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Research in Review

The Great Mimicker: A Case Highlighting Amelanotic Spitzoid Melanoma

October 2017

A 34-year-old woman presented to the clinic with a 7-month history of a solitary growth on her right upper leg. The lesion had begun as a small pink bump and had grown slowly over the past 7 months (Figure 1 and 2). She denied any associated bleeding or symptoms. She also denied any history of physical trauma or any arthropod bite to the area. She also denied having any other skin lesions. Her past medical history included migraines; however, there was no personal or family history of skin cancer. 

skin cancer

On physical exam, the patient was a well-nourished, healthy, 34-year-old woman with an 8-mm pink, slightly pedunculated, papule on her right upper outer thigh. No other similar lesions were noted on physical exam. On dermoscopy, small cerebriform pink sulci were noted within the papule, without any pigment network or atypical appearing globules or crystalline structures. No ulceration was noted and the surrounding skin was unremarkable. 

skin cancerDue to the lesion growing in size and the patient being concerned about the lesion, the lesion was biopsied with a deep shave technique and sent for histologic examination. Our differential diagnoses included an irritated compound nevus, acrochordon, neuroma, pyogenic granuloma, neurofibroma, or a connective tissue nevus. 
 
Histologic exam of the lesion showed a severely atypical compound Spitzoidmelanocytic proliferation best regarded as a malignant melanoma with dermal invasion to a thickness of 1.5 mm. The lesion was not ulcerated and had a mitotic count of 2 mitotic figures/mm2. No lymphovascular invasion or microsatellitosis was identified (Figures 3 and 4). 
 
The diagnosis of an atypical Spitzoid melanoma was made due to the severe degree of cytologic atypia, the lack of maturation of the dermal component, the presence of deep dermal mitotic figures, the loss of p16 immunopositivity, and the retention of HMB-45 immunopositivity in the dermal component. 
 
Based on the clinical and histopathologic findings, a diagnosis of an amelanotic Spitzoid malignant melanoma was made and the patient was referred to oncologic surgery for wide local excision and sentinel lymph node biopsies in her right groin was performed. The sentinel lymph nodes were negative and there was no residual melanoma at the primary site.  

{{pagebreak}}

Discussion

The majority of malignant melanomas are pigment producing. Amelanotic melanomas constitute only 2% of all melanomas.1 Our patient’s clinical presentation did not show any evidence of melanin on either gross physical exam nor on dermoscopy.

Risk factors for melanoma include exposure to sunlight and UV radiation, presence of multiple dysplastic nevi, family history of melanoma, personal history of multiple sunburns, immunosuppression and organ transplant recipients, blue or green color eye, presence of freckles, history of penetrating injury, and exposure to agricultural chemicals.1 Some cases of melanoma have also been reported in patients with filarial lymphedema.

A typical benign Spitz nevus is characterized by the presence of symmetry of the lesion with well-circumscribed lateral margins without extension of the junctional activity beyond the limits of the dermal component.2 It contains epithelioid and spindle cells with the latter being more common and may show epidermal hyperplasia or acanthosis. There is also a maturation of the deep dermal component of the nevus and solitary or clustered eosinophilic globules known as Kamino bodies may be seen. There is cell maturation, fewer mitosis, and the absence of atypical mitosis. Conversely, atypical mitoses, mitotic rate >2/mm2, and mitoses within 0.25 mm of the base of the lesion along with the clinical features of malignancy favor the diagnosis of Spitzoid melanoma.2

Clinically, Spitzoid melanomas usually evolve from amelanotic nodular lesions, growing to 1 cm or more in diameter. They often remain clinically undiagnosed because of their wide variety of clinical appearances and a lack of pigmentation. Distinguishing a Spitz nevus from a Spitzoid melanoma can be extremely difficult. Features that favor the diagnosis of a Spitzoid melanoma are asymmetrical shape, diameter >1 cm, a lesion with a deep invasive component, and a high degree of cytologic atypia.3

Most Spitz tumors occur in patients younger than 20 years. In those who are in their 20s and 30s who have Spitz nevi, there is a greater likelihood of malignancy in the lesion.3,4 Clinically, Spitzoid melanomas are usually changing amelanotic nodular lesions, and can grow fairly rapidly over several months. They often go clinically undiagnosed because of their wide range of clinical appearances and a lack of pigmentation.

