Clear Cell Hidradenoma/Hidradenocarcinoma

Total Page:16

File Type:pdf, Size:1020Kb

Clear Cell Hidradenoma/Hidradenocarcinoma UPDATE ON MALIGNANT ADNEXAL NEOPLASMS David S. Cassarino, M.D., Ph.D. Los Angeles Medical Center, Southern California Permanente Medical Group, Department of Pathology University of California, Irvine, Department of Dermatology CLASSIFICATION OF ADNEXAL TUMORS • Older classifications based on questionable morphologic and histochemical observations - Most of these are not specific for apocrine vs. eccrine diff’nt • Many tumors designated as eccrine or apocrine have features of the other category or features of adnexal ducts - Ducts of apocrine and eccrine nature show similar features and are essentially indistinguishable • Benign versus malignant determination is crucial for treatment and prognosis • Features such as asymmetry, infiltrative borders, increased mitoses, and necrosis favor malignancy • Atypical adnexal tumors show some, but not all, of these features • In many cases, due to limited sampling of the tumor (i.e., shave or punch biopsy), a definitive classification is not possible ◼ Such cases should be signed out descriptively, with a differential diagnosis, and complete excision recommended to obtain a definitive diagnosis • Newer immunohistochemical and molecular findings associated with particular tumors: • SOX10 in apocrine and some eccrine tumors • GATA3 in follicular, sebaceous, and apocrine tumors • MYB in adenoid cystic carcinoma, apocrine tumors • Beta-catenin overexpression in pilomatrical tumors • FXIIIa (nuclear) in sebaceous tumors • CYLD mutations in cylindroma, spiradenoma, trichoblastomas, and adenoid cystic carcinoma • HRAS, p53, RB1, APC, CDKN2A, and PTEN mutations in porocarcinoma • t(11;19) translocation in hidradenomas and hidradenocarcinomas • ETV6-NTRK3 translocation in primary cutaneous mammary analog secretory carcinoma CD117 and SOX-10 had similar overall positivity rates in benign apocrine and eccrine tumors (45% and 68% respectively), and were generally negative in other benign and malignant adnexal tumors. 16% (23/140) of benign adnexal tumors significantly expressed MYB, mostly in cylindromas and to a lesser extent, spiradenomas. SOX10 showed positivity in spiradenomas (13/13), cylindromas (9/10), hidradenoma papilliferum (10/10), syringocystadenoma papilliferum (8/10), apocrine adenomas (8/10), and negativity in poromas (0/12), syringomas (0/10), and basal cell carcinomas (0/13). • Nuclear GATA3 expression was seen in 70% (153/220) of cases, including sebaceous carcinoma (93%), apocrine carcinoma (93%), follicular neoplasms (100%), and predominantly apocrine neoplasms (69%), but only 38% of predominantly eccrine neoplasms. MICROCYSTIC ADNEXAL CARCINOMA (MAC) Clinical: - Occurs on face, usually lip and nasolabial area, followed by chin and cheeks, slowly enlarging firm plaque - Locally aggressive tumor, high recurrence rate, but only rare mets Molecular: - Rare mutations in p53 and CDKN2A genes Histology: - Large, poorly-circumscribed dermal tumor deeply infiltrating into subcutis (even muscle) - Consists of bland adnexal (likely apocrine) cells forming cords and small ducts infiltrating a desmoplastic stroma - Superficial horn cysts and abortive follicles (FSAU-diff’nt) - Mitoses rare, perineural invasion frequent CEA IHC: + for EMA and CEA (luminal), CK7 (diffuse), low Ki67 and p53 (versus high in Perineural invasion sBCC) • Diff’nt diagnosis: 1. Infiltrative/sclerosing BCC: - Lacks superficial follicular cysts and ductal diff’nt, only rare perineural invasion; EMA, CEA, CK7- 2. Desmoplastic trichoepithelioma: - Lacks deep and perineural invasion, has calcifications, follicular but no ductal diff’nt; EMA, CEA, CK7-; CK20+ Merkel cells 3. Syringoma: - Bland ductal structures containing proteinaceous secretions (not keratin or hair