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Journal of Cutaneous Pathology Aug 2016Mammary-like glands are normal appendages of anogenital skin and can give rise to epithelial and stromal tumors that closely resemble breast tumors. Cowden syndrome is... (Review)
Review
Mammary-like glands are normal appendages of anogenital skin and can give rise to epithelial and stromal tumors that closely resemble breast tumors. Cowden syndrome is an autosomal-dominant cancer-predisposition syndrome that is associated with increased risk of various benign and malignant tumors including breast cancers. Here, we report the first case of a proliferative lesion of mammary-like glands in the setting of Cowden syndrome. A 27-year-old female with Cowden syndrome (R130Q-PTEN mutation) presented with a 1-cm tender, polypoid perianal lesion. An excisional biopsy revealed a circumscribed, lobulated lesion with fibromyxoid stroma and epithelial hyperplasia with apocrine and columnar cell changes that was arranged in papillary, micropapillary and focal cribriform architecture. The features strikingly resembled proliferative changes commonly seen in the breast. Interestingly, the patient subsequently developed an atypical complex sclerosing lesion of the breast. Given the increased risk of breast neoplasia in Cowden syndrome, and the morphologic relationship between breast glands and mammary-like glands, this case raises the possibility of an increased risk of neoplasia arising in mammary-like glands in the setting of Cowden syndrome.
Topics: Adult; Anal Canal; Female; Hamartoma Syndrome, Multiple; Humans; Hyperplasia
PubMed: 27090873
DOI: 10.1111/cup.12721 -
Legal Medicine (Tokyo, Japan) Sep 2022MiraDry® is a microwave-based cosmetic device commonly used to treat hyperhidrosis and osmidrosis by affecting apocrine and eccrine sweat glands. In most countries, its...
MiraDry® is a microwave-based cosmetic device commonly used to treat hyperhidrosis and osmidrosis by affecting apocrine and eccrine sweat glands. In most countries, its application is limited to the axillary region. A healthy woman received MiraDry® treatment in the perineal, genital, and perianal regions for body odor in a cosmetic clinic. She experienced severe adverse effects after treatment, including persistent fever, sustained pain, and bleeding in the treated area. The condition deteriorated rapidly with systemic symptoms, and she died on the sixth day. Group A Streptococcus was detected in her skin in the treated areas, and in blood obtained in the hospital and during autopsy. Combined with the clinical diagnosis and autopsy findings, the woman's death was attributed to fatal necrotizing fasciitis (Fournier's gangrene) complicated by streptococcal toxic shock syndrome. Pathogen inoculation was most likely attributable to skin disruption caused by MiraDry® treatment. The MiraDry® application on the genital and perineum is occasionally performed by cosmetic surgeons; however, this case demonstrates the possibility of a rare but fatal complication. Therefore, this case report may be noteworthy and beneficial in forensic practice, and relevant in cosmetic clinical practice.
Topics: Debridement; Fasciitis, Necrotizing; Female; Fournier Gangrene; Humans; Male; Shock, Septic; Streptococcal Infections
PubMed: 35662070
DOI: 10.1016/j.legalmed.2022.102095 -
Inflammatory Bowel Diseases Jun 2019Perianal complications in patients with Crohn's disease are common and have a negative impact on the patients' quality of life. Data about the long-term disease course...
BACKGROUND
Perianal complications in patients with Crohn's disease are common and have a negative impact on the patients' quality of life. Data about the long-term disease course of perianal Crohn's disease in the era of biological treatment are limited. In this population-based cohort study, we sought to investigate the occurrence, clinical risk factors, and disease course of perianal disease.
METHODS
A total of 213 Crohn's disease patients were included in a prospective population-based inception cohort. Data were retrieved from medical records and national health administrative databases. Perianal disease was defined as a perianal fistula and/or abscess. Associations between outcomes and covariates were analyzed by Cox regression analysis.
