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308 CHAPTER 6 Urological neoplasia

Testicular cancer: management of seminoma, IGCN, and lymphoma

Of all seminomas, 75% are confined to the testis at presentation and are cured by radical orchiectomy; 10–15% of patients harbor regional node metastasis, and 5–10% have more advanced disease.

Treatment and follow-up depend largely on disease stage similar to the stratification of NSGCT (p. 304). RPLND is not used for seminoma. Unlike NSCGT, radiation therapy is a therapeutic modality in seminoma.

Subdiaphragmatic radiation is the traditional method: 35 Gy to the paraaortic and ipsilateral iliac lymph nodes following radical orchiectomy using a “hockey stick” template. Newer radiation techniques limit radiation to the paraortic region with contralateral testicular shielding.

Generally, radiation doses in the adjuvant setting are in the range of 25 Gy, with 35 Gy used for more bulky disease.

Prophylactic mediastinal radiation therapy has been abandoned (cardiovascular side effects, failure to significantly improve outcomes, and interference with ability to administer salvage chemotherapy).

Stage I

Radical orchiectomy and adjuvant radiation therapy in low-stage disease is the most common treatment.

Close surveillance with imaging and serial tumor markers instead of radiation therapy for low-stage seminoma is gaining acceptance.

Stage IIA

Non-bulky retroperitoneal nodes: RT 35 Gy or chemotherapy

Stage IIB/IIC

Bulky lymphadenopathy (>5–7 cm nodes) or visceral metastasis with standard NSCGT: platinum-based chemotherapy (BEP/EP) (see p. 305)

Stage III

Chemotherapy; with brain metastasis, brain radiation therapy +/ excision

Cure rate

All stages have at least a 90% cure rate:

Stage I is 98–100%.

Stage II (B1/B2 non-bulky) is 98–100%.

Stage II (B3 bulky) and stage III have a >90% complete response to chemotherapy and an 86% durable response rate to chemotherapy.

Management of intratubular germ cell neoplasia (IGCN)

Observation or orchiectomy for unilateral disease.

Radiotherapy for unilateral disease in the presence of a contralateral tumor

Radiotherapy for bilateral disease, to preserve Sertoli cells

Frozen sperm storage must be offered.

TESTICULAR CANCER 309

Management of testicular lymphoma

This may be a primary disease or a manifestation of disseminated nodal lymphoma. The median age of incidence is 60 years, but has been reported in children.

One-quarter of patients present with systemic symptoms; 10% have bilateral testicular tumors. These patients have a poorer prognosis following radical orchiectomy and chemotherapy, while those with localized disease may enjoy long-term survival.

310 CHAPTER 6 Urological neoplasia

Penile neoplasia: benign, viral-related, and premalignant lesions

Benign tumors and lesions (see Table 6.19)

Noncutaneous

Congenital and acquired inclusion cysts

Retention cysts

Syringomas (sweat gland tumors)

Neurilemoma

Angioma, lipoma

Iatrogenic pseudotumor following injections

Pyogenic granuloma following injections

Cutaneous

Pearly penile papules (normal in 15% of postpubertal males)

Zoon balanitis (shiny, erythematous plaque on glans or prepuce)

Lichen planus (flat-topped violaceous papule)

Viral-related lesions

Condyloma acuminatum

This is also known as genital warts, related to human papillomavirus (HPV) infection. There are soft, usually multiple benign lesions on the glans, prepuce, and shaft; they may occur elsewhere on genitalia or perineum.

A biopsy is worthwhile prior to topical treatment with podophyllin. 5% have urethral involvement, which may require diathermy.

HPV infection (particularly types 16 and 18) is potentially carcinogenic and condylomata have been associated with penile SCC (this is distinct from the premalignant giant condyloma acuminatum; see below).

Bowenoid papulosis

This condition resembles carcinoma in situ, but with a benign course. Multiple papules appear on the penile skin, or a flat glanular lesion. These should be biopsied. HPV is the suspected cause.

