Nevus spitz D22.-

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

acute nevus; Allen Spitz Naevus; Allen-Spitz-Naevus; benignes; Epithelial cell nevus; juvenile benign melanoma; juveniles; Melanoma; melanoma benignes juveniles; Naevus Allen-Spitz; Nevus of large spindle-and/or epitheloid cells; Nevus Spitz; Pointed tumor; Sharp Nevus; Sharps Nevus; Spike Nevus; Spindle cell nevus; Spitz-Naevus; Spitz tumor

History
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Spitz, 1948

Definition
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Benigner, histologically a tumor reminding of a malignant melanoma with a clinically very variable picture.

Manifestation
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Mainly occurring in children (3 to 8 years of age), rarely in adults.

Localization
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Mainly occurring on the face, also on the capillitium, neck and legs.

Clinical features
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  • Mostly solitary (sporadic reports of multiple occurrence), sharply defined, 0.5 mm to 2.0 cm large, hemispherically bulging, round or oval, with a smooth, shiny surface, light brown-reddish to blue-black nodule (or papule) interspersed with telangiectases. The consistency is elastic to rough.
  • Diascopic: Yellowish-brownish intrinsic infiltrate. Typical is the indication of rapid growth (within a few weeks!).
  • Incident light microscopy: Characteristic incident light features of the Spitz nevi include zonal, cocard-like or radial-strial basic architectures, annular or centro-ellipsoidally arranged areas with grey-black centropapillary globules or brown/black dots, isolated or wreath-like peripheral grey-blue pseudopodia, perivascular melanophages and inverse reticular patterns especially in epithelial cell nevi. In contrast to malignant melanomas, vascular polymorphies are extremely rare, except in desmoplastic Spitz nevus, aneurysmatic vascular sacculation and severe caliber fluctuations as well as microscopic blood lakes. In some cases, perivascular melanophagal agglomerations and bluish-grey pigment rings appear as internal mesh boundaries.

Histology
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  • Melanocytic nevus with 3 developmental phases (as with other melanocytic nevi):
    • Naevus Spitz - Junction type
    • Naevus Spitz - Compound type
    • Naevus Spitz - dermal type.
  • Tumours are usually symmetrical and sharply defined. Hyperplastic epidermis with orthokeratosis, hyperkeratosis, hypergranulose and acanthosis. Typical cleavage between melanocyte nests and surrounding keratinocytes. Cell polymorphies, mitoses and fish-like vertebrae of spindle cells are visible. Also epithelioid melanocytes with broad, homogeneous, eosinophilic cytoplasm are not rare. Multinucleated giant cells may occur. Mostly low pigmentation, but in individual cases massive. No destruction of the collagen fibres. Mitoses are found in the upper parts of the tumour, but not in the depth (important distinguishing feature from malignant melanoma). A typical feature are homogeneous, PAS-positive, light red globules (according to the first descriptor: kamino-bodies). The most important phenomenon of the Spitz nevus in demarcation to the malignant melanoma is the maturation of the melanocytes towards depth (as with other melanocytic nevi).
  • Histological variants:
    • Pigmented spindle cell nevus (reed): Here a uniform, strongly pigmented, spindle cell race predominates.
    • Pigmented epithelioid cell nevus: Composed of nests of highly pigmented, large epithelioid cells.
    • Granulomatous Spitz nevus: Predominantly dermal type. When magnified, the examiner is reminded of a sarcoid reaction.
    • Plexiform Spitz nevus: Plexiform arrangement of predominantly spindle and epithelioid melanocytes.
    • Desmoplastic Spitz nevus: Variant of a dermal Spitz nevus in which usually a few epithelioid melanocyte nests are located in a fibrosed dermis with broad, tangled collagenous fibre strands.

Therapy
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Excision in healthy individuals without a safety margin.

Progression/forecast
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Cheap.

Tables
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Feature

Specificity [%]

Sensitivity [%]

Basic radial striate structure

99

68

Zone or co-cardioid structure

97

5

Isolated or ring-shaped peripheral grey-blue pseudopodia

97

18

Annular or centroelastic areas with grey-black centropapillary globules

89

25

Symmetrical structure

80

88

Perivasal melanophages (especially epithelial cell nevi)

n.a.

n.a.

Inverted Nez pattern (especially epithelioid cell nevi)

n.a.

n.a.

Note(s)
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The pigmented spindle cell nevus (reed) is considered a variant of the Spitz nevus. The diagnosis of Spitz nevus in adults must be evaluated with a critical distance. In case of doubt this histological evaluation is to be classified as malignant.

Literature
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  1. Ackerman AB, Maize JC (1987) Pigmented lesions of the skin. Lea & Febiger, Philadelphia
  2. Ackerman AB (1990) A dermatopathologist`s guide to melanocytic nevi and malignant melanomas. In: Conley I (ed) Melanoma of the head and neck. Thieme, Stuttgart New York, pp 9-33
  3. Borsa S et al (1991) Bubble-cell variant of the Spitz nevus. Dermatologist 42: 707-708
  4. Clarke B et al (2002) Plexiform acute nevus. Int J Surg Pathol 10: 69-73
  5. Gartmann H et al (1985) The Spitz nevus - A clinical and histological analysis of 652 tumours. Z Hautkr 60: 22-42
  6. Hoang MP (2003) Myxoid Spitz nevus. J Cutan catholic 30: 566-568
  7. Reynolds N et al (2003) Nodal Metastatic Melanoma in the Neck of a 4-years old girl after diagnosis of Spitz nevus of the cheek. Ann Plast Surgery 50: 555-557
  8. Schulz H (1996) Differential diagnosis of Spitznaevi. Microscopic differentiation from malignant melanomas. TW Dermatol 26: 331-335
  9. Spitz S (1948) Melanomas of childhood. Am J Pathol 24: 591-602
  10. Zaenglein AL et al (2002) Congenital Spitz nevus clinically mimicking melanoma. J Am Acad Dermatol 47: 441-444

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Last updated on: 29.10.2020