material), lacks the deep infiltration, keratinocysts, and perineural invasion of MAC SCLEROSING BCC DesmoTE TRICHOADENOMA SYRINGOMA - Superficial, noninfiltrative ductal structures - Tadpole shapes - No follicular diff ’nt INFILTRATING ECCRINE (SYRINOGOID) CARCINOMA Clinical: - Older patients, often head and neck, upper extremities Histology: - Infiltrating ductal - but no follicular - structures in a sclerotic stroma - Small to moderately enlarged, basophilic to pale/clear cells with marked atypia, nuclear hyperchromasia, mitoses - Diffuse dermal and subQ invasion often present Prognosis: - Locally aggressive tumors, reportedly high rates of recurrence, low metastasis TRICHOBLASTIC CARCINOMA Clinical: - Rare malignant tumors of hair germ, likely arise from trichoblastomas (can be a/w/Brook-Spiegler syndrome, CYLD mutations) - Usually large tumors, > 1 cm, on the head, esp. scalp Histology: - Large, poorly circumscribed basaloid tumor w/out epidermal connections, located in deep dermis, often w/subcutis extension - Show evidence of pilar differentiation - Cells are basaloid, and show mitoses and apoptoses, but lack prominent peripheral palisading, mucoid stroma typical of BCC Ki-67 p53 TRICHOBLASTIC CARCINOMA: DIFFERENTIAL DIAGNOSIS 1. BCC: - Tumor-stroma retraction artifact, mucinous stroma, prominent palisading, and mitoses and apoptoses are abundant 2. TRICHOBLASTOMA: - Large, nodular basaloid tumor composed of relatively small, bland-appearing basaloid cells - Lacks atypia and increased mitoses, apoptoses of BCC and trichoblastic carcinoma BCC, Nodular and micronodular types TRICHOBLASTOMA CUTANEOUS LYMPHADENOMA SEBACEOUS TUMORS • Tumors of definite sebaceous differentiation: - Sebaceous adenoma - Sebaceoma (“sebaceous epithelioma”/basal cell carcinoma with sebaceous differentiation) - Sebaceous carcinoma • Tumors of questionable/partial sebaceous differentiation: - Superficial epithelioma with sebaceous diff’nt - Mantleoma (likely fibrofolliculoma) SEBACEOUS CARCINOMA Clinical: - Most on eyelids, followed by head and neck, presents as a nodule ± ulceration and hemorrhage - Often misdiagnosed (> 80% in some studies!), usually as BCC, SCC, Paget disease, other adnexal tumors - May be a/w/Muir Torre Syndrome (MLH1, MSH2, MSH6, PMS2 mutations) – less aggressive Histology: - Irregular, fusing lobules, nodules and sheet-like collections of pale/clear cells with infiltrative features - Cytologic atypia, nuclear pleomorphism, multi mitoses, often see areas of comedonecrosis - IHC: AR+, adipophilin+, FXIIIa+, EMA±, CD10±, CK7±, p53+, ↑ Ki-67, oil red O (frozens) Squamoid (left) and clear cell areas (right) EMA Androgen receptor (AR) DIFFERENTIAL DIAGNOSIS Benign sebaceous tumors - Sebaceous adenoma: Circumscribed growth of well-differentiated sebocytes surrounded by layer of small basaloid cells, > 50% well-differentiated sebocytes - Sebaceoma (“sebaceous epithelioma”): Circumscribed growth, < 50% well-differentiated sebocytes, > 50% bland basaloid cells; may see mitoses in basaloid cells, but lack atypia, mucinous stroma or retraction artifact MTS: cystic sebaceous neoplasms MLH1 MSH2 Ki-67 ADENOMA CARCINOMA Cabral et al., Amer J. Dermatopathol 2006; 28:465-71 - Clear cell BCC: Rare variant, usually has areas of more typical BCC with palisading, retraction-artifact, mucinous stroma - Clear cell SCC: Should see areas of squamous diff’nt, often w/overlying AK or SCC in situ; lack of cytoplasmic vacuoles and nuclear scalloping - Metastatic renal cell carcinoma: Usually well-circumscribed dermal nodule w/prominent vascularity; clear cells with often bland cytologic features; CK7-; CD10 & RCA+ DIFFERENTIAL DIAGNOSIS - Tricholemmal carcinoma: Peripheral palisading, uniformly clear cytoplasm lacking multivacuolar change, areas of squamoid diff, PAS+/PAS-D- (glycogen) - Clear cell hidradenoma/hidradenocarcinoma: