Jamie Groh PGY-6

PBCGME/West Palm Hospital

Objectives

Clinical Presentation

Histology

Current Treatments

Future Treatment

Case 72 yo Caucasian male

Subcutaneous nodule right posterior arm ~1year

PCP told patient it was likely a lipoma

Lesion began to grow and patient requested further evaluation

Patient was referred to dermatology

Right posterior arm 11/1/13. 1.2cm x 2.5 cm subcutaneous, mobile, skin colored nodule fixed to overlying epidermis

Case Patient presented to dermatology 5 months after

initial referral

Lesion had continued to grow rapidly

Two weeks prior to visit patient noted swelling in the right axilla.

ROS: Intentional 40 pound weight loss last several month. All other ROS non-contributory.

Case PMHx: DMII

PSHx: non-contributory

Fam Hx: Father deceased from pancreatic ca

Social hx:

Tobacco: quit 50 years ago

ETOH: hx of social use, currently none

Illicit drugs: denies

Case Physical Exam

Gen: A&O x 3 NAD

Right posterior arm: 6 cm erythematous moist, mobile, subcutaneous nodule, fixed to overlying epidermis.

Right axilla LAD

Right posterior arm 4/11/15.

Right posterior arm 4/11/15

Case-work up 5mm lesional punch biopsy for H&E

CT Chest and Right upper extremity with and without contrast.

Case CT- subcutaneous mass of posterior right arm with

evidence of necrosis. Right axillary LAD, largest lymph node 3 cm

Histology- Poorly differentiated neuroendocrinecarcinoma

Immunohistochemistry-

+CK 20 perinuclear dot, synaptophysin

Negative TTF-1 and CK7

Diagnosis: Merkel Cell Carcinoma

Right posterior arm. H&E 2x magnification

H&E 20x magnification

H&E 40x magnification

CK 20 Perinuclear Dot

Synaptophysin

Case General Surgery consult

WLE of Right posterior arm MCC and axillary lymph node dissection

Despite WLE margins were still positive

21 of 29 lymph nodes positive for MCC

Pathologic preliminary staging was T3N1

Patient was referred to oncology

H&E Right Axillary Lymph Node

Case PET scan was ordered by oncology

Metabolic areas in right distal arm, right supraclavicularlymph nodes, right axillary lymph nodes and b/l hilarlymph

Due to extent of disease radiation was not an option

Oncology recommended chemotherapy with platinum and etopiside.

Patient chose palliative care.

MCC Epidemiology MCC is an aggressive neuroendocrine tumor

High rate of recurrence and metastasis

Incidence is increasing faster than any other skin cancer

SEER program data

1986-2001 annual incidence tripled

Approximately 1600 new cases/year

Most commonly affects elderly Caucasian males

MCC Risk Factors Age >65

Increased sun exposure

Immunosuppression

Immune dysregulation due to T cell dysfunction

30x increase CLL

13x increase AIDS

10x increase in organ transplant

Infection with MCPyV

MCC Clinical Presentation Painless, solitary, rapidly growing nodule/indurated

plaque, arising on chronically sun exposed skin

Most common location face and neck followed by upper limb

Acronym AEIOU for clinical parameters Asymptomatic

Expanding Rapidly

Immune Suppressed

Older than 50 years old

UV exposed areas

MCC Histology Poorly defined dermal tumor

Strands and nests of monotonous uniform, small, oval cells

Cells 2-3 x larger than lymphs

Abundant mitotic figures

Immunohistochemical stain CK 20 perinuclear dot

EMA, NSE, chromogranin, synaptophysin, calcitonin, VIP

Negative TTF-1

MCPyV

P63- aggressive behavior

MCC Work Up 4mm punch biopsy for H&E

Clinical nodal exam

SLNB for all

PET

No consensus on CT chest to r/o small cell lung ca.

Treatment Primary

WLE with SLNB

Consider local radiation

Regional lymph node involvement

Lymph node dissection +/- lymph node basin radiation

Metastatic

Palliative chemotherapy

Cisplatin, doxorubicin, cyclophosphomide, vindesine, epirubicin,etoposide

Possible Future Treatment Targeted Molecular therapy

Ipilimumab

YM-155

Pazopanib

Lanetreotide

ILGFR-1 and mTOR inhibitor

Oblimersen

Possible Future Therapies Immunotherapy

Recombinant IL-2

Intra-lesional interferon

PD-1 inhibitor

IL-12 DNA Electroporation

Bortezomib

T-cell immunotransfer

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