES:El angiomixoma agresivo es un tumor mesenquimal muy raro que se encuentra más comúnmente en las regiones pélvica y perineal. Aunque muchos son positivos para los receptores hormonales como el estrógeno y la progesterona, la patogénesis es aún desconocida. Debido a su rareza, existe escasa literatura relacionada con esta patología. Este artículo presenta una serie de casos sobre el tratamiento del angiomixoma agresivo de pelvis.OBJETIVO:Presentar una experiencia de 35 años en el manejo del angiomixoma agresivo de pelvis.DISEÑO:Este fue un análisis retrospectivo de sistema único.AJUSTES:Este estudio se llevó a cabo en un sistema de salud académico de referencia de nivel cuaternario.PACIENTES:Todos los pacientes tratados por angiomixoma agresivo de pelvis.INTERVENCIONES:Todos los pacientes se sometieron a tratamiento quirúrgico y/o médico de su enfermedad.PRINCIPALES MEDIDAS DE RESULTADO:Los resultados primarios fueron la recurrencia de la enfermedad y la mortalidad. Los resultados secundarios incluyeron factores de riesgo de recurrencia.RESULTADOS:Se identificaron un total de 32 pacientes (94% mujeres) con una mediana de seguimiento de 65 meses. Treinta (94%) fueron sometidos a resección quirúrgica y dos fueron tratados únicamente con tratamiento médico. Quince lograron una resección R0 (márgenes microscópicos negativos) en la operación inicial, de los cuales cuatro (27%) experimentaron recurrencia tumoral. No hubo mortalidades. No se identificaron factores de riesgo para la recurrencia de la enfermedad.LIMITACIONES:Las limitaciones de nuestro estudio incluyen su naturaleza retrospectiva no aleatoria, la experiencia de un solo sistema de atención médica y el tamaño pequeño de la muestra de pacientes.CONCLUSIONES:El angiomixoma agresivo es un tumor raro, de crecimiento lento, con características localmente invasivas y un alto potencial de recurrencia incluso después de una resección con márgenes negativos. Se deben obtener modalidades de imágenes como CT y/o MRI para la ayuda diagnóstica y la planificación quirúrgica. El estudio debe combinarse con una biopsia preoperatoria y pruebas del estado de los receptores hormonales, que pueden aumentar la precisión del diagnóstico y guiar el tratamiento médico. Es imperativa una estrecha vigilancia posterior al tratamiento para detectar recurrencia. (Traducción-Dr Osvaldo Gauto ).

Surgical treatment of rectal cancer: prospective cohort study about good oncologic results and low rates of abdominoperineal excision.

OBJECTIVES: the purpose of this study was to evaluate the outcome of rectal cancer surgery, in a unit adopting the principles of total mesorectal excision (TME) with a high restorative procedure rate and with a low rate of abdominoperineal excision (APE). METHODS: we enrolles patients with extraperitoneal rectal cancer undergoing TME or TME+APE. Patients with mid rectal tumors underwent TME, and patients with tumors of the lower rectum and no criteria for APE underwent TME and intersphincteric resection. Those in which the intersphincteric space was invaded and in those with a free distal margin less than 1cm or a tumor free radial margin were unattainable underwent APE or extralevator abdominoperineal excision (ELAPE). We assessed local recurrence rates, overall survival and involvement of the radial margin. RESULTS: sixty (89.6%) patients underwent TME and seven (10.4%) TME + APE, of which five underwent ELAPE. The local recurrence, in pacientes undergoing TME+LAR, was 3.3% and in patients undergoing APE, 14.3%. The local recurrence rate (p=0.286) or the distant recurrence rate (p=1.000) was similar between groups. There was no involvement of radial margins. Survival after 120 months was similar (p=0.239). CONCLUSION: rectal malignancies, including those located in the low rectum, may be surgically treated with a low rate of APE without compromising oncological principles and with a low local recurrence rates.

Squamous Cell Carcinoma of perineal area developed from the Buschke-Löwenstein tumor.

The Buschke-Löwenstein tumor is a rare disease associated with human papillomavirus infection. The condition manifests with an ulcerative, exophytic tumor localized in the perineal area. Generally considered as non-cancerous, the growth may develop malignant transformation. Our manuscript highlights the importance of early diagnosis with histopathological analysis.

[Squamous cell carcinoma of the male perineum: a case report]. / Carcinome épidermoïde du périnée masculin: à propos d´un cas.

