Figure - available from: Case Reports in Pathology
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Verrucous Carcinoma. Marked digitated acanthosis of is associated with bulbous aggregates of squamous epithelium, producing a pushing border. Cytologic atypia was minimal. A focus of SCAP is present at the lower left 20x.
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This case report presents a case in which a collision tumor consisting of three separate pathological entities—a verrucous carcinoma (VC), syringocystadenoma papilliferum (SCAP), and a basal cell carcinoma (BCC). The presentation of this collision tumor is unexpected. It presented as an exophytic mass on the scalp. While collisions of SCAP and VC a...
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Key Clinical Message
Gaint fungating BCC is rare and aggressive. Early health‐seeking behavior may result in positive outcomes.
Abstract
Fungating giant basal cell carcinoma (BCC) is a rare and aggressive form of BCC infrequently reported in the literature. We present a giant BCC case in an old female from a rural area with a poor socioeconomic pr...
A rare case of recurrent basal cell carcinoma in the scalp that infiltrated multiple intracranial structures is presented. Basal cell carcinoma represents one of the most frequent malignant nonmelanotic skin neoplasms, but the majority of them have no aggressive and recurrent behaviour. The aim of this case report is to provide an overview of the m...
Citations
... 5 SCAP is assumed to occur more in females as was the case in our patient. 6 Seventy-five percent of SCAP lesions occur in the head and neck region and most of these lesions are found on the scalp. 6 This tumour can be found in other areas of the body and has been reported to occur in the lower limb and eye lid in studies in the Western and Northern parts of Nigeria. ...
... 6 Seventy-five percent of SCAP lesions occur in the head and neck region and most of these lesions are found on the scalp. 6 This tumour can be found in other areas of the body and has been reported to occur in the lower limb and eye lid in studies in the Western and Northern parts of Nigeria. 7,8 Multiple SCAP lesions are uncommon. ...
... 6,10 SCAP may co-exist with other malignant tumours such as verrucous carcinomas ( a variant of squamous cell carcinoma). 6 It has equally been reported to co-exist with two other histologically different cell types including the nodular variant of basal cell carcinoma and a verrucous carcinoma in a single tumour on a patient's scalp. 6 No other lesions were reported to co-exist with SCAP found in the index patient. ...
DISCLOSURE No funding was received for this manuscript ABSTRACT Syringocystadenoma papilliferum is a benign harmatomatous tumour of apocrine or eccrine sweat gland origin. We report a 30-year-old female who presented with a 24-year history of a slowly growing scalp lesion with a preceding history of trauma to the same region of the scalp as a child. Examination revealed a 2x2 cm tumor in the mid-scalp region, exuding scanty sero-sanguinous fluid on contact. She had an excision biopsy of the lesion following a tentative diagnosis of foreign body granuloma. However, pathologic findings included glandular papillary proliferation connected to the epidermis and lined by columnar cells with oval nuclei with overall impression of Syringocystadenoma papilliferum. She was subsequently reassured and has been on follow-up with no recurrence.
... DNA of HPV type 16 and 68 have been detected in SCAP of the perianal area and buttock, without HPV-related morphology [153]. Verrucous proliferations associated with SCAP demonstrate BRAF mutation in both the glandular and contiguous hyperplastic squamous epithelium [149,154,155]. Sialadenoma papilliferum exhibits the same morphology and carries BRAF p.V600E mutation in most cases, while reports of RAS mutations are currently lacking [156,157]. ...
Simple Summary
Cutaneous sweat gland tumors form an extremely diverse and heterogeneous group of neoplasms that show histological differentiation to the sweat apparatus. Due to their rarity, wide diagnostic range, and significant morphological overlap between entities, their accurate diagnosis remains challenging for pathologists. Until recently, little was known about the molecular pathogenesis of adnexal tumors. Recent findings have revealed a wide range of gene fusions and other oncogenic factors that can be used for diagnostic purposes and, for some, can be detected by immunohistochemistry. Among other organs containing exocrine glands, such as salivary glands, breasts, and bronchi, most of these biomarkers have been reported in homologous neoplasms that share morphological features with their cutaneous counterparts. This review aims to describe these recent molecular and immunohistochemical biomarkers in the field of sweat gland tumors.
Abstract
Cutaneous sweat gland tumors are a subset of adnexal neoplasms that derive or differentiate into the sweat apparatus. Their great diversity, rarity, and complex terminology make their pathological diagnosis challenging. Recent findings have revealed a wide spectrum of oncogenic drivers, several of which are of diagnostic interest for pathologists. Most of these molecular alterations are represented by gene fusions, which are shared with other homologous neoplasms occurring in organs containing exocrine glands, such as salivary and breast glands, which show similarities to the sweat apparatus. This review aims to provide a synthesis of the most recent immunohistochemical and molecular markers used for the diagnosis of sweat gland tumors and to highlight their relationship with similar tumors in other organs. It will cover adenoid cystic carcinoma (NFIB, MYB, and MYBL1 fusion), cutaneous mixed tumor (PLAG1 fusion), cylindroma and spiradenoma and their carcinomas thereof (NF-κB activation through CYLD inactivation or ALKP1 hotspot mutation), hidradenoma and hidradenocarcinoma (MAML2 fusion), myoepithelioma (EWSR1 and FUS fusion), poroma and porocarcinoma (YAP1, MAML2, and NUTM1 fusion), secretory carcinoma (ETV6, NTRK3 fusion), tubular adenoma and syringo-cystadenoma papilliferum (HRAS and BRAF activating mutations). Sweat gland tumors for which there are no known molecular abnormalities will also be briefly discussed, as well as potential future developments.