Kaposi sarcoma

This reticuloendothelial tumor has become the second-most common malignant penile tumor. It presents as a raised, painful, bleeding violaceous papule or as a bluish ulcer with local edema. It is slow growing, solitary, or diffuse. It occurs in immunocompromised men, particularly in gay men with HIV/AIDS. Urethral obstruction may occur.

Treatment is palliative, with intralesional chemotherapy, laser, cryoablation, or radiotherapy.

Premalignant cutaneous lesions

Some histologically benign lesions are recognized to have malignant potential or occur in close association with SCC of the penis. A chronic red or pale lesion on the glans or prepuce is a cause for concern. Note should be made of its color, size, and surface features. Early follow-up after use of steroid, antibacterial, or antifungal creams is recommended. If persistent, biopsy is advised.

 

 

 

PENILE NEOPLASIA

311

 

 

 

 

 

 

Table 6.19 Benign penile tumors and lesions

 

 

 

 

 

 

 

 

 

 

Disease (organism/

Characteristics

Therapy

 

 

 

cause)

 

 

 

 

 

Behçet disease

Syndrome of oral and

Symptomatic against

 

 

 

(autoimmune; ? genetic)

genital ulcers, and uveitis;

inflammation; If

 

 

 

 

lesions are painful; rule out

severe, use steroids,

 

 

 

 

STDs

immunosuppressants,

 

 

 

 

 

interferon

 

 

 

Chancroid (Haemophilus

Painful, purulent ulcer,

Azithromycin or

 

 

 

ducreyi)

single or multiple, tender

ceftriaxone as a single

 

 

 

 

suppurative inguinal nodes

dose

 

 

 

Erythema multiforme

Red target lesions 1–2 cm;

Discontinue cause,

 

 

 

(hypersensitivity to

with severe form (Steven-

local supportive care

 

 

 

chemicals, drugs, or

Johnson syndrome) blisters

 

 

 

 

infection common)

and epidermal necrolysis

 

 

 

 

Fixed drug eruption

Solitary, inflammatory

Stop medication

 

 

 

(immune reaction to

and occasionally bullous;

(antibiotics are most

 

 

 

medication)

recurrent same site with

common cause)

 

 

 

 

exposure to medication;

 

 

 

 

 

post-inflammatory

 

 

 

 

 

hyperpigmentation common

 

 

 

 

 

 

 

 

 

Genital herpes (herpes

Multiple painful vesicles

Acyclovir, valacyclovir,

 

 

 

simplex virus types 1

with tender nodes

famciclovir orally in

 

 

 

and 2)

 

acute, suppressive, or

 

 

 

 

 

prodromal therapy

 

 

 

Genital warts/

Non-tender wart-like

Surgical ablation,

 

 

 

condylomata acuminata

papillary lesions, sometimes

podophyllin

 

 

 

(HPV types 6 and 11)

friable, no adenopathy

 

 

 

 

Granuloma inguinale/

Painless, progressive beefy

Doxycycline or TMP-

 

 

 

donovanosis

red ulcers, infrequent

SMX

 

 

 

(Calymmatobacterium

adenopathy

 

 

 

 

granulomatis)

 

 

 

 

 

Lichen planus (? viral or

Whitish annular lesion on

Usually resolves

 

 

 

chemical)

the glans penis, often itchy

spontaneously, topical

 

 

 

 

papular rash in other parts

steroids if symptomatic

 

 

 

 

of body

 

 

 

 

Lichen sclerosis et

Well-circumscribed white

High-dose topical

 

 

 

atrophicus, penile lesion

patches on glans/prepuce,

steroids or circumcision

 

 

 

called balanitis xerotica

thin epidermis ulcer prone

 

 

 

 

obliterans or BXO (?

that scars and contract;

 

 

 

 

autoimmune, infectious,

secondary meatal stenosis

 

