SKIN ADNEXAL Tumours and it's approaching

This describes about skin adrenal tumours and its approach

1. CASE

2. CLINICAL HISTORY 55 year old female Ms.Jayalaksmi presented with complaints of right inguinal lymphadenopathy. PAST HISTORY: No history of previous malignancies in the past CT Abdomen: No significant changes

3. CYTOLOGY Predominantly epithelioid granulomas with few clusters Few clusters showing mild pleomorphism and anisonucleosis .

4. GROSS Nodular mass Predominantly located in the dermis and subcutis C/S variegated with grey white and grey brown areas Predominently soild , firm areas Few cystic areas were filled with gelatinous material Few friable areas seen

5. MICROSCOPY Tumour occupying deep dermis and subcutaneous tissue. Infiltrating malignant neoplasm Composed of cells arranged in nodules, sheets, nests and glandular pattern nodules separated by a fibrous septa,.

6. The sheets of tumour cells are also intercepted by cystic spaces filled with eosinophilic secretions and surrounded by foamy macrophages.

7. Round to polygonal cells Indistict cytoplasm Mild pleomorphism Coarse chromatin and irregular nuclei Prominent nucleoli

8. Extensive areas of Tumour infiltrating lymphocytes seen. Few epithelioid granulomas and multinucleate giant cells also made out.

9. IMPRESSION Malignant skin adnexal tumour - probably of eccrine origin

10. APPROACH TO SWEAT GLAND TUMOURS BENIGN APOCRINE AND ECCRINE TUMOURS: • Hidrocystoma /cystadenoma • Syringoma • Poroma • Syringofibroadenoma • Hidradenoma • Spiradenoma • Cylindroma • Tubular adenoma • Syringocystadenoma papilliferum • Mixed tumour • Myoepithelioma

11. MALIGNANT APOCRINE AND ECCRINE TUMOUR • Adnexal adenocarcinoma, NOS • Microcystic adnexal carcinoma • Cribriform tumour • Porocarcinoma • NUT carcinoma • Malignant neoplasms arising from spiradenoma , cylindroma or spiradenoma

12. • Malignant mixed tumour • Hidradenocarcinoma • Endocrine mucin producing sweat gland carcinoma • Mucinous carcinoma • Digital papillary adenocarcinoma • Adenoid cystic carcinoma • Apocrine carcinoma • Syringocystadenocarcinoma papilliferum • Secretory carcinoma • Signet ring cell/histiocytoid carcinoma

13. Clues to sweat gland and ductal differentiation: • Glandular differentiation: either eccrine or apocrine • Decapitation secretion/intracytoplasmic zymogen: “apocrine differentiation” • True ducts highlighted by PAS

14. SWEAT GLAND TUMOURS BY BASIC HISTOLOGIC PATTERN

15. PATTERN 1: DERMAL CYST WITH DOUBLE CUBOIDAL/COLUMNAR LINING Pattern 1: Dermal cyst with double cuboidal/columnar lining HYDROCYSTOMA/CYSTADENOMA: Hidrocystoma is a solitary cystic nodule usually located in the lower eyelid. Histologically, cystic lesion is lined by a single layer of columnar to cuboidal cells.

16. PATTERN 2: SOLID PINK/CLEAR/ SQUAMOID PROLIFERATION IN EPIDERMIS/OR DERMIS+CYSTIC CHANGE • Acrospiroma • Low power- eosinophilic squamoid appearance, ductal lumen+

17. POROMA • Sessile nodule, sometimes red and scaly. • Sole or side of the foot • HPE: Small round bland poroid cells that fill elongated rete. • Diagnostic clue- abrupt demarcation from the overlying/adjacent epidermal keratinocytes.

18. HPE A well circumscribed basaloid neoplasm confined to the epidermis

19. At high power, the neoplasm is composed of two populations- • Poroid cells have a rounded or oval nucleus and scant cytoplasm. • Cuticular cells have ample eosinophilic cytoplasm and a central nucleus.

