Lydia T. Laboccetta, MD
James S. Rosoff, MD
BASICS
DESCRIPTION
• Squamous cell carcinoma (SCC) of the scrotum is a very rare, environmentally induced cancer with high metastatic potential
• The first reported occupational cancer
– Initially described in 1775 by Sir Percivall Pott in chimney sweeps
• Synonyms: Chimney sweeps cancer, Mule-spinners’ disease, Pott cancer
EPIDEMIOLOGY
Incidence
• 1.5–3/10 million men in US(1); similar rates noted in Dutch series (2)
• Increasing incidence in US may be related to increases in HPV infection, Psoralen plus ultraviolet light A (PUVA) treatment for psoriasis, and also due to improved reporting
• Lower incidence among black men in older series, though increased risk in black men in more recent SEER database analysis (1)
• Most reported in men >50 yr; median age in SEER series: 68
Prevalence
N/A
RISK FACTORS
• Chemical/mechanical irritation
• Classically described in chimney sweeps: Related to soot or chemical exposure, poor hygiene
• Oil/petroleum: Machine workers (lathe workers, mule-spinners [men and boys who worked on cotton spinning machines])
• Poor hygiene
• Repeated trauma
• Rarely in patients with prior scrotal incision/scar
• PUVA: Ultraviolet A radiation for psoriasis. Effect is dose dependent.
• Associated with HPV 16, 18 in limited case reports
• Chronic immunosuppression
Genetics
N/A
PATHOPHYSIOLOGY
• SCC is a malignant tumor of epidermal keratinocytes thought to occur mainly from chronic irritation from a mechanical or chemical source.
• 3,4-benzpyrene, a polycyclic aromatic hydrocarbon, is a common occupational carcinogen in these men
• Associations with many other carcinogens have been described
ASSOCIATED CONDITIONS
• Psoriasis
• HPV/condylomata
• Sexually transmitted infections
GENERAL PREVENTION
• Decrease exposure to carcinogenic agents:
– Protective clothing for those with occupational risk
– Prevention of HPV transmission
– Shielding of area during PUVA treatment
• Improve hygiene
DIAGNOSIS
HISTORY
• Occupational exposure to chemical and/or mechanical irritants
• History of treatment for psoriasis
• History of HPV, HIV
• History of scrotal trauma, scrotal surgery
• Inflammatory conditions involving the scrotum
• Change in size of lesion or ulceration
• Fever
• Nonhealing nodule or ulcer
PHYSICAL EXAM
• Exam of external genitalia, inguinal and distant lymph nodes:
– Usually a solitary, slow-growing nodule with or without ulceration usually on the anterolateral aspect of the scrotum
– Starts as a small pimple or nodule which gradually develops ulceration, raised or rolled edges, purulent discharge
– Lesion may persist for 6 mo before ulcerating
– May have associated condylomata of penis, scrotum, perianal region
• May lead to lymphadenopathy due to malignancy or infection
– 30–60% have palpable lymphadenopathy at presentation
– 25% have inguinal metastases at presentation
DIAGNOSTIC TESTS & INTERPRETATION
Lab
• WBC count to rule out acute infection
• Urinalysis and urine culture if indicated
Imaging
• CT may help to assess size, extent of lymphadenopathy but cannot differentiate inflammation vs. malignancy
• MRI
– Improved accuracy in diagnosis and staging
– Can assess infiltrative vs. inflammatory process
• Lymphangiography is accurate in delineating metastatic vs. inflammatory nodes, but cannot detect micrometastases
Diagnostic Procedures/Surgery
Excisional biopsy of primary lesion
Pathologic Findings
• SCC:
– Most are well or moderately differentiated and contain focal areas of keratosis
– Surrounding epidermis demonstrates hyperkeratosis, acanthosis, dyskeratosis
– Diffuse lymphocytic infiltrate may be present
• Staging (staging for all scrotal carcinoma, not only SCC, no TNM classification exists) (3)
– Stage A1: Localized to scrotal wall
– Stage A2: Locally invasive involving adjacent structures (testis, spermatic cord, penis, pubis, and perineum)
– Stage B: Metastatic disease to the inguinal lymph nodes only
– Stage C: Metastatic disease to the pelvic lymph nodes without evidence of distant spread
– Stage D: Metastatic disease beyond the pelvis involving distant organs
– In SEER series, 76% presented with localized disease, 20% with regional metastases, and 4% with distant metastases (4)
DIFFERENTIAL DIAGNOSIS
• Benign scrotal lesions:
– Condyloma
– Eczema
– Hidradenitis suppurativa
– Folliculitis
– Nevus
– Periurethral abscess
– Psoriasis
– Sebaceous cysts/epidermal inclusion cyst
– Syphilis
– Tuberculous epididymitis with a draining sinus
• Malignant scrotal lesions:
– Basal cell carcinoma
– Malignant melanoma
– Paget disease
– Marjolin ulcer: Cancer arising from site of prior inflammation
– Kaposi sarcoma: A purple, papular, plaque-like, or ulcerated lesion on the penis or scrotum
– Sarcoma: Leiomyosarcoma from the Dartos layer of the scrotum is most common, though still very rare
– Metastatic lesion
TREATMENT
GENERAL MEASURES
• Management is primarily surgical.
