Spitz nevus is a benign form of melanocytic nevus that is not common. It is named after Dr. Sophie Spitz who, in 1948, described a group of cases which she called, melanomas of childhood. In her original report, the 13 cases are now classified as Spitz nevi with only one case determined to be melanoma, which spread to other parts and caused metastasis and death. In spite of its benign nature, Spitz nevus was historically called juvenile melanoma, benign juvenile melanoma or pre-pubertal melanoma. These words are deceptive, in the sense that they imply malignancy. One of the more complicated dermatopathological challenges is to differentiate between Spitz nevus and spitzoid melanoma. Recent molecular genetic research has determined that a spectrum of Spitz tumors has different mutations in Spitz nevi. This spectrum includes classic Spitz nevus to Spitz melanoma, and in between them is the Spitz melanocytoma or atypical Spitz tumor. These transitional tumors have histopathologic appearances that are unclear or indeterminate, and they represent partial differentiation to malignancy. Spitz nevus is still a specific type of melanocytic nevus, clinically and histologically, which is usually constituted of spindled and/or epithelioid melanocytes in a benign tumor pattern. Other names comprise Spitz tumor, Spitz nevus and spindle and epithelioid cell nevus. Spitz nevi are of junctional type, compound (most common type) and intradermal type. The desmoplastic Spitz nevi are mostly intradermal or mostly intradermal. The pigmented spindle cell nevus (Reed nevus) is another known variant that is generally considered to be a clinically and histologically discrete type of Spitz spectrum. Many other clinical and histologic variations are also described.
Epidemiology
Spitz nevi are rare melanocytic lesions, and they occur in less than 1 % of skin moles. They are commonly diagnosed in children and adolescents with most of them being diagnosed before the age of 20, though they can be diagnosed in adults. No intense sex predilection and some studies indicate some slight female preponderance. The lesions tend to occur more frequently in patients who have a lighter skin and are usually found on the head, neck, or extremities. Although the Spitz nevi are rare, they have clinical importance because they resemble melanoma that usually requires biopsy and histopathological tests.
Anatomy
Pathophysiology
Spitz nevi are melanocytic proliferations that are made up of spindled and/or epithelioid melanocytes that develop in the epidermis and dermis as well-circumscribed lesions. They differ histologically with the traditional melanocytic nevi, and in most cases, they harbor cytologic characteristics often resembling melanoma, and thus accuracy in diagnosis is significant. Somatic genetic changes that favor the growth of melanocytes cause the pathophysiology of Spitz nevi. The most frequent molecular alterations are HRAS mutations, sporadic BRAF or NRAS mutations, as well as, kinase gene fusions, including ALK, ROS1, NTRK1, RET and BRAF, that cause downstream pathway activation such as MAPK/ERK to promote cell growth and survival. These genetic occurrences lead to clonal proliferation of melanocytes that have restricted mitotic capabilities and therefore retain the benign nature of the lesion. Nonetheless, there are lesions known as atypical Spitz tumors or Spitz melanocytomas, which have intermediate characteristics, with unclear histology and partial capacity of malignant evolution. In general, the pathophysiology of Spitz nevi represents a range of the biologic behaviour, with either completely harmless lesions, or with atypical lesions, which demand close histopathologic and molecular evaluation.
Etiology
Spitz nevi are benign melanocytic proliferations, and its mechanism of action is not quite clear. They develop within melanocytes, the pigment-producing cells of the skin and are believed to be a product of localized proliferation of melanocytes as a consequence of somatic mutations. Molecular analyses have verified a range of genetic mutations in Spitz nevi such as mutations in HRAS, BRAF, and NRAS, and a variety of kinase fusions (e.g., ALK, ROS1, NTRK1, BRAF fusions) implicating a spectrum of biologic behavior of benign to potentially atypical lesions. Such mutations are frequently not the same as those that are observed in typical melanocytic nevi or melanomas, thus providing a molecular distinction between Spitz nevi. Most Spitz nevi do not seem to have a significant environmental influence, or triggered by trauma or UV exposure, but there are few cases that could be related to inflammatory or proliferative stimuli.
