Syringocystadenoma Papilliferum Research Articles

Syringocystadenocarcinoma Papilliferum: A rare Pathological entity

Syringocystadenocarcinoma papilliferum is a malignant form of its benign tumour that is syringocystadenoma papilliferum. Regarding clinical morphology it mimics varied skin tumors so diagnosis is difficult. Histopathology is ultimate role in final clinical diagnosis of these tumours. We present an uncommon case of SCACP arising from back mass in 45 years male in view of only handful cases have been reported in the literature with little information is available on its clinical and morphological aspect.

Rate of Benign and Malignant Secondary Tumors Associated With Nevus Sebaceous: A Systematic Review and Meta-Analysis.

Nevus sebaceous (NS) is a rare congenital skin lesion affecting approximately 0.3% of all newborns. Although benign, NS lesions can harbor malignant secondary tumors. The published rate of development of these malignant tumors varies. This meta-analysis aimed to identify the rate of malignant and benign secondary neoplasms occurring in NS. A literature search was conducted using PubMed, Embase, and Web of Science from inception to April 2023. Eligible studies reported incidence or risk of secondary neoplasms in patients with NS. Two independent reviewers screened studies, extracted data, and assessed the quality of included studies. The primary outcome was the pooled incidence of secondary neoplasms. Studies with sample sizes greater than 50 patients were eligible for meta-analysis using the random-effects model. Twenty-eight studies were identified, 22 of which were eligible for meta-analysis. The overall rate of secondary neoplasms was 12.8% (95% confidence interval [Cl], 9.2%-17.6%). The rates of development of malignant and benign tumors were 2.4% (95% CI, 1.4%-4.1%) and 10.3% (95% CI, 7.5%-13.9%), respectively. The rate of development of basal cell carcinoma was 1.7% (95% CI, 0.9%-3.2%), whereas the rate of the development of syringocystadenoma papilliferum was 3.6% (95% CI, 2.5%-5.3%) and that if trichoblastoma was 2.6% (95% CI, 1.7%-3.8%). Although the rate of development of malignant tumors within a primary NS lesion is low, it is not negligible. Prophylactic early excision remains a viable approach to prevent secondary malignant neoplasms, address cosmetic and functional complications, and preempt the need for complex reconstruction in the future. We propose that resection of NS lesions in childhood remains a reasonable first-line option in the appropriate patient keeping in mind that it may leave an undesirable scar.

Verrucous lesion mimics- A case series

Cutaneous lesions with wart like growth pattern are referred as verrucous lesions. Few lesions clinically look like warts but they are associated with different conditions, pathology, prognosis and treatment. Histopathology plays a very important role in such cases to diagnose the condition. In this article we present 5 different cases, with verrucous morphology. Our first case was a 70 year old man, with verrucous growth over hyperpigmented plaque on left leg. Biopsy showed Squamous cell Carcinoma overlying Lichen Simplex Chronicus. Second case was a 65yr old man with verrucous growth over right thigh diagnosed as well differentiated Squamous cell carcinoma on histopathology. Third case was a 22year old man, with multiple erythematous verroucous growth over left thigh. Biopsy showed syringocystadenoma papilliferum. Fourth case was a 9 year old female, with verrucous lesion over left side of forehead. Biopsy suggested chondroid syringoma. Fifth case was a 25year old female with multiple verrucous lesions over nose, diagnosed as sebaceous hyperplasia. Difficulty in diagnosing verrucous lesions can be resolved with knowledge and awareness of different diseases with verrucous morphology and histopathological examination.

Cytomorphological features of syringocystadenoma papilliferum mimicking pilomatricoma at a rare location and in an unusual age group: A cytological diagnostic challenge.

Syringocystadenoma papilliferum (SCAP) is a rare, benign skin adnexal tumor of apocrine or eccrine differentiation usually occurring in the head and neck region of children. It is a difficult entity to diagnose clinically and even cytological diagnosis remains challenging due to overlapping and masquerading cyto-morphological features with other benign cutaneous adnexal tumors. Here we present, one such intriguing case of SCAP with its cytological features mimicking those of pilomatricoma. Moreover, the lesion was located at the right lower abdomen, which in itself is a rare site and its presentation was in an adult male which is an unusual age group. However, fine needle aspiration cytology still remains a very useful investigation for cutaneous tumors as it helps in differentiating benign lesions from malignant and metastatic neoplasms and therefore aids in correct treatment and follow-up of patients.

