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Anti-cancer Drugs Jan 2022Adenoid cystic carcinoma (ACC) is a rare salivary glands tumor and often displays aggressive behavior with frequent relapse and metastasis. The terminal ACC lacks...
Adenoid cystic carcinoma (ACC) is a rare salivary glands tumor and often displays aggressive behavior with frequent relapse and metastasis. The terminal ACC lacks standard treatment guidelines and is always accompanied by poor prognosis. Here, we report a case of rare perianal ACC who received resection and palliative adjuvant radiation. Five years later, PET-computed tomography (CT) showed perianal recurrence and multiple pulmonary metastases. Combined chemotherapy with doxorubicin, carboplatin and cyclophosphamide was applied for two cycles but ineffective. Further next-generation sequencing analysis of perianal tissue demonstrated the v-myb avian myelobastosis viral oncogene homolog and nuclear factor I/B fusion gene and two novel BCL-6 corepressor (BCOR) mutations (p.F1106Tfs*5 and p.L1524Hfs*8). The therapy was switched to eribulin and anlotinib and has been performed for eight cycles. At recent follow-ups, MRI and CT examinations revealed the diminishing perianal and pulmonary lesions. This study presented the first case of perianal ACC with multiple pulmonary metastases and particular BCOR mutations, who presented a durable response to eribulin and anlotinib, providing a potential therapeutic option for advanced refractory ACC.
Topics: Anal Gland Neoplasms; Antineoplastic Agents; Antineoplastic Combined Chemotherapy Protocols; Carcinoma, Adenoid Cystic; Furans; Humans; Indoles; Ketones; Lung Neoplasms; Male; Middle Aged; Neoplasm Recurrence, Local; Quinolines
PubMed: 34321419
DOI: 10.1097/CAD.0000000000001171 -
Journal of Pharmacy & Bioallied Sciences Apr 2024Septic pulmonary embolism (SPE) represents the occurrence of septic thrombi in circulation, originating from an extrapulmonary infectious source. Perianal and perirectal...
Septic pulmonary embolism (SPE) represents the occurrence of septic thrombi in circulation, originating from an extrapulmonary infectious source. Perianal and perirectal abscesses are frequently encountered anorectal issues, often stemming from obstructed anal crypt glands, resulting in pus accumulation within the subcutaneous tissue and intersphincteric plane. Timely surgical drainage is essential upon diagnosis of anorectal abscesses. Adult males exhibit a twofold higher incidence of anorectal abscesses and fistulae compared to females, with common symptoms including excruciating anal or rectal pain. This case report details the presentation and management of a 42-year-old male patient afflicted by perianal abscesses that led to SPE. The report underscores the importance of recognizing and treating anorectal abscesses promptly to avert potentially life-threatening complications such as sepsis and fistulae.
PubMed: 38882749
DOI: 10.4103/jpbs.jpbs_1091_23 -
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 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 -
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 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 -
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