RESULTS
A total of 48 (22.5%) patients developed perianal disease after 10 years. Colonic disease location (hazard ratio [HR], 1.99; 95% confidence interval [CI], 1.01-3.92) and penetrating behavior (HR, 5.65; 95% CI, 2.65-12.03) were associated with the development of perianal disease. The cumulative risk of undergoing abdominal surgery was 51% after 10 years. Patients with perianal disease had a higher rate of resection (HR, 3.92; 95% CI, 1.86-8.67) and hospitalization (HR, 1.01; 95% CI, 1.00-1.01). There was no significant difference in the rate of sick leave, unemployment, or disability pension between patients with and without perianal disease.
CONCLUSIONS
Patients with perianal disease carry a higher risk of surgery and hospitalization, and this suggests a more severe disease course and poorer prognosis among these patients, even in the era of biological treatment. These findings underline the importance of optimizing treatment strategies for patients with perianal disease.
Topics: Adolescent; Adult; Animals; Anus Diseases; Crohn Disease; Denmark; Disease Progression; Female; Follow-Up Studies; Humans; Incidence; Male; Perianal Glands; Prognosis; Prospective Studies; Risk Factors; Time Factors; Young Adult
PubMed: 30576474
DOI: 10.1093/ibd/izy374 -
Journal of Surgical Case Reports Mar 2021Cryptoglandular fistula-in-ano is a chronic inflammatory condition of the perianal region attributed to the obstruction of the glands, located chiefly at the dentate...
Cryptoglandular fistula-in-ano is a chronic inflammatory condition of the perianal region attributed to the obstruction of the glands, located chiefly at the dentate line and their subsequent infection. Anal fistulae are difficult to treat, and minimally invasive procedures are evolving with promises. We present an improvised application of core-cut technique of fistulectomy. It is a minimally invasive, simple, effective and easy to perform procedure with minimal risk of incontinence and recurrence in simple cryptoglandular fistulae-in-ano. We performed 47 cases with good results and present this procedure to emphasize the procedural modification used.
PubMed: 33815745
DOI: 10.1093/jscr/rjab032 -
Cureus Nov 2022Most patients with advanced cancer experience debilitating pain, which significantly affects their quality of life and has both physical and psychological implications....
Most patients with advanced cancer experience debilitating pain, which significantly affects their quality of life and has both physical and psychological implications. Opioids have been the mainstay of treatment for chronic cancer pain, but some people develop serious adverse effects or may become refractory to opioid use. There is always a need and search for alternative non-opioid analgesics with an acceptable safety profile, and one such drug is ketamine. In this era of evolving analgesic therapeutics, ketamine has been noted to have favourable results. Ketamine, a phencyclidine analogue, is an N-methyl-D-aspartate antagonist (NMDA), and it has been shown to have an analgesic effect at sub-anaesthetic doses by blocking NMDA-induced pain sensitization and enhancing opioid receptor sensitization. This is a case report of a 46-year-old Indian female with recurrent metastatic adenocarcinoma endometrium (International Federation of Obstetrics and Gynecology (FIGO) Grade II) involving the vaginal vault, rectum, and adrenal glands, along with para-rectal, bilateral iliac, and retroperitoneal nodal metastases, in which ketamine infusion was used successfully to alleviate the pain that was initially not controlled with an incremental dose of opioids. The patient presented with progressive pain in the peri-anal region, rated 8/10 on the Numerical Pain Rating Scale (NRS), following which she was treated with escalating doses of intravenous (IV) fentanyl, but with little to no relief. In view of the patient's opioid-resistant pain, she was started on a low-dose ketamine IV infusion (50 mg in 50 ml of 0.9% NS) as "burst therapy," at infusion rates of 0.02 mg/kg/hr-0.08 mg/kg/hr, with adequate pain relief occurring at 0.08 mg/kg/hr. Literature suggests weight-based dosing of ketamine ranging from 0.06 mg/kg/hr to 0.8 mg/kg/hr was previously used to achieve satisfactory results. In this patient, even lower doses were effectively used to achieve optimum long-term analgesia, cause an upliftment in the patient's overall mood and quality of life, and cause a significant reduction in opioid usage. However, further research is required to assess the efficacy of ketamine at such doses and its effect on opioid consumption. This case report will promote further study regarding optimum IV ketamine dosing and administration in the management of opioid-refractory pain in cancer patients, especially in the Indian population.
