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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. 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 • P63? • Positive in basal layer of digital papillary adenocarcinoma vs. throughout in hidradenoma? 22 Digital papillary adenocarcinoma – quasi- controversial aspects • Previously used to be called aggressive, till the “aggressive” was dropped • Now, some authors have suggested they may not be that aggressive (because a myoepithelial layer is present, so is it really adenocarcinoma in situ?) • However, as there is high potential for metastasis, with a local recurrence rate of 50% and a 14% rate of metastasis, most commonly to the lungs (70%), and since atypia is no guide to how these lesions will behave, it is probably best to call glandular adnexal neoplasms on hands and feet adenocarcinoma 23 Is it on or near the eyelid and does it have a cribriform appearance 24 8 10/24/2019 25 26 27 9 10/24/2019 Endocrine mucin-producing sweat gland carcinoma • EMPSGC is histologically and immunohistochemically similar to solid papillary carcinoma of breast/ endocrine ductal carcinoma in situ, which is considered a precursor lesion to mucinous carcinoma • EMPSGC is believed to be a precursor of cutaneous mucinous carcinoma (MC) 28 Histology • Cystic, partially cystic and/or solid tumor nests • Low-grade cytology • Neuroendocrine differentiation • Mucin production 29 Immunohistochemical studies • Expression of a specific neuroendocrine marker is required for the diagnosis of EMPSGC. Positive Staining Negative Staining ER CK20 PR CK7 LMWCK (CAM 5.2) CD56/synaptophysin/chromogranin Myoepithelial cells: SMA, p63, calponin (Discordant staining between authors) 30 10 10/24/2019 EMPSGC Mucin production is typically non- copious No infiltrative growth pattern Invasive mucinous carcinomas Nests and strands of cells infiltrating the dermis Advanced cases: clusters of tumor cells floating in large stromal pools of mucin 31 32 33 11 10/24/2019 Mucinous Eccrine Carcinoma • Face/scalp/axilla/trunk • Recurrence • Metastasis 34 Does it have a cribriform appearance and is well-circumscribed? Cutaneous Cribriform Carcinoma J-D Wu et al. Indian J Dermatol Venerol Leprol. 2018; 84(5):569-572: Neck of 26 year-old female 35 Cutaneous cribriform carcinoma • S100, C-kit positive; calponin, p63, SMA negative (helps distinguish from a tubular adenoma) – DP Arps et al. J Cutan Pathol. 2015; 42:379-387: • 6 cases (3 leg, 2 arm, 1 elbow) • 5/6 had lymphoid aggregates • All had desmoplastic stroma • Circumscribed (unlike adenoid cystic carcinoma ACC, which is also C-kit & S100 positive; but ACC is not circumscribed and is neurotropic) 36 12 10/24/2019 YG Xu et al. J Oncol. 2010 Apr 7. doi: 10.1155/2010/469049 Pathology Outlines (GA Tranesh); Photo: Dr. Mark Wick 37 38 Beware of adenoid cystic carcinoma on the lower portion of the face! • It could be salivary gland adenoid cystic carcinoma extending into the skin. 39 13 10/24/2019 Does it have prominent eosinophilic secretions? Cutaneous secretory carcinoma L Requena & O Sangueza. Cutaneous adnexal neoplasms. Springer, 2018 No particular anatomic site Positive for S100, EGFR, p63, calponin, SMA Negative for CEA, p53, Her-2 neu Positive for ETV6-NTRK3 (like breast secretory carcinoma) 40 L Requena & O Sangueza. Cutaneous adnexal neoplasms. Springer, 2018 41 Does it have squamous or squamoid appearing areas with ducts? 42 14 10/24/2019 van der Horst MPJ, Brenn T. Surgical Pathology 10: 383– 397, 2017 43 van der Horst MPJ, Brenn T. Surgical Pathology 10: 383– 397, 2017 44 van der Horst MPJ, Brenn T. Surgical Pathology 10: 383– 397, 2017 45 15 10/24/2019 Squamoid eccrine carcinoma • Head and neck of elderly males • Most common: face • High recurrence • Can metastasize (most often to lymph nodes, but elsewhere as well) • Complete excision and clinical follow-up 46 Does it remind you of a rabbit burrow? 