Nail Tumors Research Articles

Diagnosis and surgical treatment of benign nail unit tumors

Little is known about benign non-melanocytic nail tumors, probably due to their low pathogenicity. They are commonly misdiagnosed as inflammatory or infective diseases. They have various features, depending on the type of tumor and its location in the nail apparatus. The typical sign of a tumor is the presence of a mass and/or secondary nail changes from damaged nail structures. In particular, if a single digit is affected by a dystrophic sign or a symptom is reported without any explanation, the presence of a tumor should always be ruled out. Dermatoscopy helps to enhance visualization of the condition and in many cases supports the diagnosis. It may also assist in identifying the right place to biopsy, but it never replaces surgery. Most common non-melanocytic nail tumors are analyzed in this paper, including glomus tumor, exostosis, myxoid pseudocyst, acquired fibrokeratoma, onychopapilloma, onychomatricoma, superficial acral fibromyxoma and subungual keratoacanthoma. The aim of our study is to review the main clinical and dermatoscopic characteristics of the most common benign non-melanocytic nail tumors, to correlate them with the histopathology and to advise practitioners of the best surgical management.

Dermatoscopy of subungual digital fibromyxoma (superficial acral fibromyxoma)

A 28-year-old female patient with a 5-year history of a nonpainful, slow-growing mass on the second right distal aspect of the phalanx noticed a nonpainful, firm, pink, dome-shaped growth elevating the nail plate 1 year ago. She had a history of accidental trauma with acrylic nails 1 year before the nail tumor. X-ray scan and soft-tissue ultrasonography (ACUSON X700; Siemens) showed hypoechogenic dermal tissue that measured 0.8 × 0.4 × 0.6 cm3 (Fig 1). Using pocket DL200 Hybrid (DermLite) (magnification: ×20), a nonpigmented tumor, with a pink, homogeneous color, and longitudinal, soft-pink and small, shiny, white streaks perpendicular to the nail plate were observed. Below the nail plate, a layer of white, hyperkeratotic projections, with sparse red, brown, and gray dots that were immediately adjacent, was observed. No vascular structures were seen in this patient, which is different from other publications that have reported this tumor (Fig 2).1Sialitti S. Abdelmouttalib A. Senouci K. Meziane M. Subungual acral fibromyxoma: new dermoscopic features.Our Dermatol Online. 2021; 12: 1-2Crossref Google Scholar The dermatopathology report described laminar compact hyperkeratosis, regular acanthosis, and elongated papillary processes. Superficial-medium dermis, a neoformation with spindle cell proliferations in the myxoid stroma, and no atypia or necrosis were also observed. CD34-positive cell staining was compatible with superficial acral fibromyxoma.Key messageSuperficial acral fibromyxoma is a benign, slow-growing, periungual or subungual tumor that was first described by Fetsch et al in 2001, with approximately 314 cases reported.2Griffiths C. Barker J. Bleiker T. Chalmers R. Creamer D. Acquired disorders of the nails and nail unit.in: Rook's Textbook of Dermatology: Specific Cutaneous Structure. 9th ed. Wiley-Blackwell, 2016: 2501Crossref Google Scholar, 3Kutzer H. Kamino H. Reddy V. Pui J. Fibrous and fibrohistiocytic proliferations of the skin and tendons.in: Bologna J. Dermatology. 4th ed. Elsevier, 2018: 2071Google Scholar, 4Bostanci S. Akay B.N. Akkaya Z. et al.Superficial acral fibromyxoma.J Am Podiatr Med Assoc. 2021; 111: 17https://doi.org/10.7547/20-119Crossref Scopus (0) Google Scholar, 5Crepaldi B. Soares R. Silveira F. et al.Superficial acral fibromyxoma: literature review.Rev Bras Ortop. 2019; 54: 491-496Crossref PubMed Scopus (0) Google Scholar, 6Fetsch JF Laskin WB Miettinen M Superficial acral fibromyxoma: a clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes.Hum Pathol. 2001; 32: 704-714Crossref Scopus (187) Google Scholar Dermatoscopic findings contribute to underdiagnosed analysis of this tumor that is identified based on several patterns reported and facilitate its challenging diagnosis compared with that of other acral tumors, including myxoid variations. Our dermatoscopy case report shows a digital fibromyxoma with no capillary patterns observed in the myxoid areas in a female patient with a history of acrylic nail trauma, and histopathology confirmed this diagnosis. No reports secondary to gel polish andsubungual acral fibromyxoma were found. Superficial acral fibromyxoma is a benign, slow-growing, periungual or subungual tumor that was first described by Fetsch et al in 2001, with approximately 314 cases reported.2Griffiths C. Barker J. Bleiker T. Chalmers R. Creamer D. Acquired disorders of the nails and nail unit.in: Rook's Textbook of Dermatology: Specific Cutaneous Structure. 9th ed. Wiley-Blackwell, 2016: 2501Crossref Google Scholar, 3Kutzer H. Kamino H. Reddy V. Pui J. Fibrous and fibrohistiocytic proliferations of the skin and tendons.in: Bologna J. Dermatology. 4th ed. Elsevier, 2018: 2071Google Scholar, 4Bostanci S. Akay B.N. Akkaya Z. et al.Superficial acral fibromyxoma.J Am Podiatr Med Assoc. 2021; 111: 17https://doi.org/10.7547/20-119Crossref Scopus (0) Google Scholar, 5Crepaldi B. Soares R. Silveira F. et al.Superficial acral fibromyxoma: literature review.Rev Bras Ortop. 2019; 54: 491-496Crossref PubMed Scopus (0) Google Scholar, 6Fetsch JF Laskin WB Miettinen M Superficial acral fibromyxoma: a clinicopathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes.Hum Pathol. 2001; 32: 704-714Crossref Scopus (187) Google Scholar Dermatoscopic findings contribute to underdiagnosed analysis of this tumor that is identified based on several patterns reported and facilitate its challenging diagnosis compared with that of other acral tumors, including myxoid variations. Our dermatoscopy case report shows a digital fibromyxoma with no capillary patterns observed in the myxoid areas in a female patient with a history of acrylic nail trauma, and histopathology confirmed this diagnosis. No reports secondary to gel polish andsubungual acral fibromyxoma were found. None disclosed.

