However, as stated by allen and spitz 1 in 1953, in some circumstances, unequivocal distinction between spitz nevus and melanoma is practically impossible. I advise doing away with the term spitz nevus, using instead spitz tumor, with modifiers typical, atypical, or malignant, as appropriate. Desmoplastic spitz nevus desmoplastic spitz nevi reside on the opposite end of the spectrum from pscn reeds nevus, being composed mostly of epithelioid cells figure 3. The typical spitz nevus is a benign tumor that can be distinguished from melanoma in most instances. In the pediatric patients aged under 18 years with suspected spitzreed nevus by dermoscopy who had submitted to excision n28, histopathology confirmed the diagnosis without any prepubertal melanoma diagnosed, although 8 cases 828, 28. Clinical features of spitz nevi are wellcircumscribed, symmetrical, small to mediumsized firm papules with smooth discrete borders and a uniform color typically pink or flesh. Pdf histopathologic features of spitzoid lesions in different age. Our dermatology online brief report hhistopathological. It also may be called a nevus, spitz lesion, juvenile melanoma, benign juvenile melanoma, or spindle and epithelioid cell nevus of reed. It typically presents in childhood or adolescence as a sharply circumscribed, domeshaped, pinkred papule or plaque most commonly located on the face or lower extremities. Highly acclaimed and considered the leading reference in the field, pathology of melanocytic nevi and melanoma has once again been fully revised, updated, and expanded to reflect the most recent advances and techniques in the field of melanoma pathology.
Spitz nevus synonym spindle cell nevus, epithelioid cell nevus, benign juvenile melanoma 2. Its histopathological hallmark is the presence of large spindle andor epithelioid cells, usually in the paucity or ab sence of melanin. Table 2 outlines the prototypic histopathological findings in spitz nevi as well as. Fluorescence in situ hybridization for the differential. A spitz naevus or spitz s nevus is an uncommon type of mole melanocytic naevus. Among spitz nevi, histopathologic dis tinction between psn and rn. Keywords circumscribed pigmented lesions melanocytes prognostic factors spitz nevus and variants. Spitz nevus are noncancerous skin lesions that tend to be domeshaped, red, reddishbrown or dark colored.
The surface is commonly smooth, and the borders may fade into surrounding skin. Spitz nevus mostly occurs in children and young adults. They tend to grow quickly initially, but then stabilize or even disappear after a period of time. The spitz nevus, named after sophie spitz, who first described it in 1948 1,2. The full text of this article hosted at is unavailable due to technical difficulties. The most obvious aspect of symmetry is a comparison of each half of. Dec 07, 20 spitz nevus, also known as spitz tumor or spindle and epithelioid cell nevus, is an usually acquired, benign melanocytic lesion and one of the main differential diagnosis of melanoma. Spitz nevus can clinically present either in the classical reddish pink or the pigmented brownish black variant. Wt1 positive in melanoma and some dysplastic nevi, negative in other benign nevi, histopathology 2007. They usually develop on the face or limbs of young children. Blue nevus bn is a common specimen in routine pathology practice.
Spitz nevus with homerwright rosettelike structures. This is a pdf file of an unedited manuscript that has. She was able to identify and describe a separate class of benign melanocytic neoplasms in children that were previously diagnosed and treated as melanoma. However, it may resemble a malignant melanoma clinically and microscopically, so spitz naevi are often excised as a precaution.
Spitz nevus melanoma a typical spitzoid tumor melanoma of childhood fish. Difficulties in the diagnosis of spitzoid melanocytic lesions. Both classic and atypical spitz nevi are uncommon melanocytic lesions usually presenting in children and adolescents. Usually single but may be multiple and clustered agminate or multiple and disseminated. Benign spitz nevi are wellcircumscribed and symmetric melanocytic lesions comprising epithelioid andor spindled. A spitz nevus is a type of rare, noncancerous skin growth that typically impacts people under 35 years old. An evaluation of the current distinguishing histopathologic criteria noreen walsh, frcp c, frcpath, kerry croiry, frcpa, allan palmer, frcpa, and stanley mccarthy, ao, frcpa because of the wellknown difficulty in distinguishing between spitz nevi and spitzoid malignant melanomas at the microscopic level, the critical importance of this task. Within the last few years, sentinel lymph node biopsy slnb has been proposed as a useful tool in the management of melanocytic neoplasms of uncertain behavior, such as the atypical spitz nevus.