Spitz tumors commonly affect the extremities and face, but atypical Spitz tumors may also, less frequently, affect other areas such as the back. They may resemble hemangiomas, pyogenic granulomas, xanthogranulomas, and basal cell carcinomas.3

Distinguishing between a Spitz nevus and a Spitzoid melanoma can be extremely challenging, both clinically and histologically Features supporting a diagnosis of Spitzoid melanoma include a large size (>10 mm in diameter); ulceration; deep dermal penetration; asymmetry; a lack of circumscription; thinning of the epidermis; extensive, lateral pagetoid extension; an absence of Kamino bodies; a high degree of cytologic atypia; a high mitotic rate especially in the deep dermis; atypical mitoses; increased nucleus-to-cytoplasm ratio; vacuolated irregular cytoplasm; and prominent eosinophilic nucleoli.5 It is also well known that multinucleated giant cells are not uncommon in Spitz tumors.3

Dermoscopy can also aid in the diagnosis of amelanotic melanoma. A multicenter study that examined the dermoscopic appearance of amelanotic melanomas revealed that the combination of structure-less zones with blue, gray, or white colors is useful for the diagnosis of malignant lesions.6 The highest diagnostic sensitivity was achieved when considering only the presence of blue, gray, or white color. Furthermore, polymorphous vessels are often also a clue for malignant melanoma.6

Immunohistochemistry (IHC) studies of such lesions are recommended to confirm the diagnosis such as S100, HMB-45, and Melan-A.7,8 An HMB-45 stain typically shows loss of staining in the deep dermal component of most benign nevi, but uniform staining of blue nevi and in some types of melanoma. A p16 stain is also a melanocytic marker where most benign nevi retain the stain. An S100A6 stain is normally positive in most Spitz nevi. S100 is reported to be the most sensitive marker, however. The specificities of markers HMB-45, S100, and Melan-A, are almost 100%, 75% to 87%, and 95% to 100 %, respectively.7 If needed, pathologists have explored the use of ancillary genomic and genetic studies to improve diagnostic accuracy such as the increasing use of array comparative genomic hybridization and/or fluorescence in situ hybridization for improved diagnostic accuracy of Spitzoid melanocytic neoplasm.9

In the presented case, the lesion had an 8-mm diameter, and histopathology showed deep dermal penetration, lack of circumscription, cytological atypia, and supporting IHC stains. These features supported the diagnosis of a Spitzoid melanoma. Spitzoid melanomas can evolve de novo or they can be associated with a preexisting Spitz nevus. However, the incidence rate of melanoma evolving in Spitz nevi is currently unknown. Furthermore, amelanotic melanomas may arise from normal melanomas with amelanotic transformation.10 

In summary, recognition of this rare form of melanoma, with its unusual morphology, is important to avoid a misdiagnosis or delay of diagnosis. The lack of pigmentation in these lesions on clinical exam can lead an unwary clinician to not suspect a melanocytic proliferation. Thus, a thorough history, physical, and dermoscopic exam is prudent as well as a skin biopsy with IHC if there is any clinical doubt or dermoscopic evidence of features of malignancy. Long-term follow-up and more studies are needed to assess the prognostic implication associated with this unusual morphology.3 Wide local excision with adequate margins for malignant melanoma is the conventional strategy for treatment and sentinel lymph node biopsy when indicated.