 

 

 

or genetic)

and phimosis; itching and

 

 

 

 

 

burning common

 

 

 

 

 

 

 

 

 

312

CHAPTER 6 Urological neoplasia

 

 

 

Table 6.19 Continued

 

 

 

 

 

 

 

 

Disease (organism/

Characteristics

Therapy

 

cause)

 

 

 

 

 

 

 

 

 

Lymphogranuloma

Small, painless vesicle or

Doxycycline;

 

venereum (Chlamydia

papule progresses to an

azithromycin or

 

trachomatis)

ulcer

erythromycin

 

 

Painful matted nodes, fistula

alternative

 

 

common

 

 

 

Molluscum contagiosum

Smooth, round, pearly

Surgical ablation

 

(A pox virus)

papules, 2–5 mm

 

 

 

Pearly penile papules

Usually uncircumcised,

Usually not clinically

 

(? viral)

painless 1–2 mm papules

important; ablation if

 

 

with variable color

bothersome

 

 

(translucent/white/yellow)

 

 

 

 

usually around corona an

 

 

 

 

dorsum of penis

 

 

 

Sclerosing lymphangitis

Asymptomatic

 

(Local trauma)

subcutaneous cordlike

 

 

swellings along the dorsal

 

 

shaft of the penis or around

 

 

the coronal sulcus; may be

 

 

edema of glans and coronal

 

 

sulcus.

 

Syphilis, primary

Painless, indurated, with a

 

(Treponema pallidum)

clean base, usually singular

 

 

lesion; tender inguinal

 

 

nodes

Traumatic in origin, selflimited; avoid vigorous sexual activity

Benzthiazide penicillin G (2.4 million units IM) single dose

Zoon balanitis (?

Found in uncircumcised

Circumcision curative

Mycobacterium

only, on prepuce or glans,

 

smegmatis)

usually painless, solitary

 

 

shiny lesion with reddish or

 

 

orange tint; can be to 2 cm,

 

 

occasionally erosive

 

 

 

 

Cutaneous horn: rare, solid skin overgrowth; extreme hyperkeratosis, the base may be malignant. Treatment is wide local excision.

Pseudoepitheliomatous micaceous and keratotic balanitis: unusual hyperkeratotic growths on the glans. They require excision, histological examination, and follow-up, as they may recur.

Balanitis xerotica obliterans (BXO): also known as lichen sclerosus et atrophicus, this is a common sclerosing condition of glans and prepuce. It occurs at all ages and most commonly presents as non-retractile foreskin (phimosis). The meatus and fossa navicularis may be affected, causing obstructed and spraying voiding. The histological diagnosis is usually made after circumcision, with epithelial atrophy, loss of rete pegs, and collagenization of the dermis. BXO occurs in association

PENILE NEOPLASIA 313

with penile SCC, but most pathologists would regard the lesion as benign unless epithelial dysplasia is present.

Leukoplakia: solitary or multiple whitish glanular plaques that usually involve the meatus. Treatment is excision and histology. Leukoplakia is associated with in situ SCC; follow-up is required.

Erythroplasia of Queyrat: also known as carcinoma in situ of the glans, prepuce, or penile shaft. A red, velvety, circumscribed painless lesion occurs, though it may ulcerate, resulting in discharge and pain. Treatment is excision biopsy if possible; radiotherapy, laser ablation, or topical 5-fluorouracil may be required. Histology reveals hyperplastic mucosal cells with malignant features.

Bowen disease: this is carcinoma in situ of the remainder of the keratinizing genital or perineal skin. Treatment is wide local excision, laser, or cryoablation.

Buschke–Löwenstein tumor: also known as verrucous carcinoma or giant condyloma acuminatum, this is an aggressive locally invasive

tumor of the glans. Metastasis is rare, but wide excision is necessary to distinguish it from SCC. Urethral erosion and fistulation may occur.