Clinical and histopathology features of spitz nevus. These were evaluated in terms of histopathology, immunohistochemistry, and molecular pathology using comparative genomic hybridization cgh and. Melanocytic nevi with features of spitz nevi and clarksdysplastic nevi sparks nevi. Pathology of melanocytic nevi and melanoma springerlink. New chapters on mucosal melanoma, ocular melanoma, and pigmented lesions of the nail apparatus have been included in this new edition. Though usually seen in children, spitz naevi may arise in adults. In the past decades, the recognition of variants of spitz naevus has resulted in a.
Spitz nevus genetic and rare diseases information center. Sophie spitz, who in 1948 reported a case series of. It is well known that spitz nevi may present with atypical dermoscopic patterns and that there are melanomas with spitzoid features. Pathology of desmoplastic spitz nevus dr sampurna roy md. Spitz naevus may pose great diagnostic difficulty due to clinical, dermatoscopic and histologic features resembling melanoma. Benign but may recur if incompletely excised am j surg pathol 2002. Jul 31, 20 dermatopathology reference describes nevus spilus congenital speckled lentiginous nevus histopathology including histologic features and provides links to additional medical references. The spitz nevus is a benign melanocytic lesion with clinical and histopathological features similar to those of melanoma.
Sabater marco v, escutia munoz b, morera faet a, roig mm, botella estrada r. Spitz nevi in the classic histopathological pattern lamb. The lesion usually is solitary and is encountered most commonly on the lower figure 1. Ever since it has been first described, it has been more frequently reported in children and young people, but recent studies show that its occurrence is not rare. A spitz naevus or spitzs nevus is an uncommon type of mole melanocytic naevus. Melanocytic nevus histologically melanocytic nevi are. Conflicts of interest atypical melanocytic proliferations. In the pediatric patients aged under 18 years with suspected spitz reed nevus by dermoscopy who had submitted to excision n28, histopathology confirmed the diagnosis without any prepubertal melanoma diagnosed, although 8 cases 828, 28. Braf and nras mutations in spitzoid melanocytic lesions. The histological distinction between spitz naevus spitz1948 and a small group of melanomas which resemble spitz naevus and which are therefore often. Clinical series and comparative molecular genetic studies support the concept that spitzoid lesions.
Histologically, spitz nevus may be junctional, compound or intradermal. Since its original description in 1948, the spitz nevus remains one of the most controversial and diagnostically challenging entities in dermatopathology. The lesions can be asymptomatic or have a history of rapid but limited growth. Pdf spitz and reed nevi, also called spindle andor epithelioid cell nevi, are a special. Spitz nevus, also known as epithelioid and spindle cell nevus, is an uncommon. State of the art, nomenclature, and points of consensus and. The typical or classic lesion is a pink papule or nodule, which appears suddenly and grows rapidly for a few months. Pathology of melanocytic nevi and melanoma is the essential reference for every practicing dermatopathologist, pathologist, dermatologist, and cancer research scientist today. The traditional dichotomous classification of spitz naevus versus spitzoid melanoma is challenged by a group of lesions that deviates from the prototypic spitz naevus.
Lesions are most commonly intradermal, but may rarely be compound. Soepronos textbook is available on and includes detailed information on over 600 entities and includes four dvd diskettes with highresolution images that provide a virtual dermatopathology reference and guide. Histopathology of spitz naevi and spitzoid melanomas. Chromatogram traces showing braf v600e mutations in a an atypical compound spitz nevus case 5. Common combinations is a compound melanocytic nevus and a blue nevus the epithelioid blue nevus is often part of a combined nevus that may include spitz nevus, desmoplastic nevus, or congenital nevus the melanocytes in blue nevi of all types express s100 protein, melana mart1, and hmb45.
As noted above, the term and concept of nevus in melanocytic lesions is a human contrivance, implying a malformation, as apposed to the localized, aberrant proliferations that they appear to be. It is likely that these lesions for which we lack specific diagnostic criteria represent. Spitz nevus, also known as epithelioid and spindle cell nevus, is an uncommon melanocytic lesion that can be difficult to differentiate from malignant melanoma. Dermatology and pathology, yale dermatopathology laboratory, 15. Spitz nevus, also known as spitz tumor or spindle and epithelioid cell nevus, is an usually acquired, benign melanocytic lesion and one of the main differential diagnosis of melanoma.
Soeprono teaches and practices dermatopathology at loma linda university, school of medicine, department of dermatology. The lesion was originally thought to occur largely in children, 1,2 but it is now well recognized that more than half of spitz nevus cases are detected in patients older than 14 years, 3 and about one fourth of the cases occur in patients older than 30 years. Junctional, compound or dermal nevus, composed of epithelioid melanocytes large, with broad, pink cytoplasm, sometimes multinuclear. Apr 30, 2016 in the pediatric patients aged under 18 years with suspected spitz reed nevus by dermoscopy who had submitted to excision n28, histopathology confirmed the diagnosis without any prepubertal melanoma diagnosed, although 8 cases 828, 28. Spitz nevus and other spitzoid tumors in children part 1.