Syringocystadenoma papilliferum associated with apocrine gland hyperplasia arising from nevus sebaceous

Syringocystadenoma papilliferum associated with apocrine gland hyperplasia arising from nevus sebaceous

Syringocystadenoma papilliferum and leiomyoma of uterus

Syringocystadenoma papilliferum is benign hamartomatous adnexal tumor commonly occurring in childhood. Our patient was a 58-year-old lady presenting with an asymptomatic plaque on the scalp for past 6 years and postmenopausal bleeding for 8 months. Histopathological examination of scalp lesion confirmed the diagnosis of syringocystadenoma papilliferum which is known to be associated with mutations of genes like KRAS, PTCH. The patient also underwent surgical removal of uterine leiomyoma which is also associated with KRAS mutation.

Linear syringocystadenoma papilliferum of neck: a rare variant adnexal tumour

Syringocystadenoma papilliferum is a rare benign adnexal tumour presenting as exuberant proliferating lesion showing differentiation in apocrine pattern. The three clinical variants are linear, plaque and solitary of which linear variant is the rarest. We report a case of linear variant of syringocystadenoma papilliferum, which had typical histopathological features. As there is risk of malignancy in long standing cases, early diagnosis and treatment is of utmost importance.

DP26 Syringocystadenocarcinoma papilliferum masquerading as squamous cell carcinoma

Abstract Syringocystadenocarcinoma papilliferum (SCACP) is a rare cutaneous adnexal neoplasm with ~30 cases reported. This case highlights the potential diagnostic and treatment challenges. A 52-year-old woman presented with a poorly differentiated carcinoma on the left parietal scalp which recurred 2 and 5 years following the original surgical excision. Initial biopsy of this lesion had shown syringocystadenoma papilliferum (SCAP) with associated syringoma, and a subsequent wider excision revealed suspected poorly differentiated squamous cell carcinoma (SCC) in the deeper dermis that was fully excised; however, the SCAP reached the margins. Excision 2 years later of the first recurrence showed similar histological features, and a second recurrence was excised twice to achieve histological clearance followed by postoperative radiotherapy. She remains tumour-free 2 years later. Upon retrospective review of all specimens, the initial primary tumour excision showed histological findings of a surface exophytic SCAP with mild nuclear enlargement, nucleoli and scattered mitotic figures. An infiltrative lesion was seen beneath this consisting of atypical ductal structures within a fibroblastic stroma. In deeper regions, there was more solid proliferation with some squamous differentiation, although scattered glandular units remained visible. However, the squamous component was predominant in future biopsies and even represented the sole morphological appearance in some. This culminated in the latest biopsy showing a poorly differentiated spindle cell tumour which, with immunohistochemistry (BerEP4-negative; cytokeratin, CK14−, CK5−, p63− and p53-positive; and high Ki67 index), was considered entirely to be a poorly differentiated SCC. Ultimately, this sequence of biopsies actually represented the rare diagnosis of SCACP, which demonstrated progressive squamous differentiation with each recurrence. A previous case published also reported SCACP transition to squamous differentiation (Zhang YH, Wang WL, Rapini RP et al. Syringocystadenocarcinoma papilliferum with transition to areas of squamous differentiation: a case report and review of the literature. Am J Dermatopathol 2012; 34:428–33). Despite the generally favourable prognosis reported, there have been a few cases of locoregional metastasis. Furthermore, recurrence up to 8 years after excision of the primary tumour has been reported (Castillo L, Moreno A, Tardío JC. Syringocystadenocarcinoma papilliferum in situ: report of a case with late recurrence. Am J Dermatopathol 2014; 36:348–52). We present a unique case of doubly recurrent SCACP managed with surgery and adjuvant radiotherapy which highlights the need for long-term follow-up. Furthermore, SCACP may pose a diagnostic challenge due to squamous differentiation of these tumours, which may result in incorrect or delayed diagnosis.

Nipple Adenoma: Clinicopathologic Characterization of 50 Cases.

Nipple adenoma (NA) is a rare, benign proliferation of the nipple ducts. It may be clinically mistaken for Paget disease or squamous cell carcinoma; thus, microscopic evaluation is paramount. A large case series of NA has not been undertaken since the 1980s. Therefore, we undertook this study to evaluate the clinicopathologic characteristics of NA, emphasizing differential diagnoses and follow-up data. We retrieved 50 cases from our in-house archives or consultation files between 2003 and 2022. Available slides were reviewed, and clinical data and follow-up information were obtained. Cases must have exhibited a dense ductal proliferation in the breast tissue with proximity to the nipple epidermis. All patients were women; median age was 56 years. In all, 68% of patients were symptomatic; 53% demonstrated a skin growth. Overall, 67% were excised completely, either primarily (33%) or via re-excision after biopsy (33%). Four histologic patterns were noted: adenosis (dense proliferation of small-to-medium ducts); large duct (medium-to-large caliber ducts); papillary-like (frond-like architecture with branching, slit-like lumens); and pseudoinfiltrative (ducts squished and distorted by dense stromal fibrosis). Follow-up in 44 patients (88%) with a median time of 66 months showed no evidence of recurrence. NA demonstrates a wide spectrum of histopathologic variation. Subtyping of this entity is unlikely to be clinically relevant. Differentiation from invasive carcinoma or other histologic mimics (syringocystadenoma papilliferum, syringomatous adenoma) may be difficult. Simple excision is curative, and recurrence is rare. A definitive link to invasive carcinoma has not been established.

