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Amelanotic Melanoma Jürgen Kreusch IV.3

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Amelanotic Melanoma Jürgen Kreusch IV.3 Chapter IV.3 Amelanotic Melanoma Jürgen Kreusch IV.3 Contents IV.3.1 Introduction and Definition. 204 IV.3.2 Hypomelanotic and Amelanotic Melanoma. 204 IV.3 IV.3.4 Clinical Features. 205 IV.3.5 Dermoscopic Criteria. 207 IV.3.6 Adequate Dermoscopic Inspection of Non-pigmented Lesions . 208 IV.3.7 Dermoscopic Features of Amelanotic Melanoma . 208 IV.3.8 Vascular Patterns Fig. IV.3.1. Amelanotic melanoma (center; SSM, L III, in Amelanotic Melanoma. 208 0.6 mm) IV.3.9 Morphological Changes of Vessels During Tumor Growth. 209 summarized under this general term; however, IV.3.10 Relevant Clinical Differential amelanotic melanoma will not be identified by Diagnosis. 210 dermoscopy nor by any other diagnostic meth- IV.3.10.1 The Way to Diagnosis od if a lesion is not considered worth inspection of Amelanotic Melanoma . 210 with the particular instrument – a screening IV.3.10.2 Strategies for Detecting Amelanotic strategy including knowledge of clinical fea- Melanoma. 211 tures, dermoscopic techniques and criteria is References. 212 ­essential to spot amelanotic melanoma among the variety of non-pigmented lesions of the skin (Figs. IV.3.1, IV.3.5a). IV.3.1 Introduction and Definition IV.3.2 Hypomelanotic and Amelanotic Melanoma “Amelanotic melanoma” is a clinical and de- scriptive term frequently used for any melano- Frequently, melanoma are classified “amelanot- ma lacking melanin pigmentation. These tu- ic” regardless of the degree and nature of hy- mors represent a large fraction of the so-called popigmentation. One must distinguish two rea- “featureless” or “undiagnosable” melanoma [3– sons for a melanoma to contain little melanin. 5, 7, 13]. This chapter gives details on the der- There are tumors which produce little or no moscopic features of amelanotic melanoma melanin. On the other hand, there are melano- which may help to classify the variety of tumors ma which have lost more or less of their melanin Chapter IV.3 Amelanotic Melanoma Chapter IV.3 205 content mostly due to regression. Both phenom- melanin in all segments warrants description as Amelanotic Melanoma ena may even occur within the same lesion. The “slightly pigmented melanoma.” Lesions with Jürgen Kreusch IV.3 varied reasons for hypopigmentation should circumscribed hypopigmentation may be char- find their equivalent in clinical and, more im- acterized as “partially hypomelanotic” or “par- portantly, in dermoscopic terminology. tially amelanotic” melanoma (Table IV.3.1). There exist tumors with great differences in melanin content, be it the proportion of the hy- IV.3.4 Clinical Features popigmented area in partially pigmented mela- noma or the intensity of pigmentation within a Completely amelanotic melanoma are quite slightly pigmented melanoma. As a proposal, a rare: estimates between 2 and 8% are given [14]. melanoma may be considered “partially pig- In general, the descriptive clinical term “amela- mented” if the section containing melanin is no notic” melanoma is used imprecisely, as it is fre- larger than 30% of the tumoral area. A melano- quently attributed to a wide variety of tumors ma should be described as “slightly pigmented” with a broad spectrum of intensity and variable if the intensity of pigmentation is an estimated distribution of their pigmentation. Hypopig- 30% of a “regular” dark-brown tumor as judged mentation may refer to the entire lesion or just by visual inspection. Colorimetric and quanti- to a segment of the tumor, and may develop or tative measurements of these details appear de- change with time [8]. One should differentiate sirable for classification of melanoma for scien- between tumors lacking melanin just in circum- tific purposes. scribed segments, and other ones which are of Regressive melanoma may best be spotted by homogenous structure and contain little mela- means of grayish or whitish areas, the white ar- nin within the entire lesion. The extreme vari- eas being whiter than the neighboring skin. Re- ant of the latter are completely “amelanotic” ports of the patient that a dark spot or nodule melanoma. A lesion containing a low amount of has become paler may be helpful. Flow-chart IV.3. For classifying hypopigmented melanoma and diagnosing amelanotic melanoma 206 J. Kreusch Table IV.3.1. Phenotypes of hypomelanotic and amelanotic melanoma Description of lesion Category of melanoma Melanoma not producing Amelanotic melanoma any trace of melanin (sensu strictu) Melanoma consisting Hypopigmented melanoma exclusively of a cell line with low production of melanin IV.3 Melanoma consisting Partially pigmented melanoma of a section (mostly a nodule) with no or little melanin content within a pigmented tumor Hypomelanotic melanoma Partially pigmented