Genetics
Prognostic Factors
Spitz nevi are generally benign with an excellent prognosis. Classic lesions in children are almost always harmless, while atypical Spitz tumors may have a slightly higher risk of local recurrence or, rarely, metastasis. The most important prognostic variables are the age of the patient, the size of the lesion, the histologic characteristics of diseases, including mitotic activity, depth of invasion, and cytologic atypia. Risk assessment can be also guided by molecular data, such as HRAS mutation or fusion of two kinases. Total excision and meticulous histopathologic review usually ensure a positive result.
Clinical History
Age group
Spitz tumors occur between 10 and 20 years of age, their distinction from melanoma is duly influenced by consideration of patient age.
Physical Examination
Age group
Associated comorbidity
Spitz nevi are generally isolated benign skin lesions and are not associated with systemic comorbidities or other medical conditions. They usually arise sporadically without links to syndromes, systemic diseases, or specific activities. Rarely, they may coexist with other melanocytic proliferations but without a consistent comorbidity pattern.
Associated activity
Acuity of presentation
Spitz nevi typically present with a sudden onset of a solitary, dome-shaped papule or nodule that often grows rapidly over weeks to months before stabilizing. This relatively acute and noticeable growth phase can raise concern for malignancy, even though the lesion is benign.
Differential Diagnoses
Pyogenic granuloma
Melanocytic nevi
Spitz melanocytoma/atypical Spitz tumor
Malignant melanoma
Juvenile xanthogranuloma
Laboratory Studies
Imaging Studies
Procedures
Histologic Findings
Staging
Treatment Paradigm
The treatment paradigm for Spitz nevi centers on distinguishing them from spitzoid melanoma, since the two can appear similar clinically and histologically. In children, small and typical Spitz nevi may be observed with close clinical follow-up, especially when the diagnosis is clear. However, because of the diagnostic uncertainty, complete surgical excision with narrow margins is often recommended to allow for thorough histopathologic evaluation. In adults, excision is generally advised more consistently due to the higher risk of melanoma in this age group. Atypical Spitz tumors or lesions with concerning features may require wider excision, sentinel lymph node biopsy, or closer surveillance. Overall, management balances the benign nature of most Spitz nevi with the need to rule out melanoma and ensure patient safety.
First-line procedure for diagnosis and management.
Ensures complete removal of the lesion and provides tissue for histopathology.
Wide local excision (in atypical Spitz tumors or when melanoma cannot be excluded):
Wider margins are taken, similar to melanoma excision protocols, to reduce recurrence risk.
Sentinel lymph node biopsy (SLNB):
Considered in atypical Spitz tumors or spitzoid melanoma with worrisome histological features.
Helps assess regional nodal involvement, though its prognostic value in Spitz tumors is still debated.
role-of-management-in-treating-spitz-nevi
Initial Evaluation The first phase involves careful clinical assessment of the lesion, including the patient’s age, history of onset, growth pattern, and any changes in appearance. Dermoscopy is often performed to look for characteristic patterns associated with Spitz nevi. Baseline photographs may also be taken to monitor the lesion over time.
Diagnostic Phase In most cases, an excisional biopsy with narrow margins is the preferred diagnostic step, as it allows for complete tissue sampling and accurate histopathological evaluation. Based on microscopic findings, lesions are classified as benign Spitz nevus, atypical Spitz tumor (Spitz melanocytoma), or spitzoid melanoma.
Definitive Management Management depends on the histological diagnosis. A benign Spitz nevus is generally cured with complete excision. Atypical Spitz tumors usually require wide local excision to ensure clear margins, and in selected cases, a sentinel lymph node biopsy may be considered. Spitzoid melanoma is treated according to standard melanoma protocols, which include wide excision, sentinel node evaluation, staging, and adjuvant therapy when appropriate.