Large nodular or cystic dermal-based lesion, usually lacks epidermal or follicular connections, lacks vacuoles, PAS+/PAS-D-; CEA & EMA highlight ductal structures CLEAR CELL BCC APOCRINE ADENOCARCINOMA Clinical: - Rare tumor, primarily axillary and anogenital, but occurs in other sites as well - Presents as nodular/multinodular mass - Aggressive, reportedly lethal in over 30% of cases History: - Unencapsulated, infiltrative nodular lesion with variable patterns including papillary, tubular, and cord-like arrangements - Atypical apocrine cells w/low to high grade cytological atypia and mitoses APOCRINE ADENOCARCINOMA Differential Diagnosis: 1. Tubular apocrine adenoma: benign cytologic features and lack of infiltration or mitoses 2. Metastatic adenocarcinoma, especially breast: may be very difficult without clinical history, exams Immunohistochemistry: - Most cases are CK 5/6, p63, AR, mammaglobin, and GCDFGP positive - Often GATA3, ER/PR positive, so cannot distinguish from Breast CA - Variable S100 and CEA staining CK 5/6 p63 GCDFP (Brst-2) ER ADENOID CYSTIC CARCINOMA Clinical: - Rare primary cutaneous tumor, often scalp or chest, may arise from apocrine glands Molecular: - CYLD mutations (aberrant fusion transcript MYB-NFIB) Histology: - Islands and nests of cells with cribiform spaces and tubules filled with mucinous and basement membrane material - Low grade histology, rare mitoses, but frequent perineural invasion Prognosis: - Indolent growth and high recurrence potential (up to 70%), but low metastatic rate S100 MALIGNANT SPIRADENOMA Clinical: - Usually malignant transformation of large, untreated spiradenoma: history of sudden enlargement of long-standing lesion - Can be a/w/Brooke-Spiegler syndrome, CYLD mutations Histology: - Areas of benign-appearing spiradenoma associated with foci of malignant transformation with cellular atypia, nuclear hyperchromasia, and pleomorphism, many mitoses Prognosis: - Mets occur, usually LNs, but few reported deaths
Recommended publications
  • Atypical Compound Nevus Arising in Mature Cystic Ovarian Teratoma
    J Cutan Pathol 2005: 32: 71–123 Copyright # Blackwell Munksgaard 2005 Blackwell Munksgaard. Printed in Denmark Journal of Cutaneous Pathology Abstracts of the Papers Presented at the 41st Annual Meeting of The American Society of Dermatopathology Westin Copley Place Boston, Massachusetts, USA October 14–17, 2004 These abstracts were presented in oral or poster format at the 41st Annual Meeting of The American Society of Dermatopathology on October 14–17, 2004. They are listed on the following pages in alphabetical order by the first author’s last name. 71 Abstracts IN SITU HYBRIDIZATION IS A VALUABLE DIAGNOSTIC A 37-year-old woman with diagnosis of Sjogren’s syndrome (SS) TOOL IN CUTANEOUS DEEP FUNGAL INFECTIONS presented with asymptomatic non-palpable purpura of the lower J.J. Abbott1, K.L. Hamacher2,A.G.Bridges2 and I. Ahmed1,2 extremities. Biopsy of a purpuric macule revealed a perivascular Departments of Laboratory Medicine and Pathology1 and and focally nodular lymphocytic infiltrate with large numbers of Dermatology2, plasma cells, seemingly around eccrine glands. There was no vascu- litis. The histologic findings in the skin were strikingly similar to those Mayo Clinic and Mayo Foundation, Rochester, MN, USA of salivary, parotid, and other ‘‘secretory’’ glands affected in SS. The cutaneous manifestations of SS highlighted in textbooks include Dimorphic fungal infections (histoplasmosis, blastomycosis, coccidiomy- xerosis, annular erythema, small-vessel vasculitis, and pigmented cosis, and cryptococcosis) can occur in immunocompromised and purpura. This case illustrates that purpura in skin of patients with healthy individuals. Cutaneous involvement is often secondary and SS may be caused by a peri-eccrine plasma-rich infiltrate.