Squamous cell carcinoma of the male perineum is exceptional. We report the case of a 42-year-old patient with no previous medical history, presenting with pelvic discomfort lasting for 4 months. The patient was treated for perineal abscess in a health centre in Bamako. The anatomo-pathological examination confirmed the diagnosis. Treatment depends on the stage of the lesion and its location but it is associated with a poor prognosis. Given the results achieved in patients with epidermoid cancers of the esophagus and anus, treatment was based on therapeutic protocols combining chemotherapy and radiotherapy. The purpose of this work was to report the first case in our hospital unit.

Infantile perianal pyramidal protrusion: A retrospective review of 27 patients.

BACKGROUND: Infantile perianal pyramidal protrusion (IPPP) is a rare benign skin condition that is typically seen in infant girls. This condition is often mistaken for other skin lesions such as acrochordons, condyloma acuminatum, and sequelae of sexual abuse. Many clinicians are unfamiliar with IPPP which can lead to aggressive workup and treatment. The purpose of this study was to elucidate demographic data, clinical features, diagnostic workup, and management of IPPP in a large cohort of patients seen at an academic medical center. METHODS: A retrospective review of patients diagnosed with IPPP at Mayo Clinic, Rochester, MN was conducted. Demographic data, examination findings, diagnostic workup, and treatments were noted. RESULTS: Twenty-seven patients with IPPP, 24 of which were girls and 3 were boys from the ages of 1 day to 4 years (mean 10.8 months, median 8.6 months) were identified. Symptoms were reported in 63% (17/27) of patients. Four patients received treatment with topical corticosteroid or local anesthetic. No patient underwent skin biopsy. CONCLUSION: IPPP is commonly observed in healthy prepubertal girls. Constipation is a common symptom. Awareness of this entity may prevent overtreatment and unnecessary evaluation.

Rare recurrence of malignant mesothelioma spreading to the perineum.

Malignant mesothelioma is a rare aggressive tumour of the mesothelium with a propensity to spread locally and, rarely, to distant organs. The latest advances in its diagnosis and treatment have led to an increase in unusual disease presentations. Although a direct invasion of the perineum has been previously described in a men, a malignant mesothelioma spreading to the perianal region was never reported in a women. We presented a rare case of malignant mesothelioma recurrence spreading from the peritoneal cavity to the perineum through the rectovaginal space.

How I Do It: Transperineal prostate biopsy using local anesthetic in an outpatient setting.

Transperineal prostate biopsy (TPPB) is proven to be an effective diagnostic tool for prostate cancer detection. It allows satisfactory sampling of apical and anterior areas which is not well achieved with the transrectal route, without the associated risks of urinary tract infection or sepsis. The main objective of this paper is to describe the technique utilized in our institution to perform transperineal prostate biopsy under local anesthetic in the outpatient clinic setting.

Dermoscopy features of infantile perianal pyramidal protrusion.

Infantile perianal pyramidal protrusion (IPPP) is a benign condition generally noted in childhood but may persist for several years. Dermoscopy may help to distinguish it from other conditions, particularly genital warts. We report six cases of IPPP and describe the dermoscopic features that will distinguish these lesions from verrucae.

Perianal psoriasis as the first manifestation of the disease

Introduction: Inverse psoriasis affects the skin of flexural areas, such as the groin, axillae, umbilicus, intergluteal fold, and external genitalia. Clinical Case: We herein report the case of a man who presented with anal pruritus and, upon physical examination, a perianal dermatosis was found, which was characterized by erythematous plaques, with fine scaling. The case was initially managed with zinc oxide, and when no improvement was observed, we decided to take an incisional biopsy, which indicated histological changes suggestive of psoriasiform dermatitis. Discussion: Inverse psoriasis affects 3% to 7% of patients with psoriasis, and it manifests with erythematous plaques without the classic scaling appearance. The skin in these areas is susceptible to maceration, irritation, and ulceration, which alter the classic clinical picture. It may present with typical lesions or, less frequently, in isolation in the anogenital region. In the anogenital presentation only, the diagnosis should be made by biopsy, looking for the classic histopathological features of psoriasis. As for the first-line treatment, low- or medium-potency topical steroids are used for short periods of time; the second-line treatment is with emollients and tar-based products; and the third-line treatment uses an immunomodulator. Conclusion: This presentation is infrequent, and it requires a high index of suspicion for the diagnosis, always supported by biopsies, in search of the classic histopathological features of psoriasis. (AU)

Aggressive Angiomyxoma. A case series of eight years of experience.