Follow-Up Phase Ongoing follow-up is recommended for all patients, especially those with atypical lesions. This includes regular skin examinations to detect recurrence or new lesions, as well as patient education on self-monitoring and sun protection. In atypical or uncertain cases, closer surveillance is warranted given the variable biological behavior of these tumors.
Spitz nevus is a benign form of melanocytic nevus that is not common. It is named after Dr. Sophie Spitz who, in 1948, described a group of cases which she called, melanomas of childhood. In her original report, the 13 cases are now classified as Spitz nevi with only one case determined to be melanoma, which spread to other parts and caused metastasis and death. In spite of its benign nature, Spitz nevus was historically called juvenile melanoma, benign juvenile melanoma or pre-pubertal melanoma. These words are deceptive, in the sense that they imply malignancy. One of the more complicated dermatopathological challenges is to differentiate between Spitz nevus and spitzoid melanoma. Recent molecular genetic research has determined that a spectrum of Spitz tumors has different mutations in Spitz nevi. This spectrum includes classic Spitz nevus to Spitz melanoma, and in between them is the Spitz melanocytoma or atypical Spitz tumor. These transitional tumors have histopathologic appearances that are unclear or indeterminate, and they represent partial differentiation to malignancy. Spitz nevus is still a specific type of melanocytic nevus, clinically and histologically, which is usually constituted of spindled and/or epithelioid melanocytes in a benign tumor pattern. Other names comprise Spitz tumor, Spitz nevus and spindle and epithelioid cell nevus. Spitz nevi are of junctional type, compound (most common type) and intradermal type. The desmoplastic Spitz nevi are mostly intradermal or mostly intradermal. The pigmented spindle cell nevus (Reed nevus) is another known variant that is generally considered to be a clinically and histologically discrete type of Spitz spectrum. Many other clinical and histologic variations are also described.
Spitz nevi are rare melanocytic lesions, and they occur in less than 1 % of skin moles. They are commonly diagnosed in children and adolescents with most of them being diagnosed before the age of 20, though they can be diagnosed in adults. No intense sex predilection and some studies indicate some slight female preponderance. The lesions tend to occur more frequently in patients who have a lighter skin and are usually found on the head, neck, or extremities. Although the Spitz nevi are rare, they have clinical importance because they resemble melanoma that usually requires biopsy and histopathological tests.
Spitz nevi are melanocytic proliferations that are made up of spindled and/or epithelioid melanocytes that develop in the epidermis and dermis as well-circumscribed lesions. They differ histologically with the traditional melanocytic nevi, and in most cases, they harbor cytologic characteristics often resembling melanoma, and thus accuracy in diagnosis is significant. Somatic genetic changes that favor the growth of melanocytes cause the pathophysiology of Spitz nevi. The most frequent molecular alterations are HRAS mutations, sporadic BRAF or NRAS mutations, as well as, kinase gene fusions, including ALK, ROS1, NTRK1, RET and BRAF, that cause downstream pathway activation such as MAPK/ERK to promote cell growth and survival. These genetic occurrences lead to clonal proliferation of melanocytes that have restricted mitotic capabilities and therefore retain the benign nature of the lesion. Nonetheless, there are lesions known as atypical Spitz tumors or Spitz melanocytomas, which have intermediate characteristics, with unclear histology and partial capacity of malignant evolution. In general, the pathophysiology of Spitz nevi represents a range of the biologic behaviour, with either completely harmless lesions, or with atypical lesions, which demand close histopathologic and molecular evaluation.
Spitz nevi are benign melanocytic proliferations, and its mechanism of action is not quite clear. They develop within melanocytes, the pigment-producing cells of the skin and are believed to be a product of localized proliferation of melanocytes as a consequence of somatic mutations. Molecular analyses have verified a range of genetic mutations in Spitz nevi such as mutations in HRAS, BRAF, and NRAS, and a variety of kinase fusions (e.g., ALK, ROS1, NTRK1, BRAF fusions) implicating a spectrum of biologic behavior of benign to potentially atypical lesions. Such mutations are frequently not the same as those that are observed in typical melanocytic nevi or melanomas, thus providing a molecular distinction between Spitz nevi. Most Spitz nevi do not seem to have a significant environmental influence, or triggered by trauma or UV exposure, but there are few cases that could be related to inflammatory or proliferative stimuli.
