Discussion - Virtual Pathology at the University of Leeds

64 y.o. F with CLL and leg tumour
Case History
• Excision with split-skin grafting
Histology moderately differentiated squamous cell
carcinoma with large areas of necrosis and brisk mitotic
activity.
Second episode of red cell aplasia- CLL-immuosupressed
Blood transfusion
Rituximab
SCC , Metatypical SCC, KA, adnexal?
One year later
PAS Stain
Revised Diagnosis
Eccrine porocarcinoma
Progress
• Dec 2005 Amputation declined
• Interferon alpha 5 million units three-times
weekly
• April 06 New inguinal lymphadenopathy
CT appearance in keeping with metastatic
disease
Commenced weekly Paclitaxel 70mg/m2
January 2006
August 2006
December 2006
Management Summary
• Interferon-alpha
Dec 2005-April 2006
– 5MU sc 3 times a week
• Paclitaxel chemotherapy April 2006
– weekly for 12/52
• Capecitabine
August 2006
– 2g bd for 14/7 2 cycles over 6/52; PO
– (Fluoropyrimidine Tegafur)
• Thalidimide
Eccrine porocarcinoma (EP)
• Rare tumour derived from the acrosyringium
of the eccrine gland
• First described in 1963 by Mehregan and
Pinkus
• 200 cases in the literature
• Most prevalent malignant eccrine sweat gland
tumour
• Many display more complex mixed
differentiated features defying classification
– F>M
Eccrine Porocarcinoma
– Age 73 years (29-91years)
– Site
• Lower Extremity (44%)
• Trunk (24%)
• Head (18%)
– Clinical Appearance
• Variable
– Histologic Pattern
• Wide variation →Diagnostic Error
– Prognosis
• Mitosis (14mitosis/high power field)
• Lymphovascular invasion
• Tumour Depth (>7mm)
Clinical presentation
•
•
•
•
•
•
•
6th to 8th decade
Equal sex preponderance
Lower limb>>trunk>head&neck>upper limb
Soliatry enlarging nodule
Variable appearance
Diagnosis rarely suspected clinically
18%-30% arise within benign eccrine poroma
ECCRINE POROCARCINOMA:
HISTOLOGY
• Cords and lobules of polygonal cell
tumor in the dermis, some of which
have squamoid features and central
necrosis
• Overt nuclear atypia with nucleoli
• Permeative peripheral growth
• Intraepidermal tumour cells in
"lakes," often centered on
acrosyringial pores
Histology
• Poromatous basaloid epithelial cells
• Ductal differentiation
• Cytological atypia
Variety of patterns:
• Squamous differentiation
• Clear cell differentiation
• Mucus metaplasia
• Spindle cell differentiation
Prognosis
• Retrospective St Johns Study 2001
• 54 cases of EP
9 (17%) local recurrence
10 (19%) regional lymph nodes
6 (11%) distant metastases (4 deaths, 7%)
Prognosis Eccrine Porocarcinoma
Regional lymph node metastasis confers 67% mortality rate
Histological parameters associated with
aggressive disease
• >14 Mitoses per high power field
• Tumour depth >7mm
• Lymphovascular invasion
• Presence of an advancing infiltrative border
Treatment-metastatic disease
• Radiotherapy not effective
• Chemotherapy
Tamoxifen
Isotretinoin
IFN-alpha
Docetaxel
5-FU
Paclitaxel
5-FU/Cisplatin/Radiotherapy
Isotretinoin/IFN-alpha
Docetaxel +topical 5-FU
IFN-alpha +IL-2
Previous reports of patients with metastatic EP (mEP) treated
with taxanes
Author
Clinical
presentation
Rx
Outcome
Plunkett et
al, 2001
45 y.o. Renal
Docetaxel
transplant, history of 100mg/m2
Hodgkins
lymphoma, mEP to
lung
Failed epirubicin
Gutermuth
et al 2004
67 y.o. mEP to
regional lymph
nodes
IFN-alpha 9Miu
No evidence of disease
3x-weekly, 5#
progression after 7
weekly Paclitaxel months
100mg/m2
De Bree et
al 2005
69 y.o,mEP to ribs
Failed IFN-alpha
and isotretinoin
Daily top 5-FU
and 3# intraarterial docetaxel
75mg/m2, 2#
systemic
docetaxel
80mg/m2
Disease stability 3/12
post treatment,required
2nd course
Disease stability after
25 months with
complete histological
response of skin
lesions (anaphylaxis to
systemic docetaxel
after 3rd# so treatment
stopped)
Pathogenesis
• Poorly understood-polyoma virus co-carcinogen?
• Role of immunosuppression?
• C Harwood et al, 2003
Immunosuppressed renal transplant population at
greatly increased risk of appendageal tumour
compared with immunocompetent population
Greater proportion of these were malignant