Molecular Profiling of Syringocystadenocarcinoma Papilliferum Reveals RAS-Activating Mutations.

Syringocystadenocarcinoma papilliferum (SCACP) is a rare adnexal carcinoma and the malignant counterpart of syringocystadenoma papilliferum (SCAP), which is commonly located on the head and neck and may arise in association with a nevus sebaceus. RAS mutations have been identified in both SCAP and nevus sebaceus. To evaluate the clinicopathologic and molecular features of SCACPs, which have not been previously explored. We obtained 11 SCACPs from 6 institutions and reviewed the clinicopathologic features. We also performed molecular profiling using next-generation sequencing. The cohort comprised 6 women and 5 men with ages ranging from 29 to 96 years (mean, 73.6 years). The neoplasms occurred on the head and neck (n = 8; 73%) and extremities (n = 3; 27%). Three tumors possibly arose in a nevus sebaceus. A total of 4 cases showed at least carcinoma in situ (adenocarcinoma, n = 3; squamous cell carcinoma [SCC], n = 1), and 7 cases were invasive (SCC, n = 5; mixed adenocarcinoma + SCC, n = 2). A total of 8 of 11 cases (73%) had hot spot mutations consisting of HRAS (n = 4), KRAS (n = 1), BRAF (n = 1), TP53 (n = 4), ATM (n = 2), FLT3 (n = 1), CDKN2A (n = 1), and PTEN (n = 1). All 4 cases with HRAS mutations occurred on the head and neck, whereas the KRAS mutation occurred on the extremity. RAS-activating mutations were detected in 50% of the cases, of which most (80%) involved HRAS and occurred on the head and neck, which shows overlapping features with SCAP, supporting that a subset may arise as a result of malignant transformation and likely an early oncogenic event.

Apocrine Papillary Hidrocystoma With Mucinous Metaplasia (Goblet Cell Type): A Case Report and Review of the Literature.

Mucinous metaplasia (goblet cell type) is exceptionally rare in the skin. This is the second case of apocrine papillary hidrocystoma with mucinous metaplasia (goblet cell type) and a review of the literature exploring the significance and frequency of mucinous metaplasia with goblet cells in nongenital skin. The patient is an elderly man who presented with a blue-pigmented nodule on the scalp that was clinically suggestive of an atypical nevus. Histologically, the lesion was composed of a simple cyst of cuboidal cells with decapitation secretion and mucinous metaplasia with goblet cells. Papillary formation was identified in the cysts. Most cases of cutaneous mucinous metaplasia have been reported on genital skin, usually after chronic inflammation of the area. This type of mucinous metaplasia is categorized as benign mucinous metaplasia of the genitalia (BMM) and is believed to be unrelated to apocrine glands owing to the different histologic features and absence of apocrine differentiation by immunohistochemistry. Mucinous metaplasia (goblet cell type) has been previously reported in benign adnexal tumors (eccrine acrospiroma/hidroadenoma, mixed tumor, and syringocystadenoma papilliferum) and in malignant tumors (apocrine hidradenocarcinoma and squamous cell carcinoma). To date, mucinous metaplasia has not been identified in the histologically normal apocrine glands.

SYNRINGOCYSTADENOMA PAPILLIFERUM: A RARE CASE REPORT

Syringocystadenoma papilliferum is a rare benign hamartomatous adnexal tumour that occurs chiey on the scalp, neck and face. It is present since birth or in early childhood. We are presenting a case of Syringocystadenoma papilliferum which was clinically diagnosed as Molluscum contagiosum. Histopathological diagnosis was crucial and conrmatory in this case since Syringocystadenoma papilliferum has the potential to transform into malignant Syringocystadenocarcinoma Papilliferum, hence, surgical excision of lesion followed by histopathology is the preferred mode of treatment

Ceruminous adenoma of the external auditory canal: 9 cases series with imaging and pathologic findings.