Spitz nevi are generally benign with an excellent prognosis. Classic lesions in children are almost always harmless, while atypical Spitz tumors may have a slightly higher risk of local recurrence or, rarely, metastasis. The most important prognostic variables are the age of the patient, the size of the lesion, the histologic characteristics of diseases, including mitotic activity, depth of invasion, and cytologic atypia. Risk assessment can be also guided by molecular data, such as HRAS mutation or fusion of two kinases. Total excision and meticulous histopathologic review usually ensure a positive result.
Age group
Spitz tumors occur between 10 and 20 years of age, their distinction from melanoma is duly influenced by consideration of patient age.
Spitz nevi are generally isolated benign skin lesions and are not associated with systemic comorbidities or other medical conditions. They usually arise sporadically without links to syndromes, systemic diseases, or specific activities. Rarely, they may coexist with other melanocytic proliferations but without a consistent comorbidity pattern.
Spitz nevi typically present with a sudden onset of a solitary, dome-shaped papule or nodule that often grows rapidly over weeks to months before stabilizing. This relatively acute and noticeable growth phase can raise concern for malignancy, even though the lesion is benign.
Pyogenic granuloma
Melanocytic nevi
Spitz melanocytoma/atypical Spitz tumor
Malignant melanoma
Juvenile xanthogranuloma
The treatment paradigm for Spitz nevi centers on distinguishing them from spitzoid melanoma, since the two can appear similar clinically and histologically. In children, small and typical Spitz nevi may be observed with close clinical follow-up, especially when the diagnosis is clear. However, because of the diagnostic uncertainty, complete surgical excision with narrow margins is often recommended to allow for thorough histopathologic evaluation. In adults, excision is generally advised more consistently due to the higher risk of melanoma in this age group. Atypical Spitz tumors or lesions with concerning features may require wider excision, sentinel lymph node biopsy, or closer surveillance. Overall, management balances the benign nature of most Spitz nevi with the need to rule out melanoma and ensure patient safety.
Dermatology, General
Excisional biopsy:
First-line procedure for diagnosis and management.
Ensures complete removal of the lesion and provides tissue for histopathology.
Wide local excision (in atypical Spitz tumors or when melanoma cannot be excluded):
Wider margins are taken, similar to melanoma excision protocols, to reduce recurrence risk.
Sentinel lymph node biopsy (SLNB):
Considered in atypical Spitz tumors or spitzoid melanoma with worrisome histological features.
Helps assess regional nodal involvement, though its prognostic value in Spitz tumors is still debated.
Dermatology, General
Initial Evaluation The first phase involves careful clinical assessment of the lesion, including the patient’s age, history of onset, growth pattern, and any changes in appearance. Dermoscopy is often performed to look for characteristic patterns associated with Spitz nevi. Baseline photographs may also be taken to monitor the lesion over time.
Diagnostic Phase In most cases, an excisional biopsy with narrow margins is the preferred diagnostic step, as it allows for complete tissue sampling and accurate histopathological evaluation. Based on microscopic findings, lesions are classified as benign Spitz nevus, atypical Spitz tumor (Spitz melanocytoma), or spitzoid melanoma.
Definitive Management Management depends on the histological diagnosis. A benign Spitz nevus is generally cured with complete excision. Atypical Spitz tumors usually require wide local excision to ensure clear margins, and in selected cases, a sentinel lymph node biopsy may be considered. Spitzoid melanoma is treated according to standard melanoma protocols, which include wide excision, sentinel node evaluation, staging, and adjuvant therapy when appropriate.
Follow-Up Phase Ongoing follow-up is recommended for all patients, especially those with atypical lesions. This includes regular skin examinations to detect recurrence or new lesions, as well as patient education on self-monitoring and sun protection. In atypical or uncertain cases, closer surveillance is warranted given the variable biological behavior of these tumors.
