Case Discussion

Miao Zhang, MD, PhD

Assistant Professor, Department of Pathology,

The University of Texas MD Anderson Cancer Center

Houston, TX

Clinical History

• 64 yo African American male presented at 2005 with perineal pain. Initial MRI of the pelvis was normal.

• 2011, patient started to experience firmness at the base of the penis.

• 2013, a 4.1 x 2.6 x 1.6 cm lesion was seen on MRI.

MRI At MDACC

Axial T2 weighted Coronal T2 weighted Sagittal T2 weighted

Urethral Mass: thick short arrows Urethra: long thin arrowProstate Gland: asterisk Rectum: arrowhead

Cytokeratin 7

p63

CD117

Biopsy Diagnosis

Basaloid carcinoma with salivary gland-like features

in fibromuscular tissue

Case# Carpenter et al., Small et al., Hisamatsu et al., Trnski et al., Myers et al.,

Age (yo) 57 66 52 60 N/A

Surgical

Procedure

Excision Pelvic

exenteration

N/A Excision Pelvic

exenteration

Size (cm) 6 cm 3cm N/A N/A N/A

LVI N/A N/A N/A N/A N/A

LN

Involvement

N/A N/A N/A N/A N/A

Other

Treatment

Radiation Radiation Chemotherapy None Radiation,

Chemotherapy

targeted

therapy

Follow up 5y N/A 5y 6 month 7y

Outcome NED N/A NED NED AWD

Complex Surgical Resection

Urethrectomy

Resection of pubic symphysis

Prostatectomy

Total penectomy

Anus and Rectum resection

PenileUrethra

GlansPenis

Anus

Tumor

Rectum

Urethral lumen

Tumor under pubic arch

Pubic Bone

UrethraProstate gland

Prostate gland

Tumor

Rectum

PSA

Final diagnosis• Adenoid cystic carcinoma (3.7 cm), involving:

– membranous urethra, prostatic urethra, penile urethra, prostate gland, penile shaft (corpus spongiosum and corpora cavernosa), urogenital diaphragm, perianal soft tissue and muscular propria of rectum.

• Adenocystic carcinoma abutting soft tissue margin and prostate base margin.

• Prostatic adenocarcinoma, Gleason 9 ( 4+5 ), with 1/15 lymph node metastasis.

• Post-op radiation to the resection bed.

18 month later, enlarging lung nodule

Follow up

• CMS 400

• Clinical trials

• Now on Nivolumab—Anti-PD-1.

• PSA stable

Case# Current case Carpenter et al., Small et al., Hisamatsu et

al.,

Trnski et

al.,

Myers et al.,

Age 73 57 66 52 60 N/A

Surgical

Procedure

Total penectomy,

urethrectomy,

resection of pubic

symphysis,

prostate, anus and

rectum.

Excision Pelvic

exenteration

N/A Excision Pelvic

exenteration

Size (cm) 3.7cm 6 cm 3cm N/A N/A N/A

LVI Not identified N/A N/A N/A N/A N/A

LN

Involvement

No N/A N/A N/A N/A N/A

Other

Treatment

Radiation Radiation Radiation Chemotherapy None Radiation,

Chemotherapy

and targeted

therapy

Follow up 40 month 5y N/A 5y 6 month 7y

Outcome AWD NED N/A NED NED AWD

Summary• ACCs of the urethra/Cowper’s gland are rare

• Little is known about their prognosis and natural history

• Published cases suggest a slow growing malignancy

• Potential for long term survival, if aggressive therapeutic options are utilized

Thank You

Questions?

Follow-Up (See Notes)• Lung nodule (?months after surgery; ?when biopsy done• CMS 400• Clinical trials• ?No on Nivolimab—Anti-PD-1. • PSA stable

Follow up

• CMS 400

• Clinical trials

• No on Nivolimab—Anti-PD-1.

• PSA stable

Case# Carpenter et al., Small et al., Hisamatsu et

al.,

Trnski et

al.,

Myers et al.,

Age (yo) 57 66 52 60 N/A

Surgical

Procedure

Excision Pelvic

exenteration

N/A Excision Pelvic

exenteration

Size (cm) 6 cm 3cm N/A N/A N/A

LVI N/A N/A N/A N/A N/A

LN

Involvement

N/A N/A N/A N/A N/A

Other

Treatment

Radiation Radiation Chemotherapy None Radiation,

Chemotherapy,

targeted therapy

Follow up 5y N/A 5y 6 month 7y

Outcome NED N/A NED NED AWD

• A firm area of induration over the right side of the pelvic floor, distal to the prostate, but also indistinguishable from the right side of the apex of the prostate.

• Biopsy

Rectal exam