Survival rate in malignant melanoma is strongly related to tumor thickness and local and distant tissue spread at the time of diagnosis. Accordingly, early detection and diagnosis are crucial for more effective treatment and greater likelihood of disease-free survival.1 Other adjuvant techniques, such as radiotherapy, chemotherapy, and immunotherapy have not been well established in the literature and success rates and disease-free survival depend on the site involved and extent of the tumor. Furthermore, the use of gene expression profiling may be an additional tool to aid in the prognosis and management of these atypical melanomas. 

Dr Dane is a board-certified dermatologist and Mohs/Procedural Dermatology fellow at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Dr Gonzales is a board-certified pathologist in a private group practice in Morristown, NJ.

Dr. Rogachefsky is the Program Director of the ACGME-approved Micrographic Surgery and Procedural Dermatology Fellowship and practicing dermatologist at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Disclosure: The authors report no relevant financial relationships.

References

1. Nayak M, Patra S, Meher S, Sasmal PK. Amelanotic melanoma arising in filarial leg: A report of a rare case. J Clin Diagn Res. 2017;11(1):ED07-ED09.

2. Goh EH, Zarina AL, Thambidorai CR, Maizaton AA, Siti AM, Somasundram S. Amelanotic spitzoid melanoma in a prepubescent boy. Pediatr Surg Int. 2008;24(4):447-449.

3. Kim HY, Yoon JH, Cho EB, Park EJ, Kim KH, Kim KJ. A case of spitzoid melanoma. Ann Dermatol. 2015;27(2):206-209. 

A 34-year-old woman presented to the clinic with a 7-month history of a solitary growth on her right upper leg. The lesion had begun as a small pink bump and had grown slowly over the past 7 months (Figure 1 and 2). She denied any associated bleeding or symptoms. She also denied any history of physical trauma or any arthropod bite to the area. She also denied having any other skin lesions. Her past medical history included migraines; however, there was no personal or family history of skin cancer. 

skin cancer

On physical exam, the patient was a well-nourished, healthy, 34-year-old woman with an 8-mm pink, slightly pedunculated, papule on her right upper outer thigh. No other similar lesions were noted on physical exam. On dermoscopy, small cerebriform pink sulci were noted within the papule, without any pigment network or atypical appearing globules or crystalline structures. No ulceration was noted and the surrounding skin was unremarkable. 

skin cancerDue to the lesion growing in size and the patient being concerned about the lesion, the lesion was biopsied with a deep shave technique and sent for histologic examination. Our differential diagnoses included an irritated compound nevus, acrochordon, neuroma, pyogenic granuloma, neurofibroma, or a connective tissue nevus. 
 
Histologic exam of the lesion showed a severely atypical compound Spitzoidmelanocytic proliferation best regarded as a malignant melanoma with dermal invasion to a thickness of 1.5 mm. The lesion was not ulcerated and had a mitotic count of 2 mitotic figures/mm2. No lymphovascular invasion or microsatellitosis was identified (Figures 3 and 4). 
 
The diagnosis of an atypical Spitzoid melanoma was made due to the severe degree of cytologic atypia, the lack of maturation of the dermal component, the presence of deep dermal mitotic figures, the loss of p16 immunopositivity, and the retention of HMB-45 immunopositivity in the dermal component. 
 
Based on the clinical and histopathologic findings, a diagnosis of an amelanotic Spitzoid malignant melanoma was made and the patient was referred to oncologic surgery for wide local excision and sentinel lymph node biopsies in her right groin was performed. The sentinel lymph nodes were negative and there was no residual melanoma at the primary site.  

{{pagebreak}}

Discussion

The majority of malignant melanomas are pigment producing. Amelanotic melanomas constitute only 2% of all melanomas.1 Our patient’s clinical presentation did not show any evidence of melanin on either gross physical exam nor on dermoscopy.

Risk factors for melanoma include exposure to sunlight and UV radiation, presence of multiple dysplastic nevi, family history of melanoma, personal history of multiple sunburns, immunosuppression and organ transplant recipients, blue or green color eye, presence of freckles, history of penetrating injury, and exposure to agricultural chemicals.1 Some cases of melanoma have also been reported in patients with filarial lymphedema.