melanoma with amelanotic components Amelanotic / hypomelanotic Partially pigmented melanoma melanoma associated to junctional or compound nevus Amelanotic Melanoma Chapter IV.3 207 Table IV.3.1. (continued) Description of lesion Category of melanoma Partially regressive melanoma Partially pigmented melanoma associated to dermal nevus Amelanotic cell line/metastasis No images available in regressive melanoma Collision tumors of amelanotic No images available melanoma and non-pigmented lesions (e.g. BCC, seborrheic keratosis) In order to complete the listing of hypopig- It is therefore suggested to follow the pathway mented variants of melanoma, collision tumors given in this chapter to distinguish the two ba- of a regressive or amelanotic melanoma (or a sic types of hypomelanotic melanoma before melanoma metastasis) next to or within a poor- analyzing the morphology any further. ly pigmented benign lesion, e.g., a dermal nevus Loss of melanin due to regression can be rec- or a seborrheic keratosis, might be taken for an ognized easily in almost any case, as it is indi- amelanotic melanoma despite the fact that the cated by the presence of melanophages visible as components are of different origin and biologi- tiny grayish granules of approximately 0.01– cal behavior. Such cases of collision tumors are 0.02 mm diameter (“peppering”), or by the pres- somewhat hypothetical and certainly very rare. ence of gray-blue veils. Progress of regression They are not dealt with any further here. results in disappearance of grayish areas and melanophages, leaving behind whitish fibrous tissue resembling a scar. These lesions should be IV.3.5 Dermoscopic Criteria excluded from the suspicion of amelanotic mel- anoma. They show differences in vasculariza- There are few reports in the literature which tion as compared with amelanotic melanoma. describe dermoscopic features of truly amela- Truly amelanotic melanoma do not contain notic melanoma [1, 2, 6, 12, 14, 15]. The diag- any melanin; thus, all features related to details nostic importance of vascular structures is of pigmented lesions must be absent. Size, asym- stressed in each contribution. In several papers metry, irregular border, and ulceration are fea- cases of hypomelanotic melanoma due to re- tures of secondary diagnostic importance as gression are viewed jointly with completely they are not restricted to melanocytic tumors; amelanotic melanoma; however, intermingling however, as vascular structures are easily visible, melanoma with different origin of hypopig- amelanotic melanoma can be diagnosed with a mentation in morphological studies will result dermoscope, provided they are inspected prop- in loss of sensitivity and specificity of features. erly. 208 J. Kreusch Theoretically, there might exist melanoma amelanotic melanoma. If reddish or pink areas consisting of a regressive section joint with an are observed, the magnification might be too amelanotic component (and even in collision low to distinguish terminal capillaries, or the with further hypopigmented tumors such as vessels might be out of focus; therefore, instru- seborrheic keratoses or basal cell carcinoma). ments permitting focus control are quite helpful They must be identified by stepwise analysis of for better analysis of vascular structures. The the various components and certainly are a only features visible dermoscopically are vessels challenge even for the experienced dermosco- and whitish structures which resemble pseudo- pist. horn cysts (Fig. IV.3.5b); the latter are somewhat misleading, as they might be taken as signs of seborrheic keratoses, basal cell carcinoma, or IV.3.6 Adequate Dermoscopic Inspec- ordinary nevi. They are present in melanoma of tion of Non-pigmented Lesions several millimeters in vertical diameter. In his- tological sections they revealed mucoid degen- Instruments with glass front plate must be set eration and no keratin (unpublished observa- onto the tumoral surface very carefully in order tions); thus, the remaining features of diagnostic to avoid compression of the soft vascular struc- importance are vascular patterns. IV.3 tures and squeezing out the blood, rendering them invisible. Ultrasound gel is recommended as a contact medium due to its low viscosity and IV.3.8 Vascular Patterns non-volatility. This helps to inspect the vessels in Amelanotic Melanoma with as little pressure as possible. Use of instru- ments with polarized light may serve better if The vascular patterns in amelanotic melanoma the light source emits an appropriate spectrum have been described as pinpoint, point-like, dot- of wavelengths for visualizing reddish struc- ted vessels, and as hairpin, loop-like, linear ir- tures such as blood vessels and the magnifica- regular vessels. In principle, all point-like or tion permits viewing small capillaries of 10 µm looped
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