BACKGROUND: Aggressive angiomyxoma is a type of mesenchymal tumor occurring predominantly in the pelvic and perineal region. The aim of our study was to reveal our experience with gonadotropin-releasing hormone (GnRH) treatment in patients with angiomyxoma and provide a comprehensive review of management. PATIENTS AND METHODS: This study is a case-series including seven female patients diagnosed with aggressive angiomyxoma from a single institution, between 2012 and 2020. Follow-up after surgery was ranged between 2-45 months with an average of 17.6 months. Resection was performed in all patients without any complications, and five had received GnRH analogue (Goserelin acetate) therapy after surgery. Immunohistochemistry analyses showed positivity for smooth muscle actin and desmin in all cases, while both estrogen receptor (ER) and progesterone receptor (PR) positivity were identified in 6 patients. None of the seven patients had recurrence during follow up period. CONCLUSION: The mean treatment of aggressive angiomyxoma is surgery, and the use of GnHR analogues in cases with positive ER and PR may be effective in preventing recurrence. KEY WORDS: Aggressive Angiomyxoma, Gonadotropin-Releasing Hormone, Soft Tissue Neoplasm.

Vulval Aggressive Angiomyxoma in a 19 year teenager: a case report.

BACKGROUND: Aggressive Angiomyxoma is a benign, slowly growing, locally aggressive and recurrent tumour that occurs in the pelvic-perineal region of females in their reproductive years. It presents as a painless, soft, gelatinous mass and metastasizes rarely. Suspicion can be made based on clinical examination and radiological imaging but final diagnosis is confirmed only after histopathology and immunohistochemistry. The choice of treatment is surgical wide local excision. CASE PRESENTATION: We hereby present a case of a 19-year, unmarried lady, with a body mass index of 21 kg/m2, who presented with a chief complaint of painless mass in left vulva which progressively increased in size in the past one year. Clinical examination revealed a large, cauliflower like, exophytic mass of 10 cm × 10 cm size. Radiological imaging confirmed involvement of lymph nodes. Wide local excision with adequate tumour free margin and depth was used as a treatment modality. The diagnosis was confirmed via histopathological examination of the excised specimen. There is no recurrence in the patient up to date. CONCLUSION: Aggressive Angiomyxoma is a rare tumour and it is most often misdiagnosed. This report highlights the importance of considering Aggressive Angiomyxoma as a differential diagnosis of vulval masses and the two-step surgical approach for its treatment in low resource setting.

Aggressive angiomyxoma of the stomach: a novel site of a rare neoplasm.

Aggressive angiomyxoma (AA) is a rare neoplasm of mesenchymal origin. It most commonly occurs in young women, predominantly in the pelvi-perineal region. We describe a case of AA arising from the lesser curvature of the stomach in a young woman who presented with a lump in the abdomen for 6 months. The patient subsequently underwent en bloc resection of the tumour and the diagnosis was confirmed on histology. This is the first reported case of gastric origin of AA to the best of our knowledge.

Aggressive angiomyxoma as a rare cause of scrotum enlargement in a 10-month-old boy: a case report.

BACKGROUND: Aggressive angiomyxoma (AAM) is a locally infiltrative mesenchymal tumour that most commonly affects the pelvis and/or perineum in adult women. AAM is very rare in males, especially in infancy. CASE PRESENTATION: A 10-month-old fulani (African) male infant was referred to our department for a large painless mass in the right testicule. The mass was detected during the neonatal period and gradually increased in size. Ultrasound examination revealed a large heterogeneous lesion; computed tomography results led to the conclusion that the mass was a mesenteric hernia. An inguinal and scrotal surgical approach was adopted. Exploratory surgery found a normal right testicle displaced upwardly and a large scrotal mass. Radical excision of the mass and orchidopexy were performed. Subsequent histology and immunohistochemstry studies indicated that the mass was a scrotal angiomyxoma. The postoperative course was uneventful. No recurrence occurred during the 6-month follow-up. CONCLUSION: To the best of our knowledge, this is the youngest patient with AAM reported to date. Angiomyxoma should be included in the differential diagnosis of scrotal masses, for which radical excision is justifiable to prevent recurrence.