A practical approach to the diagnosis of melanocytic lesions. In contrast, the atypical spitz nevus has an unknown clinical prognosis, and its clinical and histologic traits are loosely defined. A type of melanocytic nevus, it affects the epidermis and dermis it is also known as an epithelioid and spindlecell nevus, and misleadingly as a benign juvenile melanoma. Sophie spitz, who in 1948 reported a case series of melanomas of childhood. In 22 cases ali ghannadan, 1 kambiz kamyab, and mahtab rahbar 2, 1 associated professor of tehran medical university, tehran university of. Clinically, the tumour presents as a pink or flesh coloured papule or nodule, located on the face, trunk or extremities. An evaluation of the current distinguishing histopathologic criteria noreen walsh, frcp c, frcpath, kerry croiry, frcpa, allan palmer, frcpa, and stanley mccarthy, ao, frcpa because of the wellknown difficulty in distinguishing between spitz nevi and spitzoid malignant melanomas at the microscopic level, the critical importance. Spitz nevi can be very difficult to distinguish from melanoma, as a result treatment tends to involve excision of the nevi.
There is debate as to whether the pscnor is an entity in its own right or whether it is a variation of a spitz naevus. Cutaneous lesions the most common variety of spitz nevus is solitary, asymptomatic, pink or red, hairless, firm, and domeshaped. State of the art, nomenclature, and points of consensus. Spitz nevus sn and spitzoid malignant melanoma smm represent benign and. Desmoplastic spitz nevus has clinical and histopathological features that are difficult to differentiate from dermatofibroma and desmoplastic malignant melanoma dmm, which requires aggressive therapy for cure. Spitz nevus is a benign melanocytic lesion and also one of the main differential diagnosis of melanoma. The classic spitz nevus typically is benign and has characteristic clinical and histologic features. The name juvenile melanoma is generally no longer used as it is neither a melanoma, nor does it occur only in children. Spitz nevus or spitz tumor is an uncommon melanocytic lesion composed of large epithelioid andor spindled cells. Atypical spitz nevus, he 60x 440 atypical spitz nevus, he 60x 439 atypical spitz nevus, hmb45 20x 437 atypical spitz nevus, melan a 20x 438. Jul 26, 2017 a spitz nevus is a rare type of skin mole that usually affects young people and children. The pigmented spindle cell naevus of reed pscnor is a variant of a compound or occasionally a junctional naevus.
It was first described in 1948 but great controversy still remains today. Dermoscopy demonstrates that the pigmented variant is much more common than the classical variant. Recognized histological variants include the pigmented spindle cell reed nevus. Desmoplastic spitz nevus dsn is a rare variant of spitzoid tumour which was first described by reed et al. They most often occur on the extremities of adults, predominantly females, in their third decade 4. Pdf spitz nevus is one of the most difficult melanocytic lesions to diagnose in regard of malignancy, even for experienced dermatopathologists. Mar 24, 2018 combined melanocytic nevus microscopy. A spitz nevus is a rare type of skin mole that usually affects young people and children. The histological distinction between spitz naevus spitz 1948 and a small group of melanomas which resemble spitz naevus and which are therefore often referred to colloquially as spitzoid melanomas smith et al. Spitz nevi, atypical spitzoid neoplasms, and spitzoid melanoma.
Maintaining a uniform and benign cytology,cells diminish in size as the base is approached. Jun 23, 2006 chromatogram traces showing braf v600e mutations in a an atypical compound spitz nevus case 5. The washington manual of surgical pathology 1st ed. This report describes 22 spitz nevi that seemed to have been clinically removed but persisted and clinically recurred at the biopsy site. Jan 08, 2018 melanocytic nevi with features of spitz nevi and clarksdysplastic nevi sparks nevi. There is a pigmented variant the majority of spitz naevi are compound or. A spitz nevus is a melanocytic neoplasm formed of epithelioid or spindle cells described for the first time by darier and civatte. Pathology of spitz nevus, atypical spitz nevus, spitzoid. A junctional or compound lesion not fulfilling the histopathologic criteria of melanoma, the atypical spitz nevus asn has at least 1 of the following histopathologic. Although it can look like a serious form of skin cancer called melanoma, a spitz nevus lesion isnt. It is a group of nevus cells in the layers of the skin.
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