Spitz nevus is a benign form of melanocytic nevus that is not common. It is named after Dr. Sophie Spitz who, in 1948, described a group of cases which she called, melanomas of childhood. In her original report, the 13 cases are now classified as Spitz nevi with only one case determined to be melanoma, which spread to other parts and caused metastasis and death. In spite of its benign nature, Spitz nevus was historically called juvenile melanoma, benign juvenile melanoma or pre-pubertal melanoma. These words are deceptive, in the sense that they imply malignancy. One of the more complicated dermatopathological challenges is to differentiate between Spitz nevus and spitzoid melanoma. Recent molecular genetic research has determined that a spectrum of Spitz tumors has different mutations in Spitz nevi. This spectrum includes classic Spitz nevus to Spitz melanoma, and in between them is the Spitz melanocytoma or atypical Spitz tumor. These transitional tumors have histopathologic appearances that are unclear or indeterminate, and they represent partial differentiation to malignancy. Spitz nevus is still a specific type of melanocytic nevus, clinically and histologically, which is usually constituted of spindled and/or epithelioid melanocytes in a benign tumor pattern. Other names comprise Spitz tumor, Spitz nevus and spindle and epithelioid cell nevus. Spitz nevi are of junctional type, compound (most common type) and intradermal type. The desmoplastic Spitz nevi are mostly intradermal or mostly intradermal. The pigmented spindle cell nevus (Reed nevus) is another known variant that is generally considered to be a clinically and histologically discrete type of Spitz spectrum. Many other clinical and histologic variations are also described.
Spitz nevi are rare melanocytic lesions, and they occur in less than 1 % of skin moles. They are commonly diagnosed in children and adolescents with most of them being diagnosed before the age of 20, though they can be diagnosed in adults. No intense sex predilection and some studies indicate some slight female preponderance. The lesions tend to occur more frequently in patients who have a lighter skin and are usually found on the head, neck, or extremities. Although the Spitz nevi are rare, they have clinical importance because they resemble melanoma that usually requires biopsy and histopathological tests.
Spitz nevi are melanocytic proliferations that are made up of spindled and/or epithelioid melanocytes that develop in the epidermis and dermis as well-circumscribed lesions. They differ histologically with the traditional melanocytic nevi, and in most cases, they harbor cytologic characteristics often resembling melanoma, and thus accuracy in diagnosis is significant. Somatic genetic changes that favor the growth of melanocytes cause the pathophysiology of Spitz nevi. The most frequent molecular alterations are HRAS mutations, sporadic BRAF or NRAS mutations, as well as, kinase gene fusions, including ALK, ROS1, NTRK1, RET and BRAF, that cause downstream pathway activation such as MAPK/ERK to promote cell growth and survival. These genetic occurrences lead to clonal proliferation of melanocytes that have restricted mitotic capabilities and therefore retain the benign nature of the lesion. Nonetheless, there are lesions known as atypical Spitz tumors or Spitz melanocytomas, which have intermediate characteristics, with unclear histology and partial capacity of malignant evolution. In general, the pathophysiology of Spitz nevi represents a range of the biologic behaviour, with either completely harmless lesions, or with atypical lesions, which demand close histopathologic and molecular evaluation.
Spitz nevi are benign melanocytic proliferations, and its mechanism of action is not quite clear. They develop within melanocytes, the pigment-producing cells of the skin and are believed to be a product of localized proliferation of melanocytes as a consequence of somatic mutations. Molecular analyses have verified a range of genetic mutations in Spitz nevi such as mutations in HRAS, BRAF, and NRAS, and a variety of kinase fusions (e.g., ALK, ROS1, NTRK1, BRAF fusions) implicating a spectrum of biologic behavior of benign to potentially atypical lesions. Such mutations are frequently not the same as those that are observed in typical melanocytic nevi or melanomas, thus providing a molecular distinction between Spitz nevi. Most Spitz nevi do not seem to have a significant environmental influence, or triggered by trauma or UV exposure, but there are few cases that could be related to inflammatory or proliferative stimuli.