A typical benign Spitz nevus is characterized by the presence of symmetry of the lesion with well-circumscribed lateral margins without extension of the junctional activity beyond the limits of the dermal component.2 It contains epithelioid and spindle cells with the latter being more common and may show epidermal hyperplasia or acanthosis. There is also a maturation of the deep dermal component of the nevus and solitary or clustered eosinophilic globules known as Kamino bodies may be seen. There is cell maturation, fewer mitosis, and the absence of atypical mitosis. Conversely, atypical mitoses, mitotic rate >2/mm2, and mitoses within 0.25 mm of the base of the lesion along with the clinical features of malignancy favor the diagnosis of Spitzoid melanoma.2

Clinically, Spitzoid melanomas usually evolve from amelanotic nodular lesions, growing to 1 cm or more in diameter. They often remain clinically undiagnosed because of their wide variety of clinical appearances and a lack of pigmentation. Distinguishing a Spitz nevus from a Spitzoid melanoma can be extremely difficult. Features that favor the diagnosis of a Spitzoid melanoma are asymmetrical shape, diameter >1 cm, a lesion with a deep invasive component, and a high degree of cytologic atypia.3

Most Spitz tumors occur in patients younger than 20 years. In those who are in their 20s and 30s who have Spitz nevi, there is a greater likelihood of malignancy in the lesion.3,4 Clinically, Spitzoid melanomas are usually changing amelanotic nodular lesions, and can grow fairly rapidly over several months. They often go clinically undiagnosed because of their wide range of clinical appearances and a lack of pigmentation.

Spitz tumors commonly affect the extremities and face, but atypical Spitz tumors may also, less frequently, affect other areas such as the back. They may resemble hemangiomas, pyogenic granulomas, xanthogranulomas, and basal cell carcinomas.3

Distinguishing between a Spitz nevus and a Spitzoid melanoma can be extremely challenging, both clinically and histologically Features supporting a diagnosis of Spitzoid melanoma include a large size (>10 mm in diameter); ulceration; deep dermal penetration; asymmetry; a lack of circumscription; thinning of the epidermis; extensive, lateral pagetoid extension; an absence of Kamino bodies; a high degree of cytologic atypia; a high mitotic rate especially in the deep dermis; atypical mitoses; increased nucleus-to-cytoplasm ratio; vacuolated irregular cytoplasm; and prominent eosinophilic nucleoli.5 It is also well known that multinucleated giant cells are not uncommon in Spitz tumors.3

Dermoscopy can also aid in the diagnosis of amelanotic melanoma. A multicenter study that examined the dermoscopic appearance of amelanotic melanomas revealed that the combination of structure-less zones with blue, gray, or white colors is useful for the diagnosis of malignant lesions.6 The highest diagnostic sensitivity was achieved when considering only the presence of blue, gray, or white color. Furthermore, polymorphous vessels are often also a clue for malignant melanoma.6

Immunohistochemistry (IHC) studies of such lesions are recommended to confirm the diagnosis such as S100, HMB-45, and Melan-A.7,8 An HMB-45 stain typically shows loss of staining in the deep dermal component of most benign nevi, but uniform staining of blue nevi and in some types of melanoma. A p16 stain is also a melanocytic marker where most benign nevi retain the stain. An S100A6 stain is normally positive in most Spitz nevi. S100 is reported to be the most sensitive marker, however. The specificities of markers HMB-45, S100, and Melan-A, are almost 100%, 75% to 87%, and 95% to 100 %, respectively.7 If needed, pathologists have explored the use of ancillary genomic and genetic studies to improve diagnostic accuracy such as the increasing use of array comparative genomic hybridization and/or fluorescence in situ hybridization for improved diagnostic accuracy of Spitzoid melanocytic neoplasm.9

