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Cureus May 2021Extramammary Paget disease (EMPD) is a rare form of cancer that involves skin areas rich in apocrine glands. The common sites of occurrence include the vulva, perianal...
Extramammary Paget disease (EMPD) is a rare form of cancer that involves skin areas rich in apocrine glands. The common sites of occurrence include the vulva, perianal region, perineum, and axilla. Perianal Paget disease (PPD) is a subset of EMPD, which arises in the perianal skin. The disease commonly presents with a thickened plaque-like lesion with erythema or white scaly appearance. It is generally classified into two categories based on the origin of the tumor cells: (1) primary PPD if the tumor arises from intraepidermal cells and (2) secondary PPD if cancer originates from the underlying colorectal or urinary tract neoplasm. Due to its rarity, only a few sporadic case reports have been published in the literature, and treatment methods are yet to be standardized. In light of this, we report two PPD cases with different etiopathogenesis and staging: one involved only the perianal skin without regional or metastatic disease, and was not accompanied by visceral adenocarcinomas although there was a previous history of sigmoid adenocarcinoma; the other was probably secondary to an anal canal tumor spreading with disseminated disease involving the perianal and perineum area with bilateral inguinal, pelvic lymph node, and liver metastasis. The treatment plans and the outcomes of both cases were necessarily different from each other.
PubMed: 34168927
DOI: 10.7759/cureus.15161 -
Journal of Oral and Maxillofacial... 2020Hidradenoma Papilliferum (HP) is a rare, benign, cutaneous adnexal tumor of middle-aged females arising mainly in the apocrine sweat glands of perianal and genital...
Hidradenoma Papilliferum (HP) is a rare, benign, cutaneous adnexal tumor of middle-aged females arising mainly in the apocrine sweat glands of perianal and genital region. Initially, HP was considered to be an apocrine sweat gland tumor however recent research showed that it descends from the anogenital mammary-like glands, HP can be regarded theoretically as the cutaneous complement of mammary intraductal papilloma. Nongenital HP is an infrequent manifestation, ectopic forms of this entity have been defined with bulk of them occurring on the head and neck region (60%) and 85% of cases being 1.5 cm in the greatest diameter or lesser. Average age of patients with ectopic HP is 1-2 decades older than the age range of onset in patients with anogenital HP. Here, we present a 15-year-old adolescent female with an unusually large, ectopic occurrence of nodular HP on right cheek since 2 years.
PubMed: 33967496
DOI: 10.4103/jomfp.JOMFP_126_20 -
Annals of Medicine and Surgery (2012) Apr 2021Hidradenitis suppurativa (HS) is a chronic inflammatory and suppurative disease of the apocrine sweat glands. Its transformation into squamous cell carcinoma (SCC) is...
INTRODUCTION AND IMPORTANCE
Hidradenitis suppurativa (HS) is a chronic inflammatory and suppurative disease of the apocrine sweat glands. Its transformation into squamous cell carcinoma (SCC) is very rare.
CASE PRESENTATION
We describe 3 cases of males aged 57, 58 and 55-years-old. The diagnoses were confirmed by pathology examination in all cases. Two of our patients underwent extended and complete surgery. The first two patients died during the year after the diagnosis was established, the third one is still alive with no recurrence after one year of surgery.
CLINICAL DISCUSSION
Hidradenitis suppurativa mostly concerns perianal location and it targets mainly male patients with a multifactorial development. Surgical treatment consists of large excision. The SCC is associated with a high mortality rate.
CONCLUSION
Hidradenitis suppurativa needs early diagnosis and effective surgery. The transformation into squamous cell carcinoma is rare and and its management is challenging. Extended and complete excision is required with rigorous postoperative follow-up.
PubMed: 33868679
DOI: 10.1016/j.amsu.2021.102239 -
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 -
Animals : An Open Access Journal From... Mar 2021Perianal gland tumors are modified sebaceous glands present in the skin of the perianal region in the dog. Hormonal stimulation may induce hyperplasia of the perianal...