High-risk mucosal human papillomavirus-associated squamous cell carcinoma of the fingers presents distinctive clinicopathological characteristics and outcomes: a bicentric retrospective study.

High-risk mucosal human papillomavirus (HR-HPV) seems to play a role in cutaneous squamous cell carcinoma (cSCC), particularly in nail tumours, where genitodigital transmission has been suggested. The role of HR-HPV in nonungual cSCC of the finger needs to be clarified. To evaluate the prevalence, clinicopathological characteristics, surrogates and outcomes of HR-HPV in cSCC of the finger. This was an observational bicentric study including patients with an excised in situ or invasive cSCC located on the finger. Differences in HR-HPV and non-HR-HPV tumours were evaluated. Forty-five patients (45 tumours) were included. HR-HPV was detected in 33% of cases (22% HPV type 16). The mean age was lower in patients with HR-HPV than in those with non-HR-HPV (62·4 vs. 81·1 years, P = 0·001). HR-HPV tumours were smaller (10 mm vs. 15 mm, P = 0·07) and more frequently intraepidermal (60% vs. 20%, P = 0·004). The absence of elastosis (P = 0·030) and inflammation (P = 0·026) and the presence of basaloid morphology (P = 0·003) were surrogates of HR-HPV detection. Mean p16 positivity was 61% in HR-HPV and 36% in non-HR-HPV tumours (P = 0·061). Recurrence after surgery was more common in HR-HPV tumours (58% vs. 34%), although this was not statistically significant. HR-HPV was detected in 27% of the nonungual tumours. HR-HPV-associated cSCC of the finger appears in younger patients, is smaller and is less infiltrative than non-HR-HPV tumours. The presence of a basaloid morphology and the absence of elastosis and inflammation could be used as markers for HR-HPV detection. The high prevalence of HR-HPV in nonungual cSCC suggests its aetiopathogenic role in these tumours.

Nail surgery: the effect of clinical and psychological characteristics on post-surgical quality of life and pain severity

Nail disease and nail surgery are commonly perceived as painful and distressful experiences that can significantly affect patient health-related quality of life. A comprehensive management of patients undergoing dermatological surgery could improve their emotional experience, influence quality of life and, hopefully, improve their adaptation to the intervention. This study aimed to investigate whether patient psychological characteristics may relate to nail intervention procedures and influence post-surgical quality of life and pain severity. This prospective observational monocentric cohort study was conducted in the Dermatology Nail Unit of Bologna. Patient candidates for nail surgery, meeting the inclusion criteria, were invited to participate in the study which consisted of completion of a self-report questionnaire before (Time 1) and one month after (Time 2) surgery. Nail surgery was performed for 102 patients: 46 underwent highly invasive and 56 mildly invasive nail procedures. In total, 46 nail tumours were excised which were shown to be benign in 36 cases and malignant in 10 cases. Patients with a high level of pain sensitivity and trait of anxiety reported significantly lower levels of quality of life and high levels of pain intensity one month after surgery (p < 0.001). Our findings highlight the need for clinicians to consider the psychological characteristics of patients following dermatological surgery care in order to balance the negative impact of trait anxiety, pain sensitivity and negative treatment expectations regarding post-surgery quality of life and pain severity.

