8720/3 Melanoma, NOS


Related terms

Malignant melanoma, NOS

Definitions

Skin
ICD-O-3 topography code: C44
ICD10: C43
           

Malignant melanoma is a malignant, usually aggressive tumour histologically composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. They develop most frequently on the face; other commonly affected sites include ears, head, neck, back and shoulders in male patients and the lower limbs in females. Most lesions show an initial radial growth phase within the epidermis and superficial dermis, followed by a vertical growth phase which results in the formation of papules or nodules and eventually involves the subcutaneous tissue1
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.
Approximately 80% of melanoma patients are light-skinned Caucasians. The most important environmental risk factor is intermittent exposure to UV radiation, especially during childhood. Familial/genetic risk factors include skin type, number of nevi, the presence of dysplastic nevi, and a family history of melanoma. The single most important prognostic factor is vertical tumour thickness; lesions that have entered the vertical growth phase have metastatic potential and a poor prognosis. Usually, melanomas first metastasize to the regional lymph nodes. Distant metastases arise preferentially in skin, lungs, liver, central nervous system and bones, but any organ may be affected. Survival has increased substantially over the last three decades, mainly due to early detection
2
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.

Four major subtypes of malignant melanoma are distinguished:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Tumours of the central nervous system
ICD-O-3 topography code: C70, C71 and C72
ICD10: C70
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Esophagus
ICD-O-3 topography code: C15
ICD10: C15
           

Melanoma is a malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Cases of melanoma of the oesophagus typically arise in the middle or distal oesophagus of older adults
3
Click to access Pubmed
Li B, Lei W, Shao K, Zhang C, Chen Z, Shi S, He J (2007)
Characteristics and prognosis of primary malignant melanoma of the esophagus.
Melanoma Res 17: 239-42



4
Click to access Pubmed
Sanchez AA, Wu TT, Prieto VG, Rashid A, Hamilton SR, Wang H (2008)
Comparison of primary and metastatic malignant melanoma of the esophagus: clinicopathologic review of 10 cases.
Arch Pathol Lab Med 132: 1623-9



. The most common clinical complaint is dysphagia. At endoscopy, the neoplasms are polypoid and melanin pigmentation is seen in almost all cases 5
Click to access Pubmed
Fredricks JR, Bejarano PA (2008)
Primary malignant melanoma of the esophagus with separate foci of melanoma in situ and atypical melanocytic hyperplasia in a patient positive for human immunodeficiency virus: a case report and review of the literature.
Arch Pathol Lab Med 132: 1675-8



6
Click to access Pubmed
Sanchez AA, Wu TT, Prieto VG, Rashid A, Hamilton SR, Wang H (2008)
Comparison of primary and metastatic malignant melanoma of the esophagus: clinicopathologic review of 10 cases.
Arch Pathol Lab Med 132: 1623-9



7
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



.



Anus and anal canal
ICD-O-3 topography code: C21
ICD10: C21
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Anal melanoma is rare, representing 1?3% of all anal tumours. Patients most commonly present in the fifth or sixth decades of life with pain, rectal bleeding or a mass. The histological features are similar to those of cutaneous melanomas that express S100 protein. Most cases show a junctional component adjacent to the invasive tumour, and this finding is evidence that the lesion is primary rather than metastatic. Metastases are frequent at time of presentation, and the prognosis is poor; 5-year survival is < 20%
8
Click to access Pubmed
Stoidis CN, Spyropoulos BG, Misiakos EP, Fountzilas CK, Paraskeva PP, Fotiadis CI (2009)
Diffuse anorectal melanoma; review of the current diagnostic and treatment aspects based on a case report.
World J Surg Oncol 7: 64



.