    [Show full text]
  • Malignant Hidradenoma: a Report of Two Cases and Review of the Literature
    ANTICANCER RESEARCH 26: 2217-2220 (2006) Malignant Hidradenoma: A Report of Two Cases and Review of the Literature I.E. LIAPAKIS1, D.P. KORKOLIS2, A. KOUTSOUMBI3, A. FIDA3, G. KOKKALIS1 and P.P. VASSILOPOULOS2 1Department of Plastic and Reconstructive Surgery, 2First Department of Surgical Oncology and 3Department of Surgical Pathology, Hellenic Anticancer Institute, "Saint Savvas" Hospital, Athens, Greece Abstract. Introduction: Malignant tumors of the sweat glands difficult (1). Clear cell hidradenoma is an extremely rare are very rare. Clear cell hidradenoma is a lesion with tumor with less than 50 cases reported (2, 3). histopathological features resembling those of eccrine poroma The cases of two patients, suffering from aggressive and eccrine spiradenoma. The biological behavior of the tumor dermal lesions invading the abdominal wall and the axillary is aggressive, with local recurrences reported in more than 50% region, are described here. Surgical resection and of the surgically-treated cases. Materials and Methods: Two histopathological examination ascertained the presence of patients are presented, the first with tumor in the right axillary malignant clear cell hidradenoma. In addition to these region, the second with a recurrent tumor of the abdominal cases, a review of the literature is also presented. wall. The first patient underwent wide excision with clear margins and axillary lymph node dissection and the second Case Reports patient underwent wide excision of the primary lesion and bilateral inguinal node dissection due to palpable nodes. Patient 1. Patient 1 was a 68-year-old Caucasian male who had Results: The patients had uneventful postoperative courses. No undergone excision of a rapidly growing, ulcerous lesion of the additional treatment was administered.
    [Show full text]
  • Malignant Nodular Hidradenoma-Inguinal Region Clinically Masquerading As Squamous Cell Carcinoma: a Case Report
    International Journal of Research in Medical Sciences Vernekar S et al. Int J Res Med Sci. 2019 Jul;7(7):2848-2852 www.msjonline.org pISSN 2320-6071 | eISSN 2320-6012 DOI: http://dx.doi.org/10.18203/2320-6012.ijrms20192933 Case Report Malignant nodular hidradenoma-inguinal region clinically masquerading as squamous cell carcinoma: a case report Sunita S. Vernekar, Priyadharshini Bargunam* Department of Pathology, Karnataka Institute of Medical Sciences, Hubballi, Karnataka, India Received: 24 April 2019 Accepted: 05 June 2019 *Correspondence: Dr. Priyadharshini Bargunam, E-mail: [email protected] Copyright: © the author(s), publisher and licensee Medip Academy. This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License, which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. ABSTRACT Malignant Nodular hidradenoma is an extremely rare aggressive tumour originating from eccrine sweat glands with an incidence of <.001%. So far less than 80 cases have been reported in the literature. It’s known for its local recurrence (50%) and metastasis (60%) and hence early diagnosis and radical treatment is mandatory. But differentiating it from its benign counterparts and other skin tumour mimics is challenging, due to its histopathological similarity & lack of diagnostic immunomarkers. Authors report a case of 65-year-old female who presented with a short 4-month history of rapidly growing ulceroproliferative growth in the right inguinal region with bilateral inguinal node enlargement, associated with pain and discharge. Wedge biopsy of left inguinal lymph node showed malignant cutaneous adnexal tumour deposits, which after excision was typed as malignant nodular hidradenoma.