Onicopatías

Nail disorders are a common reason for dermatologic consultation. Nails may show different findings that can arise from nail unit diseases, dermatoses or systemic diseases. Several techniques are used for nail diagnosis, such as dermoscopy, ultrasound or other imaging techniques, microbiological tests, and histological analysis. This article review the features, diagnosis and treatment of different nail disorders, including nail semiology, inflammatory dermatoses, infections, systemic diseases and nail tumors.

Radiological Imaging of Nail Disorders (PART II) - Radiological Features of Nail Disease.

We have seen that radiological techniques like digital x-ray, high-frequency ultrasonography (USG), computed tomography (CT), and magnetic resonance imaging (MRI) have their unique roles in assessing a complex anatomical structure like the nail unit. Broadly speaking, USG and MRI help evaluate soft tissue components well; while, radiographs and CT scans help assess bony lesions better. In the second part of this review, salient radiological features of various nail disorders, as seen on these modalities are detailed. The radiological features mostly play a supportive role and help rule out differential diagnoses. However, in some diseases like retronychia and some nail tumors, radiological findings help clinch the diagnosis. The diagnostic features as well as the investigative modality of choice for a particular disease are highlighted based on the best level of evidence (LoE) available. This narrative review includes both infectious and non-infectious nail unit diseases, with special emphasis on nail unit tumors.

Common diseases of the nail: Diagnosis and therapy.

This review focuses on nail changes that do not belong to the group of benign or malignant nail tumors. These common afflictions of the nail include structural changes in and under the nail plate as well as inflammation around and in the nail bed. They include onychomycoses, nail psoriasis, onychodystrophies, subungual hematoma, paronychia, ingrown nails and pincer nails. Due to the peculiar anatomy and physiological growth conditions of the nail, the pathology does not necessarily stem from the site of the clinical problem and calls for careful inspection and interpretation.

Painful nails: A practical approach to the diagnosis and management of painful nail conditions.

Because several nail disorders share similar clinical features, their diagnosis and management can be challenging to clinicians. The physical examination may disclose localized abnormalities or point to an underlying systemic disease, requiring additional workup. Furthermore, cosmetic distress and nail-related symptoms (e.g., tingling, stinging, numbness, and pain) are common factors that influence the patient's search for medical assistance. Nail pain (i.e., onychalgia) can accompany both localized and systemic pathology. Onychalgia can be acute or chronic according to the time of evolution; patients may describe it as intermittent or constant, and as a throbbing, burning, sharp, or shooting sensation denoting the nature of the pain. It may be exacerbated by colder temperatures,touch,and increased activity (e.g., manipulating objects, walking). We present four main groups of conditions that might cause nail pain: nail tumors, nail deformities, inflammatory or infectious diseases, and external or traumatic agents. Our article includes an overview of the clinical features, as well as diagnosis and management pearls for each entity. Physicians (dermatologists and nondermatologists) should be aware that abnormalities of the ungual and subungual space are not exclusive of dermatological disorders but may also be present in noncutaneous contexts.

Dermoscopic Evaluation of Inflammatory Nail Disorders and Their Mimics.

Nail dermoscopy (onychoscopy) is a valuable diagnostic tool for evaluating diseases in the nail apparatus. It is non-invasive, allowing clinicians to prioritize particular nails for biopsy. Thus, it can improve diagnostic accuracy and expedite treatment. Evaluating inflammatory nail disorders using onychoscopy is a relatively new approach to clinical assessment and has the potential to augment clinical care. This review highlights key dermoscopic features of major inflammatory nail disorders, including trachyonychia, nail psoriasis, nail lichen planus, onychotillomania, nail lichen striatus and allergic contact dermatitis due to artificial nails. It also illustrates their management and differential diagnoses, including onychomycosis, onycholysis, nail dystrophy due to systemic amyloidosis and malignant nail tumours. Limitations of this review included the low amount of literature on this topic and non-standardized terminology used among researchers. As onychoscopy is a relatively new technique, further studies and standardization of terminology are warranted to consolidate the role of dermoscopy in evaluating inflammatory nail disorders.

