Difference between revisions of "Dermatologic neoplasms"

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Notes:
Notes:
*Area above gland appears crusted.
*Area above gland appears crusted.
Images:
*[http://www.flickr.com/photos/40981620@N04/3808316834/in/photostream/ Eccrine poroma - low mag. (flickr.com)]
*[http://www.flickr.com/photos/40981620@N04/3807502071/in/photostream Eccrine poroma - intermed. mag. (flickr.com)].


==Trichoepithelioma==
==Trichoepithelioma==

Revision as of 18:24, 9 September 2011

This article deals with dermatologic neoplasms. It includes dermatologic cancer, which can be deadly. Collectively, dermatologic cancers are the most common form of cancer.

An introduction to dermatopathy is found in the dermatopathology article. Non-malignant disease is covered in the non-malignant skin disease article.

The Big Three malignant

Basal cell carcinoma

  • Abbreviated BCC.

General

  • Very common.
  • Sun exposed skin.
  • Hair bearing area; tumour derived from hair follicle - a more appropriate name might be trichoblastic carcinoma.[1]
  • Very rarely metastasizes:
    • Dermatopathologists might see a couple in their career.
    • There are only ~ 300 literature reports of metastatic BCC.[2]

Clinical

  • Telangiectasias.
  • Raised pearly nodule.

As part of a syndrome

Microscopic

Features:[4][5]

  1. Basaloid cells - similar in appearance to basal cells:
    • Moderate blue/grey cytoplasm.
    • Dark ovoid/ellipsoid nucleus with uniform chromatin.
  2. Palisading of cells at the edge of the cell nests.
  3. Artefactual separation of cells (forming the nests) from the underlying stroma - key feature.
  4. Surrounded by blue (myxoid) stroma - key feature.

May be present:[5]

  • Dystrophic calcification.
  • Amyloid.
  • Inflammation.

Notes:

  • Palisading = the long axes of the cells are alined and the axes are perpendicular to the interface between the (basaloid cell) nests and stroma.
  • Key elements in a list: Artefactual clefting (of nests), Basaloid cells, Peripheral palisading, Myxoid stroma.
    • Memory device PAM: palisading, artefactual clefts, myxoid stroma.

DDx:

Images:

Subtypes/unique features

Many patterns exist:[8]

Pattern Key histologic feature Other histologic features Other
Superficial pattern connected to epidermis
Nodular pattern nodules partial detachment from epidermis subgroup micronodular = nests equal size ~ 0.2 mm dia., >=25% of lesion
Morpheaform (sclerosing) pattern stroma sclerosis often seen with infiltrative pattern
Infiltrative pattern small irregular cell aggregates often also sclerosing or morpheaform
Fibroepitheliomatous pattern cords and columns of basaloid cells fibrous stroma name of pattern comes from fibroepithelioma of Pinkus
Infundibulocystic pattern small keratocysts (keratin cysts) usu. small, often in cords usu. indolent
Adenoidal pattern cribriform / pseudoglandular arch. myxoid stroma, peripheral palisading DDx: adenoid cystic carcinoma

Unique features/differentiation:[8]

Differentiation / unique cell Key histologic feature Other histologic features Other
Pigmented cells any pattern can have pigmentation pigment may be in malignant cell DDx: collision lesion with melanocytic lesion
Squamous differentiation (metatypical BCC) pink cytoplasm, keratinization assoc. with ulceration/tumour recurrence
Eccrine differentiation focal duct formation very rare, DDx: BCC engulfing sweat ducts
Clear cells (Clear cell BCC) clear cytoplasm due to glycogen

IHC

  • CK5/6 +ve.
    • Useful to assess margins... if very close.

Squamous cell carcinoma

Main article: Squamous carcinoma

General

Precursor:[9]

  • Actinic keratosis (solar keratosis).
    • Clinical: yellow-brown scaly, patches, sandpaper sensation.

Risk factors:[9]

  • Sun exposure.
  • Immune suppression (e.g. organ transplant recipients).

Notes:

  • Keratoacanthoma.
    • Some don't believe this entity exists.
      • These people sign this entity as low grade squamous cell carcinoma, keratoacanthoma type.[10]

Microscopic

Bowen disease

Bowen disease is squamous cell carcinoma in situ of the skin.

  • Its histomorphologic appearance may be similar to Paget disease, Toker cell hyperplasia and melanoma. **IHC is used to separate the entities definitively.

Histologic DDx of Bowen disease:

Images:

IHC

Bowen's disease panel:

Melanoma

Main article: Malignant melanoma

General

  • Known as the great mimicker in pathology; it may look like many things.