    [Show full text]
  • Sample Research Poster
    Surgical management and lymph node biopsy of rare malignant cutaneous adnexal carcinomas: a population-based analysis of 7591 patients Amrita Goyal MD, 1 Theodore Marghitu,2 Nikhil Goyal BS,3 Nathan Rubin MS,4 Krishnan Patel MD,6 Kavita Goyal MD,1 Daniel O’Leary MD,5 Kimberly Bohjanen MD, 1 Ian Maher MD 1 1Department of Dermatology, University of Minnesota, Minneapolis, MN 2University of Minnesota Medical School, Minneapolis, MN 3National Institutes of Health/National Cancer Institute, Bethesda, MD 4Biostatistics Core, Masonic Cancer Center, University of Minnesota, Minneapolis MN 5Division of Hematology, Oncology, and Transplantation, Department of Medicine, University of Minnesota, Minneapolis, MN 6Department of Radiation Oncology, University of Minnesota, Minneapolis, MN Background Overall and Disease-Specific Survival Lymph Node Biopsy and Survival Cutaneous adnexal carcinomas comprise a group of Vital status* All Sweat Hidradenocarc Spiradenocarci Sclerosin Porocarcin Eccrine Sebaceous Lymph Nodes All adnexal tumors adnexal gland inoma noma g sweat oma adenocarci carcinoma Lymph Nodes Examined carcino duct noma Nodes not examined 6592 (91.9) rare cutaneous malignancies that are generally ma tumor Nodes examined 578 (8.1) (MAC) Positive (% of examined) 138 (23.9) considered non-aggressive. Guidelines for the Stage (Derived AJCC N=1863 N=70 N=127 N=46 N=236 N=229 N=187 N=968 Negative (% of examined) 440 (76.1) Stage Group, 6th ed treatment of many of these malignancies are sparse, (2004-2015) Total N=1221 5-year OS 5-year DSS 1,2 I 1221 40 (57.1) 56 (44.1) 14 (30.4) 150 140 (61.1) 103 (55.1) 718 (74.2) Stage I Examined N=112 including guidance on surgical management (65.5) (63.6) Nodes not examined (% of total) 1109 (90.8) 69.7 (66.1-72.4) 99.3 (99.6-100) 3,4 II 440 14 (20.0) 54 (47.5) 28 (60.9) 47 (19.9) 64 (27.9) 51 (27.3) 182 (18.8) Nodes positive (% of examined) 0 (0) -- -- including the utility of lymph node biopsy.
    [Show full text]
  • University of Dundee Hidradenoma Masquerading Digital
    CORE Metadata, citation and similar papers at core.ac.uk Provided by University of Dundee Online Publications University of Dundee Hidradenoma masquerading digital ganglion cyst Makaram, Navnit; Chaudhry, Iskander H.; Srinivasan, Makaram S. Published in: Annals of Medicine and Surgery DOI: 10.1016/j.amsu.2016.07.017 Publication date: 2016 Document Version Publisher's PDF, also known as Version of record Link to publication in Discovery Research Portal Citation for published version (APA): Makaram, N., Chaudhry, I. H., & Srinivasan, M. S. (2016). Hidradenoma masquerading digital ganglion cyst: a rare phenomenon. Annals of Medicine and Surgery , 10, 22-26. DOI: 10.1016/j.amsu.2016.07.017 General rights Copyright and moral rights for the publications made accessible in Discovery Research Portal are retained by the authors and/or other copyright owners and it is a condition of accessing publications that users recognise and abide by the legal requirements associated with these rights. • Users may download and print one copy of any publication from Discovery Research Portal for the purpose of private study or research. • You may not further distribute the material or use it for any profit-making activity or commercial gain. • You may freely distribute the URL identifying the publication in the public portal. Take down policy If you believe that this document breaches copyright please contact us providing details, and we will remove access to the work immediately and investigate your claim. Download date: 17. Feb. 2017 Annals of Medicine and Surgery 10 (2016) 22e26 Contents lists available at ScienceDirect Annals of Medicine and Surgery journal homepage: www.annalsjournal.com Case report Hidradenoma masquerading digital ganglion cyst: A rare phenomenon * Navnit Makaram a, , Iskander H.