PubMed: 36545179
DOI: 10.7759/cureus.31662 -
Journal of Surgical Case Reports Jun 2023Extramammary Paget disease (EMPD) is an uncommon slow-growing skin adenocarcinoma originating in the anogenital region and axilla outside the mammary glands, often in...
Extramammary Paget disease (EMPD) is an uncommon slow-growing skin adenocarcinoma originating in the anogenital region and axilla outside the mammary glands, often in regions with apocrine glands. The most common location is the vulva, followed by perineal, perianal, scrotal and penile skin. Here, we report a case of a 63-year-old male with EMPD in the perianal region. He reported 4 years of pain associated with an increasing region of skin irritation and bleeding on defecation that did not improve with topical agents. A biopsy sample revealed poorly differentiated carcinoma consistent with adenocarcinoma and associated with Paget disease. Workup was done. The patient tolerated local excision of the region well with no complications. A rare disease, EMPT, is challenging to diagnose and manage. Histopathological findings can, however, differentiate it from a wide array of similar skin conditions. Thorough investigations should be undertaken before initiating treatment to ensure the best outcomes.
PubMed: 37337540
DOI: 10.1093/jscr/rjad291 -
In Vivo (Athens, Greece) 2023Extramammary Paget's disease (EMPD) is a rare, slow growing intra-epidermal malignant neoplasm that arises in areas rich in apocrine glands. Several common sites of...
BACKGROUND/AIM
Extramammary Paget's disease (EMPD) is a rare, slow growing intra-epidermal malignant neoplasm that arises in areas rich in apocrine glands. Several common sites of occurrence have been reported, including the vulva, perianal region, perineum, and scrotum. Most relevant studies rely on small data bases. Our objective was to evaluate prognostic factors of EMPD patients at a single medical center.
PATIENTS AND METHODS
We retrospectively analyzed 19 patients (8 males, 11 females) diagnosed with genital EMPD who were treated at the Taichung Veterans General Hospital between 2006/04 and 2022/08. Collected information included tumor location, margin condition in the case of surgical resection, recurrence rate, recurrence management, accompanied gastrointestinal malignancy, treatment details and survival data.
RESULTS
Among 19 cases, 4 with initial margin being positive, and 3 received second surgery (one refused surgery and another expired within a year). Tumor recurrence was found in 7 cases, with 6 of them later receiving second surgery, and the remaining one received radiation therapy. Median DFS was 7.57 years. During the 15-year follow-up, 2 patients expired. Overall survival rate was 87.5%. Among all factors we had analyzed, only those accompanied with GI tract malignancy had significantly worse survival rate (p=0.018). Frozen sections taken at surgical margin during surgery significantly reduced cancer recurrence rate (p=0.45). Permanent pathology margins appeared to affect the recurrence rate, but that was not significant when comparing with intraoperative frozen sections.
CONCLUSION
Local wide excision with skin flap reconstruction remains the major treatment option for genital EMPD. Following the standard-of-care procedure, the overall patient outcome was excellent. Among factors potentially associated with recurrence rate, intraoperative frozen biopsy was the most significant one. Performing intraoperative frozen biopsy is essential for recurrence-free rate elevation.
Topics: Male; Female; Humans; Retrospective Studies; Paget Disease, Extramammary; Prognosis; Taiwan; Neoplasm Recurrence, Local
PubMed: 37905610
DOI: 10.21873/invivo.13390 -
The Journal of Small Animal Practice Oct 2019To evaluate the diagnostic utility of individual cytological criteria and their best combination to differentiate benign from malignant perianal gland proliferative...
OBJECTIVES
To evaluate the diagnostic utility of individual cytological criteria and their best combination to differentiate benign from malignant perianal gland proliferative lesions in dogs.
MATERIALS AND METHODS
Retrospective study of cytological samples of canine perianal gland proliferative lesions that had subsequent histopathological confirmation.
RESULTS
Seventy-seven perianal gland nodules from 56 dogs were included. Histologically, lesions were diagnosed as hyperplasia (n=2), adenoma (n=53), epithelioma (n=6) and carcinoma (n=16). Of the 28 cytological criteria assessed, 13 showed promise for distinguishing benign from malignant lesions. A diagnostic algorithm with an 87% accuracy (sensitivity, 90.9%; specificity, 85.4%) was developed from these data.