In the presented case, the lesion had an 8-mm diameter, and histopathology showed deep dermal penetration, lack of circumscription, cytological atypia, and supporting IHC stains. These features supported the diagnosis of a Spitzoid melanoma. Spitzoid melanomas can evolve de novo or they can be associated with a preexisting Spitz nevus. However, the incidence rate of melanoma evolving in Spitz nevi is currently unknown. Furthermore, amelanotic melanomas may arise from normal melanomas with amelanotic transformation.10 

In summary, recognition of this rare form of melanoma, with its unusual morphology, is important to avoid a misdiagnosis or delay of diagnosis. The lack of pigmentation in these lesions on clinical exam can lead an unwary clinician to not suspect a melanocytic proliferation. Thus, a thorough history, physical, and dermoscopic exam is prudent as well as a skin biopsy with IHC if there is any clinical doubt or dermoscopic evidence of features of malignancy. Long-term follow-up and more studies are needed to assess the prognostic implication associated with this unusual morphology.3 Wide local excision with adequate margins for malignant melanoma is the conventional strategy for treatment and sentinel lymph node biopsy when indicated.

Survival rate in malignant melanoma is strongly related to tumor thickness and local and distant tissue spread at the time of diagnosis. Accordingly, early detection and diagnosis are crucial for more effective treatment and greater likelihood of disease-free survival.1 Other adjuvant techniques, such as radiotherapy, chemotherapy, and immunotherapy have not been well established in the literature and success rates and disease-free survival depend on the site involved and extent of the tumor. Furthermore, the use of gene expression profiling may be an additional tool to aid in the prognosis and management of these atypical melanomas. 

Dr Dane is a board-certified dermatologist and Mohs/Procedural Dermatology fellow at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Dr Gonzales is a board-certified pathologist in a private group practice in Morristown, NJ.

Dr. Rogachefsky is the Program Director of the ACGME-approved Micrographic Surgery and Procedural Dermatology Fellowship and practicing dermatologist at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Disclosure: The authors report no relevant financial relationships.

References

1. Nayak M, Patra S, Meher S, Sasmal PK. Amelanotic melanoma arising in filarial leg: A report of a rare case. J Clin Diagn Res. 2017;11(1):ED07-ED09.

2. Goh EH, Zarina AL, Thambidorai CR, Maizaton AA, Siti AM, Somasundram S. Amelanotic spitzoid melanoma in a prepubescent boy. Pediatr Surg Int. 2008;24(4):447-449.

3. Kim HY, Yoon JH, Cho EB, Park EJ, Kim KH, Kim KJ. A case of spitzoid melanoma. Ann Dermatol. 2015;27(2):206-209. 

A 34-year-old woman presented to the clinic with a 7-month history of a solitary growth on her right upper leg. The lesion had begun as a small pink bump and had grown slowly over the past 7 months (Figure 1 and 2). She denied any associated bleeding or symptoms. She also denied any history of physical trauma or any arthropod bite to the area. She also denied having any other skin lesions. Her past medical history included migraines; however, there was no personal or family history of skin cancer. 

skin cancer

On physical exam, the patient was a well-nourished, healthy, 34-year-old woman with an 8-mm pink, slightly pedunculated, papule on her right upper outer thigh. No other similar lesions were noted on physical exam. On dermoscopy, small cerebriform pink sulci were noted within the papule, without any pigment network or atypical appearing globules or crystalline structures. No ulceration was noted and the surrounding skin was unremarkable. 

skin cancerDue to the lesion growing in size and the patient being concerned about the lesion, the lesion was biopsied with a deep shave technique and sent for histologic examination. Our differential diagnoses included an irritated compound nevus, acrochordon, neuroma, pyogenic granuloma, neurofibroma, or a connective tissue nevus. 
 
Histologic exam of the lesion showed a severely atypical compound Spitzoidmelanocytic proliferation best regarded as a malignant melanoma with dermal invasion to a thickness of 1.5 mm. The lesion was not ulcerated and had a mitotic count of 2 mitotic figures/mm2. No lymphovascular invasion or microsatellitosis was identified (Figures 3 and 4). 
 