• Local wide excision is diagnostic and therapeutic.
MEDICATION
First Line
• Broad-spectrum antibiotics for 4–6 wk in patients with lymphadenopathy
• Chemotherapy has not demonstrated success for primary treatment (single agent or combination therapy)
– Methotrexate, bleomycin, and cisplatin have been used with radiotherapy in 1 case report, but the patient later required surgical resection
– Bleomycin has been reported as successful in 2 cases
– Multiple case reports exist in the literature of combined adjuvant chemotherapy and radiotherapy
• Topical 5-FU has not been successful in treating carcinoma in situ of the scrotum
Second Line
N/A
SURGERY/OTHER PROCEDURES
• Primary lesion:
– Wide local excision of lesion with a 2-cm margin of skin and dartos fascia.
– Small lesions may be closed primarily.
– Large lesions may require split-thickness skin grafting or local flaps.
– If hemiscrotectomy is performed, the ipsilateral testis may be placed in a thigh pouch or moved to the contralateral hemiscrotum.
– Excision of all scrotal contents is required only when structures are directly involved by tumor.
• Regional lymph nodes
– If palpable adenopathy resolves after antibiotics or was never present, then a superficial inguinal lymph node biopsy should be performed:
Ipsilaterally if lesion is lateral.
Bilaterally if lesion at the median raphe
– If palpable lymphadenopathy persists after antibiotics, then a bilateral superficial lymph node biopsy should be performed.
– Full ilioinguinal lymphadenectomy should be performed only on the side of the positive biopsy:
If performing a unilateral ilioinguinal lymphadenectomy, a contralateral superficial inguinal lymph node biopsy should also be performed.
If there is a positive frozen section, then perform a bilateral ilioinguinal lymphadenectomy.
• Laser vaporization of the primary lesion has been used in poor surgical candidates or those who refused surgery.
• Mohs micrographic surgery has also been used for primary lesions.
ADDITIONAL TREATMENT
Radiation Therapy
• Has not been effective and is reserved for recurrences and poor surgical candidates.
• Has been described in multiple case reports as adjuvant therapy.
Additional Therapies
N/A
Complementary & Alternative Therapies
N/A
ONGOING CARE
PROGNOSIS
• Scrotal cancer survival is worse for SCC histology than all other histologies except melanoma (4)
• Survival at 5 yr (1):
– Stage A: 70–80%
– Stage B: 40–50%
– Stage C: Rare
– Stage D: Rare
• Local recurrence rates:
– 21–40%
– May require additional excision
– Patients with industrial exposure may be at higher risk for recurrence
COMPLICATIONS
• Femoral hernias after ilioinguinal lymphadenectomy
• Lymphedema
• Lymphoceles
• Wound infections
FOLLOW-UP
Patient Monitoring
• Patient Monitoring
– Self-exams for local recurrence of lesion or lymphadenopathy
– Periodic follow-up by physician for monitoring of local recurrence or lymphadenopathy
• Follow-up is required for life
Patient Resources
N/A
REFERENCES
1. Wright JL, Morgan TM, Lin DW. Primary scrotal cancer: Disease characteristics and increasing incidence. Urology. 2008;72(5):1139–1143.
2. Verhoeven RH, Louwman WJ, Koldewijn EL, et al. Scrotal cancer: Incidence, survival and second primary tumours in the Netherlands since 1989. Br J Cancer. 2010;103(9):1462–1466.
3. Ray B, Whitmore WF Jr. Experience with carcinoma of the scrotum. J Urol. 1977;117(6):741–745.
4. Johnson TV, Hsiao W, Delman KA, et al. Scrotal cancer survival is influenced by histology: A SEER study. World J Urol. 2013;31(3):585–590.
ADDITIONAL READING
N/A
See Also (Topic, Algorithm, Media)
• Scrotum and Testicle Mass
• Scrotum, Epidermal Inclusion Cyst
• Scrotum, Hemangioma
• Scrotum, Idiopathic Calcinosis
• Scrotum, Tumors, Benign and Malignant
• Seborrheic Dermatitis
• Skin Tags, External Genitalia (Acrochordon, Pedunculated Papilloma)
CODES
ICD9
187.7 Malignant neoplasm of scrotum
ICD10
C63.2 Malignant neoplasm of scrotum
CLINICAL/SURGICAL PEARLS
A nonhealing ulcer or nodule on the scrotum should raise suspicion for squamous cell carcinoma.