    [Show full text]
  • An Institutional Experience
    Original Research Article Skin Adnexal Tumors- An Institutional Experience 1 2* 3 4 5 6 Rekha M Haravi , Roopa K N , Priya Patil , Rujuta Datar , Meena N Jadhav , Shreekant K Kittur 1,5Associate Professor, 2Post Graduate Student, 3,4Assistant Professor, 6Professor & HOD, Department of Pathology, Belgaum Institute of Medical Sciences Dr B R Ambedkar Road, Belagavi, Karnataka – 590001, INDIA. Email: [email protected] Abstract Background: Skin adnexal tumors are a wide spectrum of benign and malignant tumors that differentiate towards one or more adnexal structures found in normal skin. The adnexal structures of skin are the hair follicles, sebaceous glands, eccrine and apocrine sweat glands. These skin adnexal tumors are often difficult to diagnose clinically. This retrospective study was undertaken to know the various histomorphological patterns of skin adnexal tumors at our institution and to determine the incidence among the genders and age groups along with the site distribution. Materials and methods: A total of 40 specimens received and diagnosed as skin adnexal tumors in the department of Pathology at Belgaum Institute of Medical Sciences, Belagavi for a period of 6 years from January 2014 to December 2019 were taken for the study. Histopathological slides prepared from tissue blocks retrieved from departmental archives were reviewed and classified according to the WHO classification 2017. Results: Out of the total 40 samples, benign tumors were 36 (90%) and malignant were 4 (10%). Largest group was the benign tumors of apocrine and eccrine differentiation (47.5%) followed by benign tumors of hair follicle differentiation (40%). Malignant tumors of sebaceous differentiation were 5%, malignant tumors of eccrine and apocrine differentiation were 2.5% and malignant hair follicle differentiation tumors were 2.5% of the total.
    [Show full text]
  • Eyelid Conjunctival Tumors
    EYELID &CONJUNCTIVAL TUMORS PHOTOGRAPHIC ATLAS Dr. Olivier Galatoire Dr. Christine Levy-Gabriel Dr. Mathieu Zmuda EYELID & CONJUNCTIVAL TUMORS 4 EYELID & CONJUNCTIVAL TUMORS Dear readers, All rights of translation, adaptation, or reproduction by any means are reserved in all countries. The reproduction or representation, in whole or in part and by any means, of any of the pages published in the present book without the prior written consent of the publisher, is prohibited and illegal and would constitute an infringement. Only reproductions strictly reserved for the private use of the copier and not intended for collective use, and short analyses and quotations justified by the illustrative or scientific nature of the work in which they are incorporated, are authorized (Law of March 11, 1957 art. 40 and 41 and Criminal Code art. 425). EYELID & CONJUNCTIVAL TUMORS EYELID & CONJUNCTIVAL TUMORS 5 6 EYELID & CONJUNCTIVAL TUMORS Foreword Dr. Serge Morax I am honored to introduce this Photographic Atlas of palpebral and conjunctival tumors,which is the culmination of the close collaboration between Drs. Olivier Galatoire and Mathieu Zmuda of the A. de Rothschild Ophthalmological Foundation and Dr. Christine Levy-Gabriel of the Curie Institute. The subject is now of unquestionable importance and evidently of great interest to Ophthalmologists, whether they are orbital- palpebral specialists or not. Indeed, errors or delays in the diagnosis of tumor pathologies are relatively common and the consequences can be serious in the case of malignant tumors, especially carcinomas. Swift diagnosis and anatomopathological confirmation will lead to a treatment, discussed in multidisciplinary team meetings, ranging from surgery to radiotherapy.