The diagnosis of an atypical Spitzoid melanoma was made due to the severe degree of cytologic atypia, the lack of maturation of the dermal component, the presence of deep dermal mitotic figures, the loss of p16 immunopositivity, and the retention of HMB-45 immunopositivity in the dermal component. 
 
Based on the clinical and histopathologic findings, a diagnosis of an amelanotic Spitzoid malignant melanoma was made and the patient was referred to oncologic surgery for wide local excision and sentinel lymph node biopsies in her right groin was performed. The sentinel lymph nodes were negative and there was no residual melanoma at the primary site.  

{{pagebreak}}

Discussion

The majority of malignant melanomas are pigment producing. Amelanotic melanomas constitute only 2% of all melanomas.1 Our patient’s clinical presentation did not show any evidence of melanin on either gross physical exam nor on dermoscopy.

Risk factors for melanoma include exposure to sunlight and UV radiation, presence of multiple dysplastic nevi, family history of melanoma, personal history of multiple sunburns, immunosuppression and organ transplant recipients, blue or green color eye, presence of freckles, history of penetrating injury, and exposure to agricultural chemicals.1 Some cases of melanoma have also been reported in patients with filarial lymphedema.

A typical benign Spitz nevus is characterized by the presence of symmetry of the lesion with well-circumscribed lateral margins without extension of the junctional activity beyond the limits of the dermal component.2 It contains epithelioid and spindle cells with the latter being more common and may show epidermal hyperplasia or acanthosis. There is also a maturation of the deep dermal component of the nevus and solitary or clustered eosinophilic globules known as Kamino bodies may be seen. There is cell maturation, fewer mitosis, and the absence of atypical mitosis. Conversely, atypical mitoses, mitotic rate >2/mm2, and mitoses within 0.25 mm of the base of the lesion along with the clinical features of malignancy favor the diagnosis of Spitzoid melanoma.2

Clinically, Spitzoid melanomas usually evolve from amelanotic nodular lesions, growing to 1 cm or more in diameter. They often remain clinically undiagnosed because of their wide variety of clinical appearances and a lack of pigmentation. Distinguishing a Spitz nevus from a Spitzoid melanoma can be extremely difficult. Features that favor the diagnosis of a Spitzoid melanoma are asymmetrical shape, diameter >1 cm, a lesion with a deep invasive component, and a high degree of cytologic atypia.3

Most Spitz tumors occur in patients younger than 20 years. In those who are in their 20s and 30s who have Spitz nevi, there is a greater likelihood of malignancy in the lesion.3,4 Clinically, Spitzoid melanomas are usually changing amelanotic nodular lesions, and can grow fairly rapidly over several months. They often go clinically undiagnosed because of their wide range of clinical appearances and a lack of pigmentation.

Spitz tumors commonly affect the extremities and face, but atypical Spitz tumors may also, less frequently, affect other areas such as the back. They may resemble hemangiomas, pyogenic granulomas, xanthogranulomas, and basal cell carcinomas.3

Distinguishing between a Spitz nevus and a Spitzoid melanoma can be extremely challenging, both clinically and histologically Features supporting a diagnosis of Spitzoid melanoma include a large size (>10 mm in diameter); ulceration; deep dermal penetration; asymmetry; a lack of circumscription; thinning of the epidermis; extensive, lateral pagetoid extension; an absence of Kamino bodies; a high degree of cytologic atypia; a high mitotic rate especially in the deep dermis; atypical mitoses; increased nucleus-to-cytoplasm ratio; vacuolated irregular cytoplasm; and prominent eosinophilic nucleoli.5 It is also well known that multinucleated giant cells are not uncommon in Spitz tumors.3