20. DD to poroma: Porocarcinoma Porocarcinoma : Spontaneous necrosis and infiltrative growth Individual cells show nuclear enlargement

21. HIDRADENOMA • Flesh-colored or erythematous nodule, usually in adults, that can occur anywhere • HPE: circumscribed dermal nodule, often multilobulated, and sometimes with prominent clear cell and/or central cystic change • Well formed ducts+ • Clear cells- may mimic metastatic RCC; due to abdundant intracellular glycogen No epidermal connection + clear and/or eosinophilic cytoplasm ± cystic changes = hidradenoma and hidradenocarcinoma

22. Pattern 3: Blue basaloid proliferation in dermis Spiradenoma and cylindroma • Morphological overlap with cylindroma . • Painful nodules on the ventral surfaces of the body above the waist. No epidermal connection + basaloid tumor + two cell types + intermingled lymphocytes = spiradenoma and cylindroma

23. SPIRADENOMA

24. Small basaloid cells at the periphery of nests, and larger ovoid cells with open chromatin toward the center of nests. Spiradenomas are also often peppered with lymphocytes. (Hematoxylin-eosin, original magnification ×400).

25. CYLINDROMA Multiple aggregates of basaloid cells- ”Jigsaw appearance” • Prominent basement membrane that gives a positive periodic acid–Schiff (PAS) reaction

26. Pattern 4- Tadpoles/Paisley tie- Syringoma • Clinically- small skin coloured papules often on the face • Eruptive lesions – neck, chest, axillae, anogenital areas

27. HPE • Superficial, ill-defined dermal tumor with background sclerosis. • Syringoma : on a high power, the tumor is composed of small solid nests, cords, and tubules of epithelial cells within a dense stroma .

28. Cystic spaces and papillary projections Syringocystadenoma papilliferum - benign apocrine neoplasm • HPE: Multiple downward extensions from the epidermis forming small cystic areas lined by double layer of epithelial and myoepithelial cells with evidence of decapitation (apical snout) secretion Epidermal connection + ducts, glands, and papillae = syringocystadenoma papilliferum

29. Pattern 6: Dermal Nodule With Variable Mixture of Cords/Chains/Tubules and Chondromyxoid Stroma Mixed Tumor (Chondroid Syringoma ) Benign sweat gland neoplasm composed of epithelial, myoepithelial, and mesenchymal elements; it is the cutaneous analogue of pleomorphic adenoma

30. Well-circumscribed smooth-surfaced nodule; histologically containing tubules and cords of glandular epithelium embedded in a myxohyalinized or a cartilaginous stroma; In eccrine mixed tumours, there are no signs of decapitation secretion, sebaceous or follicular differentiation.

31. METASTATIC TUMOURS VS PRIMARY CUTANEOUS ADNEXAL NEOPLASM • Primary cutaneous adnexal adenocarcinomas with sweat gland differentiation may raise suspicion for a metastatic adenocarcinoma from a visceral primary site • P63/P40 maybe used for almost all primary cutaneous adnexal neoplasms. • Except skin metastasis from squamous cell carcinoma, urothelial carcinoma and many salivary carcinomas- will also be positive for the above markers

32. REFERENCES Edward H Fulton, Jennifer R Kaley, Jerad M Gardner; Skin Adnexal Tumors in Plain Language: A Practical Approach for the General Surgical Pathologist. Arch Pathol Lab Med 1 July 2019; 143 (7): 832–851. doi : https://doi.org/10.5858/arpa.2018-0189-RA • Alhumidi AA. Simple approach to histological diagnosis of common skin adnexal tumors . Pathology and Laboratory Medicine International. 2017;9:37-47 https://doi.org/10.2147/PLMI.S139767 • WHO Classification of Tumours Editorial Board. Skin tumours. Lyon (France): International Agency for Research on Cancer; forthcoming. (WHO classification of tumours series, 5th ed.; vol. 12). https://publications.iarc.fr. • Weedon’s skin pathology • Lever’s histopathology