    [Show full text]
  • Adnexal Tumors
    10/24/2019 What’s a gland like you doing in a place like this? A practical approach to cutaneous adnexal neoplasms Hafeez Diwan, MD, PhD Departments of Pathology & Immunology and Dermatology Baylor College of Medicine 1 Conflict of interest • None 2 Disclosures • I have nothing to disclose 3 1 10/24/2019 Is the adnexal neoplasm glandular? And if so, where is it located? • Hands and Feet: Digital papillary adenocarcinoma 4 5 6 2 10/24/2019 7 8 Digital Papillary Adenocarcinoma • Solitary • Fingers/toes/palms/soles • Recurrence/metastases 9 3 10/24/2019 10 11 12 4 10/24/2019 3 Points about digital papillary adenocarcinoma • 1. Atypia doesn’t matter – if there is no atypia, it doesn’t mean that it isn’t digital papillary adenocarcinoma 13 3 Points about digital papillary adenocarcinoma • 1. Atypia doesn’t matter – if there is no atypia, it doesn’t mean that it isn’t digital papillary adenocarcinoma • 2. How high can the glandular lesion go up the extremity? • Example of one case that occurred on the thigh? (Alomari A, Douglas S, Galan A, Narayan D, Ko C. Atypical Presentation of digital papillary adenocarcinoma (abstract) J Cutan Pathol. 2014;41:221) 14 3 Points about digital papillary adenocarcinoma (cont’d) • 3. What if you don’t see glands • Hidradenoma on hands and feet • Hunt for a gland? If you see a gland, then what? • Probably best to err on the side of caution and say that a digital papillary adenocarcinoma is not ruled out 15 5 10/24/2019 16 17 18 6 10/24/2019 19 20 21 7 10/24/2019 3 Points about digital papillary adenocarcinoma (cont’d) • 3.
    [Show full text]
  • Histopathology of Dermal Adnexal Tumours - a Four Years Study
    International Journal of Science and Research (IJSR) ISSN (Online): 2319-7064 Index Copernicus Value (2013): 6.14 | Impact Factor (2015): 6.391 Histopathology of Dermal Adnexal Tumours - A Four Years Study Mukund Dhokiya1, Dr. Hemlata Talwelkar2, Dr. Sanjay Talwelkar3 13rd year Postgraduate Student, PDU Medical College & Hospital, Rajkot, India 2DA, MBBS, PDU Medical College & Hospital, Rajkot, India 3MD Pathology, Associate Professor, PDU Medical College & Hospital, Rajkot, India Abstract: Background: Dermal adnexal tumours (DAT) are a large and diverse group of benign and malignant tumours which exhibit morphological differentiation towards one of the different types of adnexal tumours present in normal skin: pilosebaeceous unit, eccrine and apocrine. Methods: 50 cases of skin adnexal tumours diagnosed in histopathological study over a period of 4 years (May 2012 to September 2016) in the Department of Pathology, PDU Medical College, Rajkot. Histopathological study is done in Formalin fixed, Paraffin embedded tissue sections stained with Haematoxylin and Eosin. Results: Skin adnexal tumours found most common in the age group of 21 to 60 years (74%, 37/50). Male to female ratio was 1:1.2. 98% cases found benign with only a single case (2%) malignant. The sweat gland tumors formed the largest group involving 52% of cases followed by hair follicle tumors (40%),sebaceous gland tumours (6%) and mixed (2%). Nodular hidradenoma (22%) and trichilemmal cyst (22%) found the most common benign tumours. Chondroid syringoma with malignant changes is the only malignant adnexal tumour reported in our study. Conclusion: Dermal adnexal tumours are relatively rare. Benign adnexal tumors are far more common than their malignant counterparts.
    [Show full text]
  • Inherited Skin Tumour Syndromes
    CME GENETICS Clinical Medicine 2017 Vol 17, No 6: 562–7 I n h e r i t e d s k i n t u m o u r s y n d r o m e s A u t h o r s : S a r a h B r o w n , A P a u l B r e n n a n B a n d N e i l R a j a n C This article provides an overview of selected genetic skin con- and upper trunk. 1,2 These lesions are fibrofolliculomas, ditions where multiple inherited cutaneous tumours are a cen- trichodiscomas and acrochordons. Patients are also susceptible tral feature. Skin tumours that arise from skin structures such to the development of renal cell carcinoma, lung cysts and as hair, sweat glands and sebaceous glands are called skin pneumothoraces. 3 appendage tumours. These tumours are uncommon, but can Fibrofolliculomas and trichodiscomas clinically present as ABSTRACT have important implications for patient care. Certain appenda- skin/yellow-white coloured dome shaped papules 2–4 mm in geal tumours, particularly when multiple lesions are seen, may diameter (Fig 1 a and Fig 1 b). 4 These lesions usually develop indicate an underlying genetic condition. These tumours may in the third or fourth decade.4 In the case of fibrofolliculoma, not display clinical features that allow a secure diagnosis to be hair specific differentiation is seen, whereas in the case of made, necessitating biopsy and dermatopathological assess- trichodiscoma, differentiation is to the mesodermal component ment.