47 48 16 10/24/2019 THE RABBIT BURROW 49 50 51 17 10/24/2019 52 53 54 18 10/24/2019 55 Syringocystadenoma Papilliferum • Scalp and forehead • Other sites possible • Associated with NS • Coexistent BCC also possible 56 Does it remind you of a maze 57 19 10/24/2019 58 59 60 20 10/24/2019 THE MAZE 61 62 63 21 10/24/2019 64 Hidradenoma papilliferum with lactational changes 65 Primary vs metastatic • Primary – p63, p40, CK15, calretinin, D2-40, CK5/6 • Primary – look for a benign component; if there is one, it might be primary • Caveats • Triple negative BCC and lung CA can be p63 positive • Calretinin will be positive in metastatic mesothelioma • Apocrine carcinoma is p63 negative 66 22 10/24/2019 Primary vs metastatic • What about clear cells as in hidradenocarcinoma? • Ducts will be EMA/CEA, tumor itself will be LMWK and S100 • Renal cell carcinoma can have clear cells – but PAX8, CD10, RCC can help • Thyroid carcinoma can have clear cells – but TG and TTF-1 can help 67 Sebaceous neoplasms – to mismatch or not to mismatch • High false positives • Do it if the lesions look cystic or “complicated” or if there is a keratoacanthoma with sebaceous differentiation, or • If there are multiple lesions • If there is a family history 68 69 23 10/24/2019 70 71 72 24 10/24/2019 Is it sebaceous or not? • EMA (also SCC, but not BCC) • Adipophilin • Factor 13a • AR (but BCC can be positive) • NOT USEFUL ONES: • BerEP4 (BCC and sebaceous) • Cam5.2 (BCC, SCC, and sebaceous) • CK7 (BCC, SCC, and sebaceous) 73 Danialan R, Mutyambizi K, Aung PP, Prieto VG, Ivan D. J Clin Pathol 2015;68: 992–1002. 74 75 25 10/24/2019 Tjarks et al., JCP 45: 1-7, 2018 Factor 13a clone AC-1A1 76 Tjarks et al., JCP 45: 1-7, 2018 Factor 13a clone AC- 1A1) 77 What about focal sebaceous differentiation? • If it looks like SCCIS, or BCC, or invasive SCC with focal sebaceous differentiation, then it is probably just focal sebaceous differentiation rather than a sebaceous neoplasm. 78 26 10/24/2019 It might be benign, if it weren’t malignant • Can you see the bottom of the lesion? • Can you see the entire lesion? • Is the border infiltrative? 79 • 80 year-old male with left arm lesion – 11/30/12 80 81 27 10/24/2019 82 83 84 28 10/24/2019 • 2 months later, 1/28/13, the lesion was re-excised 85 86 87 29 10/24/2019 88 89 90 30 10/24/2019 91 92 93 31 10/24/2019 94 P16 95 • 87 year-old, center forehead lesion, 8/17/2012 96 32 10/24/2019 97 98 99 33 10/24/2019 100 • Nearly a year and a half later, re-biopsy 101 102 34 10/24/2019 103 104 105 35 10/24/2019 106 What might help in distinguishing benign from malignant sebaceous neoplasms • P53, Ki-67 – both increased in sebaceous carcinoma 107 Related concept – what’s the architecture of the lesion? • Circumscribed or infiltrative? • Even if it doesn’t look too atypical, infiltrativeness trumps atypia 108 36 10/24/2019 109 110 111 37 10/24/2019 112 Is there cytologic atypia? • If the border is infiltrative, then lack of atypia doesn’t matter much • But cytologic atypia can be helpful 113 114 38 10/24/2019 115 116 117 39 10/24/2019 118 119 120 40 10/24/2019 121 122 123 41 10/24/2019 124 Porocarcinoma • Local recurrence • Metastasis – both regional and distant 125 126 42 10/24/2019 127 Eccrine Carcinoma • Rare • Scalp/extremities/trunk • Local recurrence 128 129 43 10/24/2019 130 131 132 44 10/24/2019 133 Malignant pilomatricoma 134 Trichoepithelioma vs. BCC vs. MAC vs syringoma 135 45 10/24/2019 136 137 138 46 10/24/2019 BCC vs. TE • Bcl2 – BCC (also desmoplastic TE); peripheral cells in TE • BerEP4 – BCC (also desmoplastic TE) • CK 20 – TE • CD34 – TE stroma • CD10 – TE stroma; CD10 – BCC tumor • AR – BCC – But some studies show AR in TE • ?p75 in BCC and desmoplastic TE 139 140 141 47 10/24/2019 142 143 CD34 144 48 10/24/2019 CK20 145 Desmoplastic Trichoepithelioma • Face • Solitary • Very important differential diagnosis 146 147 49 10/24/2019 148 149 150 50 10/24/2019 Syringoma • Multiple, lower eyelids and cheeks • Adolescent females • Clear cell variant in DM • More
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  • Pilomatricoma: a Case Report