Spitz nevi are generally benign with an excellent prognosis. Classic lesions in children are almost always harmless, while atypical Spitz tumors may have a slightly higher risk of local recurrence or, rarely, metastasis. The most important prognostic variables are the age of the patient, the size of the lesion, the histologic characteristics of diseases, including mitotic activity, depth of invasion, and cytologic atypia. Risk assessment can be also guided by molecular data, such as HRAS mutation or fusion of two kinases. Total excision and meticulous histopathologic review usually ensure a positive result.
Age group
Spitz tumors occur between 10 and 20 years of age, their distinction from melanoma is duly influenced by consideration of patient age.
Spitz nevi are generally isolated benign skin lesions and are not associated with systemic comorbidities or other medical conditions. They usually arise sporadically without links to syndromes, systemic diseases, or specific activities. Rarely, they may coexist with other melanocytic proliferations but without a consistent comorbidity pattern.
Spitz nevi typically present with a sudden onset of a solitary, dome-shaped papule or nodule that often grows rapidly over weeks to months before stabilizing. This relatively acute and noticeable growth phase can raise concern for malignancy, even though the lesion is benign.
Pyogenic granuloma
Melanocytic nevi
Spitz melanocytoma/atypical Spitz tumor
Malignant melanoma
Juvenile xanthogranuloma
The treatment paradigm for Spitz nevi centers on distinguishing them from spitzoid melanoma, since the two can appear similar clinically and histologically. In children, small and typical Spitz nevi may be observed with close clinical follow-up, especially when the diagnosis is clear. However, because of the diagnostic uncertainty, complete surgical excision with narrow margins is often recommended to allow for thorough histopathologic evaluation. In adults, excision is generally advised more consistently due to the higher risk of melanoma in this age group. Atypical Spitz tumors or lesions with concerning features may require wider excision, sentinel lymph node biopsy, or closer surveillance. Overall, management balances the benign nature of most Spitz nevi with the need to rule out melanoma and ensure patient safety.
Dermatology, General
Excisional biopsy:
First-line procedure for diagnosis and management.
Ensures complete removal of the lesion and provides tissue for histopathology.
Wide local excision (in atypical Spitz tumors or when melanoma cannot be excluded):
Wider margins are taken, similar to melanoma excision protocols, to reduce recurrence risk.
Sentinel lymph node biopsy (SLNB):
Considered in atypical Spitz tumors or spitzoid melanoma with worrisome histological features.
Helps assess regional nodal involvement, though its prognostic value in Spitz tumors is still debated.
Dermatology, General
Initial Evaluation The first phase involves careful clinical assessment of the lesion, including the patient’s age, history of onset, growth pattern, and any changes in appearance. Dermoscopy is often performed to look for characteristic patterns associated with Spitz nevi. Baseline photographs may also be taken to monitor the lesion over time.
Diagnostic Phase In most cases, an excisional biopsy with narrow margins is the preferred diagnostic step, as it allows for complete tissue sampling and accurate histopathological evaluation. Based on microscopic findings, lesions are classified as benign Spitz nevus, atypical Spitz tumor (Spitz melanocytoma), or spitzoid melanoma.
Definitive Management Management depends on the histological diagnosis. A benign Spitz nevus is generally cured with complete excision. Atypical Spitz tumors usually require wide local excision to ensure clear margins, and in selected cases, a sentinel lymph node biopsy may be considered. Spitzoid melanoma is treated according to standard melanoma protocols, which include wide excision, sentinel node evaluation, staging, and adjuvant therapy when appropriate.
Follow-Up Phase Ongoing follow-up is recommended for all patients, especially those with atypical lesions. This includes regular skin examinations to detect recurrence or new lesions, as well as patient education on self-monitoring and sun protection. In atypical or uncertain cases, closer surveillance is warranted given the variable biological behavior of these tumors.
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