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Neoplasms: Benign and Malignant

PILOMATRIX CARCINOMA (28914451)

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Background                                                                                                                                                                                                   ​​

  • Malignant variant of pilomatrixoma (also spelled pilomatricoma), but uncertain if it arises de novo or from malignant transformation. 

  • Alternate names: calcified epithelial carcinoma, matrical carcinoma, malignant matricoma.  

  • In one review of literature of 36 cases (including one pilomatrical carcinomsarcoma): mean age 57yo (range 8-85; bimodal distribution with >90% occurring in the 0-29yo and 50-69yo age groups), M:F 1.4:1, 2/3rds in caucasian.  Of note, NO patients in this cohort had an associated genetic condition. 28914451 

Natural history                                                                                                                                                                                               

  • Tendency for local recurrence and need for multiple excisions to attain clear margins (6 of 35 cases requiring 2 or more excisions). 28914451

  • In this cohort, 31% of patients had a recurrence of some type at an average of 6.5mo post-op.  3 of 35 patients presented with metastatic disease, and 11 ultimately ended up with locoregional or metastatic recurrences.  Sites included local lymph nodes, vertebrae, lungs, and intracranial involvement. 28914451

Diagnosis and Treatment                                                                                                                                                                              

Pathology

  • Ki-67, CK14, beta-catenin, cyclin D1, and p53 have been used in conjunction with routine histopathology

Excision​

  • No gold standard for margins, with 4mm up to 3cm being reported.

Imaging​

  • Over half of patients in this cohort received pre or post-op imaging with contrast CT or MRI.  28914451

Adjuvant Radiation

  • Typically reserved for recurrent metastatic disease, locoregional disease, or when clear margins cannot be obtained.  

  • External beam radiation therapy or combined EBRT/high-dose-rate brachytherapy 

Other Literature                                                                                                                                                                                             

  • A more recent review of the literature demonstrated similar findings to the above. 36826070

EXTRAMAMMARY PAGET'S DISEASE (30497678)

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Background                                                                                                                                                                                                   ​​

  • EMPD is a pagetoid lesion (histologically) that typically affects sites with apocrine glands (groin, vulva, scrotum, perianal, penis, axilla, umbilicus, eyelid, external auditory canal) and is thought to be a malignancy of apocrine duct origin, or, when involving the vulva, derived from Toker cells.  Broadly speaking there is primary EMPD, which is EMPD arising intraepidermally and unrelated to another malignancy; and, there is secondary EMPD, the derivation of which is not clearly understood. You can consider secondary EMPD as having 2 subtypes: secondary to adjacent malignancy (an extension of an underlying adjacent visceral malignancy or adjacent cutaneous apocrine malignancy), or, secondary to a distant malignancy in which case it may be considered either a paraneoplastic-like phenomenon or a distant cutaneous metastasis.  Mammary Paget's disease involves the breast and is always associated with an underlying breast malignancy.  More broadly speaking, some (Andrews') divide EMPD into Four types:

    • Primary EMPD - arises intraepidermally and can be invasive or in situ​

    • Secondary EMPD, a/w [cutaneous] apocrine carcinoma

    • Secondary EMPD, a/w adjacent [visceral] malignancy

    • Secondary EMPD, a/w distant carcinoma

  • Morphology is identical regardless of subtype.  ​

Natural history                                                                                                                                                                                               

  • Usually unifocal lesion but can present as multifocal lesions in different anatomic locations.  

  • Association with other cancers is mixed, with reports ranging from a  minority of patients with EMPD to nearly half or more.   

  • Invasion portends poor prognosis.  

Diagnosis and Treatment                                                                                                                                                                              

Pathology

  • Primary EMPD - CK7+/CK20-/GCDFP15+​

  • Secondary EMPD - CK7+/CK20+/GCDFP15-

  • Vulvar and perianal EMPD:

    • Type 1 (endodermal pattern, a/w distant cancers) - CK7+/CK20+/GCDFP15-​

    • Type 2 (ectodermal pattern, a/w cutaneous origin) - CK7+/CK20-/GCDFP15+

    • Bladder Ca associated - uroplakin and p63 positive

    • Rectal Ca associated - CK7-/CK20+/CDX2+

    • Prostate Ca associated - PSA or P504S positive

  • Invasion of 1mm or more may portend poorer prognosis.  ​

Labs

  • Serum RCAS1 levels can be monitored in patients with invasive disease similar to how PSA is used for prostate cancer. 

Excision​

  • Mohs, with significantly better cure rates if CK7 IHC is used; excision with 2-5cm margins.

SLNBx

  • indicated when invasion is present

Non-surgical treatment modalities

  • Radiation treatment with a variety of dosing regimens have been utilized both as primary and adjuvant treatment with varying success.

  • Imiquimod has been utilized with varying response rates and long term maintenance therapy may be needed. 

  • 5-FU has weaker evidence than imiquimod.

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Other Literature                                                                                                                                                                                             

Herrel LA, Weiss AD, Goodman M, Johnson TV, Osunkoya AO, Delman KA, Master VA. Extramammary Paget's disease in males: survival outcomes in 495 patients. Ann Surg Oncol. 2015. PMID: 25384700.​​​

- Case serious of 495 male patients with good descriptive statistics.

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