Ultrasound features of the subungual glomus tumor and squamous cell carcinomas.

Nail tumors often pose a significant challenge for the clinician, as they are burdened by a high delay in diagnosis and paucity of clinical signs in the early stage. In most cases, the onset of pain or nail plate dystrophy leads to imaging investigations such as plain radiography, magnetic resonance imaging, and ultrasonography (US) when surgery is pending. A 2-year monocentric study evaluated the histologically confirmed cases of subungual glomus tumor (GT) and squamous cell carcinoma (SCC) with a pre-surgical US assessment, and reviewed their sonography features. A total of 7 digital GTs and 6 SCCs of the nail were collected. GT and SCC are easily distinguishable on US. Compared to SCC, GT was positively associated with an ovoid shape, well-defined borders, bone cup-scalloping without cortical erosion, and a rich intralesional vascular pattern (P < 0.05). Vice versa, SCC was significantly correlated with irregular shape, ill-circumscribed margins, an infiltrative growth pattern with peripheral hypoechoic foci, posterior acoustic shadow, cortical erosion, and a highly vascular multipolar peripheral pattern (P < 0.05). The rapid assessment of GT and SCC sonographic features may contribute to reduce diagnostic delay, expedite management, and decrease the risk of surgical relapse.

Dermoscopic manifestations of nail diseases

Nail dermoscopy in recent years has become an auxiliary noninvasive tool for the diagnosis of nail diseases. It detects the differentiating characteristics of the nail units and assesses in the management of nail diseases. Dermoscopy may not be a diagnostic tool for all nail diseases; however, it is very useful for early observation with high magnification. This article discusses the important and common dermoscopic manifestations of nail disease cases in the daily practice. Currently, the indications of dermoscopy include viral, bacterial, fungal, inflammatory, pigmented, traumatic nail diseases, nail tumors, and connective tissue disease.

Diagnosis and treatment of malignant nail tumors

Diagnosis and treatment of malignant nail tumors

Squamous cell carcinoma of the nail unit.

Squamous cell carcinoma (SCC) of the nail apparatus is a rare malignant tumor that usually originates underneath the nail plate and grows slowly with possible bone invasion. The etiology remains unknown, although a strong association with different conditions, such as high-risk human papillomavirus (HPVs), trauma, or radiation exposure has been demonstrated. Nail SCC is called “the great mimicker nail tumor” because different clinical presentations may coexist, resembling benign or malignant nail lesions. For this reason, there is often a significant delay between the onset of nail SCC and the diagnosis. Clinical manifestation includes onycholysis and erythema, while in the advanced stages nail ulceration can be observed. The association with pain, swelling, and inflammation usually indicates an invasive SCC with involvement of the underlying bone. Metastasis is rare but possible with involvement of lymph nodes. A multidisciplinary approach to assessment, management, and follow-up is advised. Using careful examination and modern diagnostic methods, including onychoscopy, biopsy, and histopathology, will help identify SCC and prevent the invasive progression. X-ray is important to investigate the bone invasion to determine the best surgical approach that will have satisfying cosmetic and functional outcomes. Nevertheless, local excision with sufficient surgical margins, best if using Mohs surgery, is usually sufficient and superior to amputation of the distal phalanx. This review aims to highlight the correct approach in suspected SCC of the nail unit.

Mohs micrographic surgery for nail unit tumours: an update and a critical review of the literature.

Mohs micrographic surgery (MMS) is a good treatment option for epithelial neoplasms, especially when localized in areas where tissue conservation is crucial, such as the nail unit (NU). MMS is a method of radical excision offering high cure rates due to the margin control and functional preservation. Our aim is to provide a review on the use of MMS for the treatment of the most common nail tumours. We revised the current literature on the use of MMS to treat malignant neoplasms (Bowen's disease, squamous cell carcinoma, melanoma, basal cell carcinoma, keratoacanthoma, carcinoma cuniculatum) and benign neoplasms (onychomatricoma and glomus tumour). MMS represents a successful surgical option for nail tumours, firstly in terms of tissue conservation: the NU anatomy is complex and the preservation of the component structures is imperative for its functionality. Secondly, due to the surgical radicality, which is essential not only for the clearing of malignant tumours, but also for benign cases, in order to reduce recurrences. Although a conservative treatment of NU melanoma with MMS has been proposed, in our experience, the conservative approach with functional surgery is a good option for the treatment of non-invasive melanoma (in situ and Ia).