CLINICAL SIGNIFICANCE
Cytological evaluation can provide useful information for presurgical differentiation between benign and malignant hepatoid gland proliferative lesions. The proposed algorithm must be validated and tested for reproducibility in further, preferably larger, series of cases.
Topics: Anal Gland Neoplasms; Animals; Carcinoma; Dog Diseases; Dogs; Perianal Glands; Reproducibility of Results; Retrospective Studies
PubMed: 31368201
DOI: 10.1111/jsap.13062 -
European Journal of Histochemistry : EJH Apr 2016Circumanal gland tumors are very common neoplasms of dogs. Their classification relies on microscopic examination and is further supported by a few immunohistochemical...
Circumanal gland tumors are very common neoplasms of dogs. Their classification relies on microscopic examination and is further supported by a few immunohistochemical markers that help indicate their prognosis. However, new additional tests would be highly useful. The purpose of this study was to develop such a test using fractal analysis which is increasingly being applied in science, especially in the field of biomedicine. A total of 53 circumanal gland tumors were chosen from our department archives. After a precise histological classification according to the World Health Organization classification, the number of de novo classified samples was as follows: 15 adenomas, 11 epitheliomas, 21 well differentiated carcinomas, 6 poorly differentiated carcinomas. Ten samples of normal circumanal gland were also included as control. All samples were immunohistochemicaly stained with vimentin. All immunohistochemical reactions were photographed at two different magnifications -100X and 400X and converted to 1 bit in black and white (bitmap) images thus enhancing the positive vimentin reactions. These images were used for the assessment of fractal dimension applying the box counting method and computer software Fractalyse. To determine the significance of results, conventional statistics were performed using Statistica software. The overall vimentin stain score was significantly higher in epitheliomas and carcinomas than in normal circumanal glands (CG) or adenomas. Mean values of fractal dimension estimated at magnification 100X and 400X were as follows: normal CG 1.318 and 1.372, CG adenomas 1.384 and 1.408, CG epitheliomas 1.547 and 1.597, CG well differentiated carcinomas 1.569 and 1.607, CG poorly differentiated carcinomas 1.679 and 1.723. Significant differences (at level of 5%) of these values were observed between individual groups of CG adenomas or normal CG, and epitheliomas or carcinomas. The above results indicate vimentin immunohistochemistry staining and assessment of fractal dimension as an ancillary diagnostic method of choice when discerning between benign and malignant tumors of circumanal glands. Additional development of the method of fractal dimension assesment may yield a possibility for this tool to successfully discern between all of the types of CG tumors.
Topics: Anal Gland Neoplasms; Animals; Dog Diseases; Dogs; Perianal Glands
PubMed: 27349313
DOI: 10.4081/ejh.2016.2609 -
The Pan African Medical Journal 2022Adnexal carcinomas are rare, accounting for less than 1% of skin carcinomas. Sclerosus carcinoma of the sweat glands was first described by Goldstein et al. in 1982. We...
Adnexal carcinomas are rare, accounting for less than 1% of skin carcinomas. Sclerosus carcinoma of the sweat glands was first described by Goldstein et al. in 1982. We here report the case of a 33-year-old female patient with a retracted perianal skin lesion. Histological examination of perilesional skin biopsy, immunohistochemistry, and negative results of laboratory tests, radiological and endoscopic investigations allowed for the diagnosis of eccrine sclerosus carcinoma. This is a rare tumor, usually characterized by facial localization and slow but aggressive progression. It poses problems in differential diagnosis with benign and malignant tumors; hence the challenge encountered by pathologist of suspecting this carcinoma in patients with any sclerotic and infiltrating skin lesion characterized by slow progression, in a context of preservation of the general state and in the absence of neoplastic history as well as of feeling free to ask for new deep biopsies when in doubt.
Topics: Adult; Breast Neoplasms; Carcinoma; Carcinoma, Skin Appendage; Diagnosis, Differential; Female; Humans; Immunohistochemistry; Skin Neoplasms; Sweat Gland Neoplasms
PubMed: 35865833
DOI: 10.11604/pamj.2022.41.329.28965