Perianal gland tumors are modified sebaceous glands present in the skin of the perianal region in the dog. Hormonal stimulation may induce hyperplasia of the perianal glands or their neoplastic progression. The presence of androgen (AR) and estrogen (ER) receptors have been demonstrated both in normal perianal glands as well as in perianal tumors. The aim of the study was an immunohistochemical assessment of the expression of estrogen and androgen receptors in perianal gland tumors in dogs as an applicatory marker for antihormonal treatment. Biopsy samples of perianal masses were collected from 41 male dogs. A histopathological examination revealed 24 adenomas, 12 epitheliomas and five carcinomas. The immunohistochemical staining showed a mainly nuclear expression of AR and ER in the neoplastic cells. Both the androgen and estrogen receptors were expressed in adenoma, epithelioma and carcinoma cases; however, the highest expression of the receptors was stated in the adenoma and epithelioma. In the case of the carcinoma, the expression of sex hormone receptors was very weak. The differences of the number of cells expressing AR and ER as well as the observed differentiated intensity of staining in the studies demonstrated that the determination of the expression of the sex hormone receptors may be useful to elaborate a diagnostic and therapeutic algorithm.
PubMed: 33808541
DOI: 10.3390/ani11030875 -
Cureus Feb 2021Hidradenoma papilliferum (HP) is a benign pathologic finding that has been described primarily in the vulvar region. While thought to arise from ectopic tissue along the...
Hidradenoma papilliferum (HP) is a benign pathologic finding that has been described primarily in the vulvar region. While thought to arise from ectopic tissue along the mammary line, it is now known to also arise from mammary-like anogenital glands (MLG), which are part of the normal anogenital cellular and glandular milieu. Previous work has demonstrated the relationship between HP and hormone receptors, but this has not been documented in a clinical setting. In this case, we present HP in a patient undergoing infertility treatments, presenting with a painful, enlarging anal mass. Upon histopathologic review, the mass was found to be an HP with no malignant changes. This unique case adds to the existing literature on perianal HP. While this case follows the known clinical and histological patterns of HP, the unique temporal relationship to in vitro fertilization (IVF) treatment supports previous in vitrowork on the relationship between HP and hormone receptors.
PubMed: 33680603
DOI: 10.7759/cureus.13061 -
The Journal of International Medical... Feb 2021Cryptoglandular perianal fistula is a common benign anorectal disorder that is managed mainly with surgery. A fistula is typically defined as a pathological... (Review)
Review
Cryptoglandular perianal fistula is a common benign anorectal disorder that is managed mainly with surgery. A fistula is typically defined as a pathological communication between two epithelialized surfaces. More specifically, perianal fistula manifests as an abnormal tract between the anorectal canal and the perianal skin. Perianal fistulas are often characterized by significantly decreased patient quality of life. The cryptoglandular theory of perianal fistulas suggests their development from the proctodeal glands, which originate from the intersphincteric plane and perforate the internal sphincter with their ducts. Involvement of proctodeal glands in the inflammatory process could play a primary role in the formation of cryptoglandular perianal fistula. The objective of this narrative review was to investigate the current knowledge of the pathogenesis of cryptoglandular perianal fistula with the specific aims of characterizing the potential role of proinflammatory factors responsible for the development of chronic inflammation. Further studies are crucial to improve the therapeutic management of cryptoglandular perianal fistulas.
Topics: Anal Canal; Humans; Quality of Life; Rectal Fistula; Treatment Outcome
PubMed: 33595349
DOI: 10.1177/0300060520986669 -
Journal of Ultrasonography Nov 2020The presence of ectopic functional endometrial glands and struma anywhere except in the lining of the uterine cavity is considered as endometriosis. Extrapelvic...
The presence of ectopic functional endometrial glands and struma anywhere except in the lining of the uterine cavity is considered as endometriosis. Extrapelvic endometriosis involving the abdominal wall cesarean section scar is uncommonly seen, and it rarely involves the perineum, umbilicus, pleura, kidneys, lungs and liver. The purpose of the present study is to highlight rare ectopic sites, explain the pathogenesis of extrapelvic endometriosis, and evaluate the diagnostic significance of clinical findings, serum CA 125 level, and ultrasonography. 24 female patients with extrapelvic endometriomas in whom the final diagnosis was based on the surgical results and histopathological reports of the excised specimens. The patients underwent a clinical examination, an ultrasound scan, and evaluation of the serum CA 125 level. They were also examined by transvaginal ultrasound to rule out ovarian endometriosis or uterine adenomyosis. They were further subjected to abdominal wall ultrasound in cases of cesarean section scar or umbilical region swellings, and transperineal ultrasound for perianal lesions. Transvaginal ultrasound was performed in patients with perineal endometrioma to assess the relation between the lesion and the external anal sphincter. In 19 patients, abdominal wall cesarean section scar endometrioma was detected. Three patients had perianal endometriomas, and two patients - umbilical endometriomas. Ultrasound scanning was a useful diagnostic tool to evaluate extrapelvic endometriosis and its extension, especially in cases without typical clinical features that can be suggestive of endometrioma, low diagnostic sensitivity of serum CA 125, and low incidence of concomitant intrapelvic disease.