Ceruminous adenoma is a rare benign tumor of the external auditory canal. This study aimed to present the clinical characteristics, imaging findings, pathological results and the management outcomes of the ceruminous adenoma. Retrospective case series review. Tertiary referral center. Patients undergoing surgery for ceruminous adenoma of the external auditory canal between the years 2004 to 2018. All patients with ceruminous adenoma were analyzed for demographic, clinical, radiological features and pathologic findings. The outcomes of the management were also evaluated. Nine patients with ceruminous adenoma were included in the study. Hearing loss was the most common complaint (5/9, 56%), followed by otalgia (4/9, 44%), pruritus (4/9, 44%), and otorrhea (2/9, 22%). The tumors originated mostly from the cartilaginous portion of the external auditory canal (8/9, 89%) and merely from the bony portion of the external auditory canal (1/9, 11%). Pathohistological study indicated that the ceruminous adenomas were divided into three types: the ceruminous gland adenoma (6/9, 67%), the ceruminous pleomorphic adenoma (2/9, 22%) and the ceruminous syringocystadenoma papilliferum (1/9, 11%). No recurrence was found during follow-up for two to fifteen years after surgical resection. Ceruminous adenomas are rare entities. They originate mainly from the cartilaginous portion of the EAC, but occasionally from the bony portion of the EAC. The surgical section with enough margin is adequate for management of these tumors.

CeruminousSyringocystadenoma papilliferum: An Extremely Rare Neoplasm of the External Ear

Abstract Introduction/Objective Syringocystadenoma papilliferum (SCAP) is a rare benign adnexal tumor derived from the apocrine or the eccrine sweat glands on the face or the scalp. Ceruminous glands are modified apocrine glands in the external auditory canal and produce cerumen that helps the ear canal against physical damage and microbial invasion. Neoplastic transformation of these glands is even rarer among these benign tumors. Methods/Case Report Here we report a case of a 29-year-old female with a three-month history of progressively worsening right-sided ear pain, muffled hearing, aural fullness, and right-sided shooting aches. She reported that her ear felt sensitive to touch and was painful if she lay on it. Previous treatments included steroids and oral antibiotics without any improvement in symptoms. A physical exam revealed a right narrow ear canal with a cystic mass to the superior lateral canal wall. A CT Temporal Bone showed a 1 cm oval soft tissue lesion in the lateral osseous portion of the right external auditory canal with no underlying osseous erosions identified. This is nonspecific, and neoplasm cannot be excluded. The decision was made for excision. The mass ruptured during excision with dishwater appearing fluid. Microscopic examination showed papillae lined by bilayered glandular epithelium projecting into a cystic lumen. Well defined myoepithelial cell layer is appreciated. Decapitation secretions and plasma cells are noted to be present in the stroma. A final diagnosis of Syringocystadenoma papilliferum is made. Results (if a Case Study enter NA) NA. Conclusion Ceruminous Syringocystadenoma papilliferum is an extremely rare benign tumor of the ceruminous glands of the external ear. Complete excision is the treatment of choice

A Case of Ceruminous Syringocystadenoma in the External Auditory Canal

It is rare to find tumor of the ceruminous gland, which is a modified apocrine gland located within the skin of external auditory canal (EAC). Syringocystadenoma papilliferum (SCAP) is a benign adnexal tumor that occurs in the head and facial area in forms of infiltrative macules, papules, or keratinous nodules, but rarely in the EAC. To date, fewer than 150 case reports of SCAP have been reported, with SCAP in the EAC being extremely rare. We report a case of 51-year-old male with 2 year-history of a left EAC mass arising from the superior wall of the EAC. We performed a microscopic surgery, with the mass resected via retro-auricular approach under general anesthesia. The histopathological examination confirmed the specimen as SCAP. There were no complications or recurrence during the 4 months follow-up period.

Intraoral Sialadenoma Papilliferum: A Comprehensive Review of the Literature with Emphasis on Clinical and Histopathological Diagnostic Features

Background. Sialadenoma papilliferum (SP) is a rare benign epithelial tumor of salivary gland origin, its diagnosis being potentially challenging. It was first described by Abrams and Finck in 1969 as an analog of the cutaneous syringocystadenoma papilliferum. The aim of this comprehensive review is to highlight the clinical and histopathological diagnostic aspects of intraoral SP, analyzing cases previously described and reporting new cases. Methods. Medline, Scopus, and Web of Science were searched up to February 2022, using as entry term “sialadenoma papilliferum”. No time limits were applied and only studies in English were taken into account. Only cases involving the mouth were included. Conference proceedings, personal communications, and letters to the editor were excluded. Results. In total, 42 out of 234 articles fulfilled the inclusion criteria, with 64 cases reported. Mean age of patients with SP was 57.2 years, with a higher prevalence among males. The most affected site was the palate, particularly the hard palate. Four cases with uncertain malignant features have been reported. While clinical manifestations of SP are rather unspecific (e.g., submucosal swelling with ulceration), histopathological and immunohistochemical features are quite peculiar, SP have a limited growth potential, leading to conservative excision as treatment of choice. Conclusions. SP, though rare, should be taken into consideration in the differential diagnosis of intraoral swellings, particularly those located on the palate.