Microscopic

Features:

  • Classic appearance of melanoma:
    • Loosely cohesive; mix of small nests of cells, single cells.
    • Mixed of spindle and ovoid cell morphology.
    • +/-Occasional large binucleated cells.
    • Cytoplasm: brown pigment (melanin).
    • Prominent (large) red nucleoli (like in serous carcinoma of the ovary).
    • Marked nuclear pleomorphism - variation in cell size, shape & staining (like in serous carcinoma of the ovary).
    • Nuclear pseudoinclusions (like in papillary thyroid carcinoma).

Less common malignant

Dermatofibrosarcoma protuberans

  • Abbreviated DFSP.

General

  • Dermal location.
  • Destroys adnexal structures.

Treatment:[14]

  • Wide excision.
  • May include imatinib (Gleevec).

Microscopic

Features:[14]

  • Dermal spindle cell lesion with storiform pattern.
    • Spokes of the wheel-pattern.
  • Contains adipose tissue within the tumour -- key feature.
    • Described as "honeycomb pattern" and "Swiss cheese pattern".

Notes:

  • Adnexal structure within tumour are preserved -- this is unusual for a malignant tumour -- important.


Main DDx:

  • Dermatofibroma - has entrapment of collagen bundles at the edge of the lesion.

DDx of storiform pattern:

Images:

IHC

Panel:[15]

  • CD34 +ve.
    • Usually negative in dermatofibroma.[16][17]
  • Factor XIIIa -ve.
    • Usually positive in dermatofibroma.[16][17]
  • S100 -ve (screen for melanoma).
  • Caldesmin -ve (screen for muscle differentiation).
  • Beta-catenin. (???)
  • MIB-1 (proliferation marker).
    • Should not be confused with MIB1 a gene that regulates apoptosis.

Molecular

A characteristic translocation is seen:[18] t(17;22)(q22;q15) COLA1/PDGFB.

Cutaneous B-cell lymphoma

  • Abbreviated CBCL.

General

  • CBCL is less common than cutaneous T-cell lymphoma (CTCL).[19]

Microscopic

Features:

  • Dermal lymphoid infiltrate.
  • "Grenz zone" - space between the epidermis and the dermal infiltrate - key feature.

IHC

  • B cell and T cell markers.

Cutaneous T-cell lymphoma

  • Abbreviated CTCL.

General

  • Mycosis fungoides - is a subtype (???).
  • CTCL is more common than cutaneous B-cell lymphoma (CBCL).[20][21]

Microscopic

  • Atypical lymphocytes:
    • Have folded "cerebriform" nuclei; Sezary-Lutzner cells.[22]
  • Grouping:
    • Nests in the epidermis - known as "Pautrier microabscesses".
    • Single lymphocytes in epidermis; "lymphocyte exocytosis".[23]
    • Short linear arrays of lymphocytes along the basal layer of the epidermis; "epidermotropism".[22]

Images:

IHC

Features:

Merkel cell carcinoma

  • Abbreviated MCC.

General

  • Aggressive.
  • Rare.
  • Increased risk in the immunodeficient.
  • Most caused by Merkel cell polyomavirus.[25]

General

Features:[26]

  • Rare.
  • Aggressive course/poor prognosis.
  • Neuroendocrine-like.[27]

Etiology:

  • Polyomavirus (?).[26]
  • Immunocompromised/immunosuppressed (e.g. organ transplant recipients).

Microscopic

Features:[28]

  • Neuroendocrine nuclear features - round nucleus, small nucleoli/no nucleolus, stippled chromatin - key feature.
  • Typically medium size cells ~3x resting lymphocyte.
    • May be small or large.
  • Architecture: nests, sheets or trabeculae.
  • Scant cytoplasm.
  • Abundant mitoses.
  • +/-Nuclear moulding.

DDx:

Images:

IHC

Features:

  • CK7 -ve.
  • CK20 +ve (perinuclear ???).
  • CAM5.2 +ve (dot-like pattern).

Others:

  • TTF-1 -ve.

Eccrine carcinoma

General

  • Arises from the proximal sweat duct.

Microscopic

Features:

  • Pleomorphic nuclei with nucleoli.
  • Duct-like structures - key feature.
  • Extends from dermis into epidermis (follows path of a benign sweat duct).

Notes:

Images:

Kaposi sarcoma

See Kaposi sarcoma.

Sebaceous carcinoma

General

Notes:

Microscopic

Features:

  • Nuclear atypia.
  • Sebaceous differentiation:
    • Abundant pale fluffy cytoplasm.

Image:

Intermediate

Atypical fibroxanthoma

  • Abbreviated AFX.

General

Clinical:

  • Rapid growth.
  • Elderly.
  • Good prognosis.[32]

Microscopic

Features:[33]

  • Dermal lesion - key point.
  • Marked nuclear atypia.
  • Mitoses.
  • Mulitnucleated cells.
  • Foamy cytoplasm - key feature.