PubMed: 33365153
DOI: 10.15557/JoU.2020.0029 -
Veterinary Pathology May 2020Pediatric patients receiving solid organ transplants may develop lymphoproliferative diseases, including graft-versus-host disease (GvHD) and posttransplant...
Pediatric patients receiving solid organ transplants may develop lymphoproliferative diseases, including graft-versus-host disease (GvHD) and posttransplant lymphoproliferative diseases (PTLDs). We characterized lesions in 11 clinically ill NOD.Cg-/SzJ (NSG) mice that received pediatric-patient-derived solid tumors (PDXs) and developed immunodeficiency-associated lymphoproliferations comparable to GvHD and PTLDs over a period of 46 to 283 days after implantation. Lymphoproliferations were diffusely positive for human-specific biomarkers, including NUMA1, CD45, and CD43, but lacked immunoreactivity for murine CD45. Human immune cells were CD3-positive, with subsets having immunoreactivity for CD4 and CD8 as well as PAX5, CD79a, and IRF4, resulting from populations of human T and B cells present within the xenotransplants. Tissues and organs infiltrated included mucocutaneous zones (oral cavity and perigenital and perianal regions), haired skin, tongue, esophagus, forestomach, thyroid, salivary glands, lungs, liver, kidneys, spleen, lymph nodes, bone marrow, and brain. In 4 of 5 mice with PTLD, Epstein-Barr virus (EBV)-encoded small RNAs (EBERs) were detected by hybridization in PAX5 human B cells associated with the PDX ( = 1/4) or with engrafted human immune cells at other anatomic locations ( = 4/11). One of the 4 mice had an EBV-associated human large B-cell lymphoma. NSG mice receiving xenotransplants can develop combinations of GvHD, EBV-driven PTLD, and B-cell lymphoma similar to those occurring in human pediatric patients. Therefore, pediatric xenotransplants should undergo histopathologic and immunohistochemical assessment upon collection to ensure that the specimen is not a lymphoma and does not contain lymphoma cells because these neoplasms can morphologically mimic small round blue cell pediatric solid tumors.
Topics: Animals; B-Lymphocytes; Biomarkers, Tumor; Cell Cycle Proteins; Epstein-Barr Virus Infections; Graft vs Host Disease; Heterografts; Humans; Leukocyte Common Antigens; Leukosialin; Lymphoma; Lymphoproliferative Disorders; Mice; Mice, Inbred NOD; Neoplasm Transplantation; T-Lymphocytes; Transplantation, Heterologous
PubMed: 32202225
DOI: 10.1177/0300985820913265 -
Annals of Coloproctology Dec 2019Hidradenoma papilliferum is a rare benign cystic tumor that originates from apocrine glands or anogenital mammary glands. Here, we describe 2 cases of hidradenoma...
Hidradenoma papilliferum is a rare benign cystic tumor that originates from apocrine glands or anogenital mammary glands. Here, we describe 2 cases of hidradenoma papilliferum of the anus. Two female patients aged 39 and 35 presented with perianal masses with hemorrhoids. The patients underwent hemorrhoidectomy and excision of the lesion. Histopathology confirmed the masses as hidradenoma papilliferum. The postoperative course was uneventful for both patients, and there were no recurrences after 18 and 12 months of follow-up, respectively. Proctologists should consider hidradenoma papilliferum in their differential diagnosis of benign anal tumors. Surgical excision is necessary for diagnosis and treatment of hidradenoma papilliferum.
PubMed: 31937076
DOI: 10.3393/ac.2018.08.03