Dermoscopy can also aid in the diagnosis of amelanotic melanoma. A multicenter study that examined the dermoscopic appearance of amelanotic melanomas revealed that the combination of structure-less zones with blue, gray, or white colors is useful for the diagnosis of malignant lesions.6 The highest diagnostic sensitivity was achieved when considering only the presence of blue, gray, or white color. Furthermore, polymorphous vessels are often also a clue for malignant melanoma.6

Immunohistochemistry (IHC) studies of such lesions are recommended to confirm the diagnosis such as S100, HMB-45, and Melan-A.7,8 An HMB-45 stain typically shows loss of staining in the deep dermal component of most benign nevi, but uniform staining of blue nevi and in some types of melanoma. A p16 stain is also a melanocytic marker where most benign nevi retain the stain. An S100A6 stain is normally positive in most Spitz nevi. S100 is reported to be the most sensitive marker, however. The specificities of markers HMB-45, S100, and Melan-A, are almost 100%, 75% to 87%, and 95% to 100 %, respectively.7 If needed, pathologists have explored the use of ancillary genomic and genetic studies to improve diagnostic accuracy such as the increasing use of array comparative genomic hybridization and/or fluorescence in situ hybridization for improved diagnostic accuracy of Spitzoid melanocytic neoplasm.9

In the presented case, the lesion had an 8-mm diameter, and histopathology showed deep dermal penetration, lack of circumscription, cytological atypia, and supporting IHC stains. These features supported the diagnosis of a Spitzoid melanoma. Spitzoid melanomas can evolve de novo or they can be associated with a preexisting Spitz nevus. However, the incidence rate of melanoma evolving in Spitz nevi is currently unknown. Furthermore, amelanotic melanomas may arise from normal melanomas with amelanotic transformation.10 

In summary, recognition of this rare form of melanoma, with its unusual morphology, is important to avoid a misdiagnosis or delay of diagnosis. The lack of pigmentation in these lesions on clinical exam can lead an unwary clinician to not suspect a melanocytic proliferation. Thus, a thorough history, physical, and dermoscopic exam is prudent as well as a skin biopsy with IHC if there is any clinical doubt or dermoscopic evidence of features of malignancy. Long-term follow-up and more studies are needed to assess the prognostic implication associated with this unusual morphology.3 Wide local excision with adequate margins for malignant melanoma is the conventional strategy for treatment and sentinel lymph node biopsy when indicated.

Survival rate in malignant melanoma is strongly related to tumor thickness and local and distant tissue spread at the time of diagnosis. Accordingly, early detection and diagnosis are crucial for more effective treatment and greater likelihood of disease-free survival.1 Other adjuvant techniques, such as radiotherapy, chemotherapy, and immunotherapy have not been well established in the literature and success rates and disease-free survival depend on the site involved and extent of the tumor. Furthermore, the use of gene expression profiling may be an additional tool to aid in the prognosis and management of these atypical melanomas. 

Dr Dane is a board-certified dermatologist and Mohs/Procedural Dermatology fellow at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Dr Gonzales is a board-certified pathologist in a private group practice in Morristown, NJ.

Dr. Rogachefsky is the Program Director of the ACGME-approved Micrographic Surgery and Procedural Dermatology Fellowship and practicing dermatologist at Affiliated Dermatologists and Dermatologic Surgeons in Morristown, NJ.

Disclosure: The authors report no relevant financial relationships.

References

1. Nayak M, Patra S, Meher S, Sasmal PK. Amelanotic melanoma arising in filarial leg: A report of a rare case. J Clin Diagn Res. 2017;11(1):ED07-ED09.

2. Goh EH, Zarina AL, Thambidorai CR, Maizaton AA, Siti AM, Somasundram S. Amelanotic spitzoid melanoma in a prepubescent boy. Pediatr Surg Int. 2008;24(4):447-449.

3. Kim HY, Yoon JH, Cho EB, Park EJ, Kim KH, Kim KJ. A case of spitzoid melanoma. Ann Dermatol. 2015;27(2):206-209. 

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