    Pilomatricoma: a Case Report

    Open Access Austin Journal of Dermatology Case Report Pilomatricoma: A Case Report Jayakar Thomas*, Tamilarasi S, Asha D and Zohra Begum C Abstract Department of Dermatology, Sree Balaji Medical College Pilomatricoma is a benign tumor that arises from hair follicle matrical cells. & Bharath University, India Involvement of the upper limb is relatively uncommon and can be mistaken for *Corresponding author: Jayakar Thomas, other soft tissue tumors. We report the case of a 12 year old boy, who presented Department of Dermatology, Sree Balaji Medical College with an asymptomatic firm nodule over the left arm whose histology was & Bharath University, Chennai 600044, India, Email: suggestive of Pilomatricoma. [email protected] Keywords: Pilomatricoma; Shadow cells; Ghost cells; Basaloid cells Received: April 05, 2015; Accepted: May 29, 2015; Published: June 02, 2015 Introduction 1). The neurovascular status of the left hand was noted to be intact; there were no other palpable masses in the extremities and no axillary Pilomatricoma also known as Pilomatrixoma or calcifying adenopathy was present. Excision biopsy was performed under epithelioma of Malherbe is a benign neoplasm, which is derived regional anesthesia. Grossly the tumor was white in appearance and from hair follicle matrix cells. These tumors are typically present well circumscribed. in the head and neck region, but also occur in the upper limbs and are rarely reported in other sites[1].Pilomatricoma represents as an Histopathology revealed a capsulated benign neoplasm over asymptomatic, solitary, firm to hard, freely mobile nodule of the the subcutis, composed of lobules ofirregularly shaped masses of dermis or subcutaneous tissue. These tumors are generally exhibits ghost or shadow cells, scattered basophilic cells that undergo abrupt no fixation to neighbouring tissues and have an osseous- or cartilage- keratinization forming ghost [shadow] cells and areas of calcification.
  • Giant Proliferating Pilomatricoma and Superficial Angiomatous Proliferation: Case Report and Review of the Literature

    Giant Proliferating Pilomatricoma and Superficial Angiomatous Proliferation: Case Report and Review of the Literature

    Yu K, et al., J Clin Dermatol Ther 2020, 6: 042 DOI: 10.24966/CDT-8771/100042 HSOA Journal of Clinical Dermatology and Therapy Case Report angiomyxoma has rarely been reported to be associated with pilo- Giant Proliferating Pilomatricoma matricoma. In the current report, we describe a case of a giant pro- liferating pilomatricoma associated with superficial angiomatous and Superficial Angiomatous proliferation. This tumor was unusually large and the rapid growth raised clinical suspicion of malignancy. The pathogenesis of such an Proliferation: Case Report and association will be discussed in this report. Review of the Literature Case Presentation A 83 year-old woman presented with an enlarging mass on the Kate Yu1, Natasha Singh1, Amin Maghari2 and Yong Kang2* thoracic area of her back. The mass was noted several years prior and 1Department of Pathology, Robert Wood Johnson Banarbas Health/Saint slowly growing since. Recent rapid growth raised clinical suspicion Banarbas Medical Center, Livingston, New Jersey, USA of malignancy. The physical examination revealed a 13 x 10 cm firm, exophytic, and reddish-brown mass with overlying bullous skin in 2 Department of Pathology, Robert Wood Johnson Banarbas Health/Mon- posterior thorax (Figure 1). She denied any prior surgery or trauma mouth Medical Center, Long Branch, New Jersey, USA to the affected area. There was no family history of similar lesions or known genetic syndromes. Macroscopic examination showed a well-circumscribed, nodular, dull white mass, measuring 12 x 9 x Abstract 4.7 cm. Sectioning of the mass found significant ossification of the involved tissue. Microscopic examination revealed a mutilobular pro- Giant proliferating pilomatricoma is a rare variant of pilomatrico- ma.