... BCC also occurs at the same site with other malignant tumors (Table 2). 12,19,36, Discussion Nomenclature ...
... However, BCC associated with two or more additional skin neoplasms have been observed (Table 4). [4][5][6][7][8][9][10][11][12]24,32,34,43,45,46,[79][80][81] Epidemiology In a retrospective study from the dermatopathology laboratory of a single institution, a total of 40,000 cutaneous biopsy specimens were evaluated. Only 69 specimens (0.17%) had two or more contiguous neoplasms that were within a millimeter of each other. ...
... [4][5][6][7][8][9][10][11] One patient, a 65-year-old man with a growing 6 centimeter exophytic mass on his left occipital scalp of 5-years duration, also had a concurrent verrucous carcinoma. 12 BCC has also been associated with benign follicular and sebaceous adnexal tumors. MUSK IN A NEST consisting of a follicular tumor and BCC include not only trichilemmoma (on the left cheek of a 75-year-old farmer), 15 and trichofolliculoma (on the right nasolabial sulcus of a 52-year-old woman), 18 but also trichoepithelioma; the latter has been observed in individuals who have either a solitary adnexal tumor 16 or multiple facial neoplasms. ...
Multiple skin neoplasms at one site (MUSK IN A NEST), initially referred to as a collision tumor, describes the occurrence of two or more benign or malignant neoplasms that are adjacent or intermingled at the same cutaneous site. A mononeoplastic cutaneous tumor refers to a single tumor at any cutaneous site. Two, three, four, five, and six tumors at the same site are described as dineoplastic, trineoplastic, tetraneoplastic, pentaneoplastic, and hexaneoplastic cutaneous tumors, respectively; the prefixes are based on the numerical multiplier used by the International Union of Pure and Applied Chemistry (IUPAC). MUSK IN A NEST can be classified based upon their mechanism of pathogenesis–either being composed of mixed clones of cells (clonalium, which has three subtypes: collision, colonization, and combination) or the same clone of cells that has undergone clonal evolution (clonalidem, which has one subtype: biphenotypic). Basal cell carcinoma (BCC)-associated MUSK IN A NEST can be observed with either benign tumors, malignant tumors, or both. Nevi and seborrheic keratoses are the most common benign tumors associated with BCC; melanoma in situ and invasive melanoma are the most commonly reported malignant tumors associated with BCC. The definitive etiology of BCC-associated MUSK IN A NEST remains to be established–whether the development of the BCC at that site occurs as a direct or indirect consequence of the coexisting neoplasm or whether the occurrence of the BCC and the other neoplasm is merely the result of a coincidental juxtaposition of the tumors.
... It has a 5 year survival rate of about 50% [3,5]. Seeking reliable prognostic markers of oral verrucous carcinoma, can predict the course of the disease and the response to therapeutic intervention among affected patients [6]. ...
Context: A strong cause/effect relationship is evident between the expression levels of Cathepsin D (CD) in oral cancers and their local invasion and progression. Aims: The present study aims to assess the immunohistochemical expression of CD in normal oral mucosa, Verrucous Carcinoma (VC) and Oral Squamous Cell Carcinoma (OSCC). Settings and Design : A retrospective immunohistochemical study conducted in a tertiary care dental hospital with a sample size of 30. Materials and Methods: Immunohistochemical staining for CD was performed in 10 tissue sections each of normal mucosa, OSCC and VC. CD positivity, site of expression and intensity were analyzed semi-quantitatively under 10x and 40x magnification. Statistical Analysis Used: Cramer's V test was done and p value is calculated. Results: CD expression was positive in 90% of normal oral mucosa. CD expression was mild in 33.3%, moderate in 33.3% and severe in 33.3% of well differentiated SCC, respectively. CD expression was mild in 25 %, moderate in 50% and severe in 25% cases of moderately differentiated SCC respectively. All cases of poorly differentiated SCC showed severe expression of CD. CD expression was positive in all cases of VC. Increased expression of CD correlated significantly with the presence of metastasis (p<0.03) and poor histologic malignancy grade (p < 0.05). Conclusion: CD was demonstrated in normal oral mucosa. There was a positive correlation between increasing grades of OSCC and increasing intensity of CD. It closely correlated with carcinoma invasion and progression. CD maybe a prognostic factor for SCC and may serve as a potential target for cancer therapy. More studies are needed to correlate CD expression in VC with its biologic behaviour.
