64 y.o. F with CLL and leg tumour

Transcription

1 64 y.o. F with CLL and leg tumour

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6 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

7 SCC, Metatypical SCC, KA, adnexal?

8 One year later

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10 PAS Stain

11 Revised Diagnosis Eccrine porocarcinoma

12 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/m 2

13 January 2006

14 August 2006

15 December 2006

16 Management Summary Interferon-alpha Dec 2005-April MU sc 3 times a week Paclitaxel chemotherapy April 2006 weekly for 12/52 Capecitabine August g bd for 14/7 2 cycles over 6/52; PO (Fluoropyrimidine Tegafur) Thalidimide

17 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

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19 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)

20 Clinical presentation 6 th to 8 th 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

21 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

22 Histology Poromatous basaloid epithelial cells Ductal differentiation Cytological atypia Variety of patterns: Squamous differentiation Clear cell differentiation Mucus metaplasia Spindle cell differentiation

23 Prognosis Retrospective St Johns Study cases of EP 9 (17%) local recurrence 10 (19%) regional lymph nodes 6 (11%) distant metastases (4 deaths, 7%)

24 Prognosis Eccrine Porocarcinoma Regional lymph node metastasis confers 67% mortality rate

25 Histological parameters associated with aggressive disease >14 Mitoses per high power field Tumour depth >7mm Lymphovascular invasion Presence of an advancing infiltrative border

26 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

27 Previous reports of patients with metastatic EP (mep) treated with taxanes Author Plunkett et al, 2001 Gutermuth et al 2004 De Bree et al 2005 Clinical presentation 45 y.o. Renal transplant, history of Hodgkins lymphoma, mep to lung Failed epirubicin 67 y.o. mep to regional lymph nodes 69 y.o,mep to ribs Failed IFN-alpha and isotretinoin Rx Outcome Docetaxel Disease stability 3/12 100mg/m 2 post treatment,required 2nd course IFN-alpha 9Miu 3x-weekly, 5# weekly Paclitaxel 100mg/m 2 Daily top 5-FU and 3# intraarterial docetaxel 75mg/m 2, 2# systemic docetaxel 80mg/m 2 No evidence of disease progression after 7 months Disease stability after 25 months with complete histological response of skin lesions (anaphylaxis to systemic docetaxel after 3 rd # so treatment stopped)

28 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