Secondary Tumors Arising from Nevus Sebaceus: A Multicenter Collaborative Study and Literature Review

Background: Although various benign and malignant tumors can arise in nevus sebaceus (NS), the demographic and clinicopathological features of the secondary tumors vary among different published reports. The aim of this study was to obtain a better understanding of the features of tumors secondary to NS. Methods: A multicenter study was performed by reviewing patients diagnosed with NS from the Dermatology Department of the First Affiliated Hospital of Chongqing Medical University and Qijiang Hospital of the First Affiliated Hospital of Chongqing Medical University from 2010 to 2022. A literature review of case series of secondary tumors arising in NS was also conducted. Results: We retrospectively analyzed the literature on tumors secondary to NS published since 2000, with a total of 443 cases, and carefully studied cases of tumors arising in NS from multiple centers since 2010, with a total of 54 cases. There were 497 cases in this study; 90.3% were benign tumors (n = 449), and the rest were malignant tumors (9.7%, n = 48). Syringocystadenoma papilliferum was the most common benign tumor, followed by trichoblastoma and trichilemmoma. Basal cell carcinoma was the most common secondary malignant tumor to NS, followed by squamous cell carcinoma. Two of these cases developed metastasis. Secondary malignancies developed at an earlier age in Caucasians than in Hispanics and Asians. Conclusion: The present study is the largest analysis of tumors secondary to NS. Racial differences were seen in secondary tumors to NS and may contribute to healthcare disparities between races.

SYRINGOCYSTADENOMA PAPILLIFERUM OF SCALP – A CASE REPORT OF A RARE CUTANEOUS ADNEXAL NEOPLASM

Syringocystadenoma papilliferum is a rare benign hamartomatous tumor arising from apocrine or eccrine sweat glands with a predilection for head and neck. Here we report a case of a 30 year old female who presented to our opd with a nodule over scalp since birth which turned into a hairless , exophytic pinkish growth with blood tinged discharge slowly over the last 2 years . A punch biopsy of the lesion revealed syringocystadenoma papilliferum. Excision of the lesion is performed and specimen is sent for (hpe) histopathological examination which revealed syringocystadenoma papilliferum with no malignant transformation. Post operative recovery was uneventful and the patient was followed up till 6 months with no history of recurrence.

Dermoscopy of De Novo Syringocystadenoma Papilliferum

Syringocystadenoma papilliferum is a uncommon benign cutaneous tumor seen to arise from the pluripotent cells with the potential to exhibit either apocrine or eccrine lineage. We report the dermoscopic features of a case of de novo syringocystadenoma papilliferum. Clinically the lesion was characterised by a nodular and crusted surface. Dermoscopically, yellowish-white structures were the prevalent feature.

Study of clinicohistopathological correlation of skin adnexal tumours

Appendageal tumours (Appendageal tumours) are neoplasms which differentiate towards or arise from pilosebaceous apparatus, apocrine gland or eccrine sweat glands. Overall incidence of Appendageal tumours is low, yet they can be challenging to diagnose. The study aims at determining the frequency, profile, and distribution of skin adnexal tumours and association of their distribution with patient’s age and sex. Cross-sectional descriptive study, conducted over 1-year period. All clinically suspected cases of Appendageal tumours were evaluated and subjected to histopathological examination. Confirmed cases of Appendageal tumours were finally analyzed. : Out of 43586 new patients attending OPD, 64(0.14%) cases of Appendageal tumours were suspected. And 60 underwent histopathological examination. Histopathology was confirmatory in only 42 (70%) cases. Out of 42, the hair follicle tumors comprised of trichoepithelioma 18/42(42.85%), trichofolliculoma 1/42 (2.38%), pilomatricoma 4/42 (9.52%), and trichilemmal cyst 4/42 (9.52%). The sweat gland tumors are comprised of chondroid syringoma 12/42(28.57%), hidrocystoma 2/42(4.76%) and syringocystadenoma papilliferum 1/42(2.38%). Females (61.90%) outnumbered males (38.09%) in our study population. The incidence of benign skin Appendageal tumours is more as compared to the malignant ones as found in our study. Histopathology remains the gold standard for diagnosis of these tumours.