DDx:

Notes:

  • No Grenz zone. (???)

Image:

IHC

Features:[33]

  • S100 -ve (done to r/o melanoma).
  • 34betaE12 -ve, p63 -ve (done to exclude SCC).
  • Desmin -ve (done to r/o leiomyosarcoma).

Benign

Sebaceous adenoma

General

Microscopic

Features:

  • Abnormal sebaceous glands (pale fluffy cytoplasm):
    • Increased basal epithelium.
    • Multiple dilated glands - opening to the surface.

Image:

Trichilemmoma

  • May be spelled tricholemmoma.

General

  • Benign neoplasm with features of the pilosebaceous follicular epithelium.[35]
  • Associated with nevus sebaceous.[36]
  • Muliple trichilemmomas associated with Cowden syndrome.[37]

Microscopic

Features:[37]

  • Superficial dermal lesion contiguous with the epidermis:
    • Core of lesion:
      • Cuboidal cells with round nuclei, eosinophilic-clear cytoplasm.
    • Periphery of lesion:
      • Surrounded by hyaline band.
      • Peripheral palisading.

Images:

DDx:

Eccrine poroma

  • AKA nodular hidradenoma. (???)

General

  • Benign tumour arising from the distal sweat duct.
  • Erythematous - gross.

Microscopic

Features:[40]

  • Broad sheets of basaloid cells containing ductal structures - key feature.
  • Biphasic stroma:
    1. Edematous stroma.
    2. Sclerotic stroma.
  • Moderate nuclear pleomorphism.
  • +/-Occasional mitoses.

Notes:

  • Area above gland appears crusted.

Images:

Trichoepithelioma

General

  • Benign.
  • May be familial:
    • Multiple familial trichoepithelioma.[41]
    • Brooke-Spiegler syndrome.

Microscopic

Features:[42]

  • Well-circumscribed cell nest in the superficial dermis.
  • Surrounding by a fibrous stroma.
  • Basaloid cells with peripheral pallisading.
  • +/-Surround keratin-filled cysts.
  • Fibroblasts-like cell aggregate, similar to a follicular papillae (papillary-mesenchymal body).

Notes:

Variants:

  • Desmoplastic.

DDx:

Images:

See also

References

  1. ↑ Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 389. ISBN 978-0443066542.
  2. ↑ Ting, PT.; Kasper, R.; Arlette, JP. (Jan 2005). "Metastatic basal cell carcinoma: report of two cases and literature review.". J Cutan Med Surg 9 (1): 10-5. doi:10.1007/s10227-005-0027-1. PMID 16208438.
  3. ↑ URL: http://emedicine.medscape.com/article/1101146-diagnosis. Accessed on: 6 May 2010.
  4. ↑ 4.0 4.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1180-1. ISBN 978-1416031215.
  5. ↑ 5.0 5.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 390. ISBN 978-0443066542.
  6. ↑ Tadrous, Paul.J. Diagnostic Criteria Handbook in Histopathology: A Surgical Pathology Vade Mecum (1st ed.). Wiley. pp. 284. ISBN 978-0470519035.
  7. ↑ URL: http://missinglink.ucsf.edu/lm/DermatologyGlossary/basal_cell_carcinoma.html. Accessed on: 4 September 2011.
  8. ↑ 8.0 8.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 392-5. ISBN 978-0443066542.
  9. ↑ 9.0 9.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1180. ISBN 978-1416031215.
  10. ↑ RS. 17 May 2010.
  11. ↑ RS. May 2010.
  12. ↑ URL: http://emedicine.medscape.com/article/1101235-workup#a0721. Accessed on: 2 September 2011.
  13. ↑ Nofech-Mozes, S.; Hanna, W.. "Toker cells revisited.". Breast J 15 (4): 394-8. doi:10.1111/j.1524-4741.2009.00743.x. PMID 19601945.
  14. ↑ 14.0 14.1 Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1183. ISBN 978-1416031215.
  15. ↑ AP. May 2009.
  16. ↑ 16.0 16.1 Abenoza P, Lillemoe T (October 1993). "CD34 and factor XIIIa in the differential diagnosis of dermatofibroma and dermatofibrosarcoma protuberans". Am J Dermatopathol 15 (5): 429–34. PMID 7694515.
  17. ↑ 17.0 17.1 Goldblum JR, Tuthill RJ (April 1997). "CD34 and factor-XIIIa immunoreactivity in dermatofibrosarcoma protuberans and dermatofibroma". Am J Dermatopathol 19 (2): 147–53. PMID 9129699.
  18. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1249. ISBN 978-1416031215.
  19. ↑ URL: http://emedicine.medscape.com/article/1099540-overview. Accessed on: 24 August 2010.
  20. ↑ URL: http://emedicine.medscape.com/article/1099540-overview. Accessed on: 24 August 2010.
  21. ↑ URL: http://emedicine.medscape.com/article/1098342-overview. Accessed on: 24 August 2010.
  22. ↑ 22.0 22.1 Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 385. ISBN 978-1416002741.
  23. ↑ URL: http://www.mdconsult.com/das/book/body/199872830-2/0/1709/I4-u1.0-B978-0-443-06694-8..50117-2--f2.fig. Accessed on: 6 May 2010.
  24. ↑ Kumar, Vinay; Abbas, Abul K.; Fausto, Nelson; Aster, Jon (2009). Robbins and Cotran pathologic basis of disease (8th ed.). Elsevier Saunders. pp. 1185. ISBN 978-1416031215.
  25. ↑ Feng, H.; Shuda, M.; Chang, Y.; Moore, PS. (Feb 2008). "Clonal integration of a polyomavirus in human Merkel cell carcinoma.". Science 319 (5866): 1096-100. doi:10.1126/science.1152586. PMID 18202256.
  26. ↑ 26.0 26.1 Calder, KB.; Smoller, BR. (May 2010). "New insights into merkel cell carcinoma.". Adv Anat Pathol 17 (3): 155-61. doi:10.1097/PAP.0b013e3181d97836. PMID 20418670.
  27. ↑ Pulitzer, MP.; Amin, BD.; Busam, KJ. (May 2009). "Merkel cell carcinoma: review.". Adv Anat Pathol 16 (3): 135-44. doi:10.1097/PAP.0b013e3181a12f5a. PMID 19395876.
  28. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 491. ISBN 978-0781765275.
  29. ↑ 29.0 29.1 URL: http://www.ispub.com/journal/the_internet_journal_of_otorhinolaryngology/volume_9_number_2_11/article/sebaceous_adenocarcinoma_of_the_parotid_gland_a_case_report_and_literature_review.html. Accessed on: 8 September 2011.
  30. ↑ URL: http://emedicine.medscape.com/article/1056204-overview. Accessed on 2 September 2011.
  31. ↑ Withers, AH.; Brougham, ND.; Barber, RM.; Tan, ST. (Jun 2011). "Atypical fibroxanthoma and malignant fibrous histiocytoma.". J Plast Reconstr Aesthet Surg. doi:10.1016/j.bjps.2011.05.004. PMID 21664889.
  32. ↑ Beer, TW.; Drury, P.; Heenan, PJ. (Aug 2010). "Atypical fibroxanthoma: a histological and immunohistochemical review of 171 cases.". Am J Dermatopathol 32 (6): 533-40. doi:10.1097/DAD.0b013e3181c80b97. PMID 20526171.
  33. ↑ 33.0 33.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 521. ISBN 978-0443066542.
  34. ↑ Vandergriff, TW.; Reed, JA.; Orengo, IF. (2008). "An unusual presentation of atypical fibroxanthoma.". Dermatol Online J 14 (1): 6. PMID 18319023.
  35. ↑ URL: http://emedicine.medscape.com/article/1059940-overview. Accessed on: 2 September 2011.
  36. ↑ Baykal, C.; Buyukbabani, N.; Yazganoglu, KD.; Saglik, E. (Jan 2006). "[Tumors associated with nevus sebaceous].". J Dtsch Dermatol Ges 4 (1): 28-31. doi:10.1111/j.1610-0387.2006.05855.x. PMID 16503928.
  37. ↑ 37.0 37.1 Busam, Klaus J. (2009). Dermatopathology: A Volume in the Foundations in Diagnostic Pathology Series (1st ed.). Saunders. pp. 386. ISBN 978-0443066542.
  38. ↑ 38.0 38.1 URL: http://ccr.cancer.gov/staff/gallery.asp?profileid=12822. Accessed on: 2 September 2011.
  39. ↑ URL: http://dermatlas.med.jhmi.edu/derm/indexDisplay.cfm?ImageID=667496720. Accessed on: 2 September 2011.
  40. ↑ URL: http://www.pathconsultddx.com/pathCon/diagnosis?pii=S1559-8675(06)70190-5. Accessed on: 2 July 2010.
  41. ↑ Salhi, A.; Bornholdt, D.; Oeffner, F.; Malik, S.; Heid, E.; Happle, R.; Grzeschik, KH. (Aug 2004). "Multiple familial trichoepithelioma caused by mutations in the cylindromatosis tumor suppressor gene.". Cancer Res 64 (15): 5113-7. doi:10.1158/0008-5472.CAN-04-0307. PMID 15289313.
  42. ↑ URL: http://emedicine.medscape.com/article/1060049-workup#a0723. Accessed on: 31 August 2011.
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