    [Show full text]
  • Cancer Immunoprevention: a Case Report Raising the Possibility of “Immuno-Interception” Jessica G
    CANCER PREVENTION RESEARCH | RESEARCH BRIEF Cancer Immunoprevention: A Case Report Raising the Possibility of “Immuno-interception” Jessica G. Mancuso1, William D. Foulkes1,2,3, and Michael N. Pollak1,2 ABSTRACT ◥ Immune checkpoint blockade therapy provides substan- or neoplastic lesions over a period of 19 years (mean tial benefits for subsets of patients with advanced cancer, 7.5 neoplasms/year, range 2–26) prior to receiving but its utility for cancer prevention is unknown. Lynch pembrolizumab immunotherapy as part of multi- syndrome (MIM 120435) is characterized by defective modality treatment for invasive bladder cancer. He not DNA mismatch repair and predisposition to multiple only had a complete response of the bladder cancer, but cancers. A variant of Lynch syndrome, Muir–Torre also was noted to have an absence of new cancers during a syndrome (MIM 158320), is characterized by frequent 22-month follow-up period. This case adds to the rationale gastrointestinal tumors and hyperplastic or neoplastic skin for exploring the utility of immune checkpoint blockade tumors. We report the case of a man with Muir–Torre forcancerprevention,particularlyforpatientswithDNA syndrome who had 136 cutaneous or visceral hyperplastic repair deficits. Introduction There is an obvious clinical need to reduce cancer incidence in patients with DNA repair deficits, and prophylactic surgery The clinically demonstrated utility of antiviral vaccines to is commonly employed. Clinical trials designed to evaluate reduce risk of virally initiated cancers represents a major strategies to reduce cancer incidence are challenging: in popu- success in cancer immunoprevention. There is interest in the lations where baseline risk is low, a large number of subjects and possibility that immunoprevention may also be useful where long follow-up periods are required.
    [Show full text]
  • The Best Diagnosis Is: H&E, Original Magnification 2
    Dermatopathology Diagnosis The best diagnosis is: H&E, original magnification 2. a. adenoid cysticcopy carcinoma arising within a spiradenoma b. cylindroma and spiradenoma collision tumor c. microcysticnot change within a spiradenoma d. mucinous carcinoma arising within a spiradenoma Doe. trichoepithelioma and spiradenoma collision tumor CUTIS H&E, original magnification 100. PLEASE TURN TO PAGE 211 FOR DERMATOPATHOLOGY DIAGNOSIS DISCUSSION Amanda F. Marsch, MD; Jeffrey B. Shackelton, MD; Dirk M. Elston, MD Dr. Marsch is from the Department of Dermatology, University of Illinois at Chicago. Drs. Shackelton and Elston are from the Ackerman Academy of Dermatopathology, New York, New York. The authors report no conflict of interest. Correspondence: Amanda F. Marsch, MD, University of Illinois at Chicago, 808 S Wood St, Chicago, IL 60612 ([email protected]). 192 CUTIS® WWW.CUTIS.COM Copyright Cutis 2015. No part of this publication may be reproduced, stored, or transmitted without the prior written permission of the Publisher. Dermatopathology Diagnosis Discussion Trichoepithelioma and Spiradenoma Collision Tumor he coexistence of more than one cutaneous adnexal neoplasm in a single biopsy specimen Tis unusual and is most frequently recognized in the context of a nevus sebaceous or Brooke-Spiegler syndrome, an autosomal-dominant inherited disease characterized by cutaneous adnexal neoplasms, most commonly cylindromas and trichoepitheliomas.1-3 Brooke-Spiegler syndrome is caused by germline muta- tions in the cylindromatosis gene, CYLD, located on band 16q12; it functions as a tumor suppressor gene and has regulatory roles in development, immunity, and inflammation.1 Weyers et al3 first recognized the tendency for adnexal collision tumors to present in patients with Brooke-Spiegler syndrome; they reported a patient with Brooke-Spiegler syndrome with spirad- Figure 1.
    [Show full text]