Urinary system: Renal pelvis, ureter, bladder, urethra
ICD-O-3 topography code: C65-C68
ICD10: C65-C68
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Penis
ICD-O-3 topography code: C60
ICD10: C60
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Cervix uteri
ICD-O-3 topography code: C53
ICD10: C53
           

Malignant melanoma is a malignant, usually aggressive tumour composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain
9
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma

Malignant melanoma of the cervix is very rare. The tumours are usually polypoid or fungating, pigmented masses. Approximately 50% of cases contain a junctional component. Spindle-shaped cells are frequently present.
The tumour arises in adults. Patients typically present with abnormal vaginal bleeding. The prognosis is dismal
10
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



.



Vulva
ICD-O-3 topography code: C51
ICD10: C51
           

Malignant melanoma is a malignant, usually aggressive tumour histologically composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. They develop most frequently on the face; other commonly affected sites include ears, head, neck, back and shoulders in male patients and the lower limbs in females. Most lesions show an initial radial growth phase within the epidermis and superficial dermis, followed by a vertical growth phase which results in the formation of papules or nodules and eventually involves the subcutaneous tissue
11
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.
Approximately 80% of melanoma patients are light-skinned Caucasians. The most important environmental risk factor is intermittent exposure to UV radiation, especially during childhood. Familial/genetic risk factors include skin type, number of nevi, the presence of dysplastic nevi, and a family history of melanoma. The single most important prognostic factor is vertical tumour thickness; lesions that have entered the vertical growth phase have metastatic potential and a poor prognosis. Usually, melanomas first metastasize to the regional lymph nodes. Distant metastases arise preferentially in skin, lungs, liver, central nervous system and bones, but any organ may be affected. Survival has increased substantially over the last three decades, mainly due to early detection
12
 
LeBoit PE, Burg G, Weedon D, Sarasin A (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Skin Tumours
3rd Edition
IARC Press: Lyon 2005



.

Four major subtypes of malignant melanoma are distinguished:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma

In the vulva, malignant melanomas are uncommon; they account for 2-10% of all vulvar malignancies
13
Click to access Pubmed
Räber G, Mempel V, Jackisch C, Hundeiker M, Heinecke A, Kürzl R, Glaubitz M, Rompel R, Schneider HP (1996)
Malignant melanoma of the vulva. Report of 89 patients.
Cancer 78: 2353-8



. The tumour arises in labia majora, labia minora or clitoris. Patients are predominantly elderly White women; they frequently present with vulvar bleeding, pruritus or dysuria. Treatment of tumours with a thickness of <4mm is usually wide local excision 14
Click to access Pubmed
Trimble EL (1996)
Melanomas of the vulva and vagina.
Oncology (Williston Park) 10: 1017-23; discussion 1024



; larger melanomas are usually treated by radical vulvectomy15
 
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
3rd Edition
IARC Press: Lyon 2003



.



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Lip, Oral Cavity and Oropharynx
ICD-O-3 topography code: C00-C06, C09-C10, C14
ICD10: C00-C06, C09-C10, C14
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Hypopharynx, Larynx and Trachea
ICD-O-3 topography code: C13, C32, C33
ICD10: C13, C32, C33
           

Mucosal malignant melanomas (MMM) of the upper aerodigestive tract represent up to 3% of melanomas. In contrast to their cutaneous conterpart, neural crest-derived primary laryngeal mucosal malignant melanomas (PLMMM) are very rare and predominantly affect adult males.



Ovary
ICD-O-3 topography code: C56
ICD10: C56
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Vagina
ICD-O-3 topography code: C52
ICD10: C52
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Prostate gland
ICD-O-3 topography code: C61
ICD10: C61
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Bronchus and lung
ICD-O-3 topography code: C34
ICD10: C34
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma



Colon and rectum
ICD-O-3 topography code: C18-C20
ICD10: C18-C20
           

A malignant, usually aggressive tumor composed of atypical, neoplastic melanocytes. Cutaneous melanomas may arise from acquired or congenital melanocytic or dysplastic nevi. Melanomas frequently metastasize to lymph nodes, liver, lungs, and brain.

Histologic subtypes of malignant melanoma include:
Superficial spreading melanoma
Nodular melanoma
Acral lentiginous melanoma
Lentigo maligna melanoma