Surgical management of onychopapilloma, onychomatricoma, and subungual osteochondroma: Case series

The clinical diagnosis of benign and malignant nail tumors can be difficult. Dermoscopy can provide a clue to the diagnosis but nail biopsy is the gold standard in establishing the diagnosis. Here, we report three cases of rare nail tumors, that is, onychopapilloma, onychomatricoma, and subungual osteochondroma, which were diagnosed on histopathology and managed surgically.

Nail Biopsy: A User's Manual.

Nail biopsy is a procedure not routinely resorted to; but when indicated, it is often the only clue left for diagnosis. At such times, it pays to be conversant with it. It is an investigation that not only provides etiologic, diagnostic, and prognostic information but also aids in understanding the pathogenesis of nail diseases. It can be of therapeutic value, especially with respect to nail tumors. This article compiles the procedural techniques for nail biopsy of various types and attempts to summarize the evidence available in the literature. The objective of nail biopsy is to clinch a precise diagnosis of nail pathology with a simple and safe surgical procedure, avoiding pain or permanent nail damage. Patient selection is of utmost importance, wherein, the patient does not have typical skin lesions, yields inadequate information on routine nail investigations, and has no peripheral vascular compromise. The patient needs to be explained about the risks associated, the expected functional handicap, the time required for regrowth, a possibility of permanent nail dystrophy, and a possibility of not achieving a diagnosis even after the biopsy. Techniques and types of various nail biopsies are being discussed in this article. The specimen could be collected as an excision biopsy, punch biopsy, shave biopsy, or longitudinal biopsy. The trick lies in choosing the appropriate area for biopsy. Various biopsy types discussed in this article include nail plate biopsy (easiest and least scarring); nail bed biopsy (elliptical excision or punch); nail matrix biopsy (elliptical excision, punch excision (≤3 mm) or tangential/shave excision); and nail fold biopsy. Complications reported along with means to minimize them are also discussed.

Nail unit ultrasound: a complete guide of the nail diseases.

The nails have a functional and esthetic importance for patients. Almost always, the nail disorders are diagnosed on the basis of clinical findings, but imaging methods may be required for a better assessment. These imaging methods, such as ultrasound and magnetic resonance, may help to establish an accurate diagnosis. Magnetic resonance imaging is not widely available and sometimes may be very expensive; that is why, ultrasound is an excellent imaging modality. Our objective is to expose the nail unit anatomy, the nail unit anatomy in ultrasound, and some of the frequent pathologies found in our daily practice. A review of the literature was done to review the anatomy, technical aspects, and different findings in normal and abnormal nail unit ultrasound. Ultrasound offers an appropriate alternative for the evaluation of the nail unit, allows a real-time evaluation of each one of the components of the nail unit with an optimal visualization of these structures, and allows the evaluation of the thickness of the components, the vascularity, and blood flow by Doppler application. In addition, the nail unit disorder, such as infectious diseases, inflammatory and rheumatologic conditions, nail tumors, among others, may be assessed, not only in the diagnosis but also in the follow-up. Pre-surgical evaluation, surgical follow-up, and some procedures, such as biopsies, may be done by this technique. Ultrasound is an excellent technique for evaluation of normal anatomy, diagnosis, and follow-up of patients with nail unit diseases. This is an alternative for other imaging methods and may be used for an accurate diagnosis approach.

Important malignant and new nail tumors.

The nail apparatus is an integral part of the functional unit of the digital tip. Although overall uncommon, all cells and tissues occurring in this area can give rise to neoplastic lesions. Given the special anatomical location, such tumors frequently show morphological and symptom-related differences compared to similar lesions located elsewhere on the skin. Though particularly threatening, there is often a substantial delay in the diagnosis of Bowen's disease, ungual squamous cell carcinoma, and melanoma. Nevertheless, local excision with sufficient surgical margins is usually sufficient and superior to amputation of the distal phalanx. In recent years, a number of nail-specific tumors have been described. Tumors such as onychomatricoma, onychocytic matricoma, onychocytic carcinoma, and onychopapilloma originate from the nail matrix. Onycholemmal cysts, onycholemmal horn, and the proliferating onycholemmal tumor are characteristic nail bed tumors. Onycholemmal carcinoma is a slowly growing low-grade malignancy. Using modern diagnostic methods, careful examination - including biopsy and histopathology - of nail changes not responding to conservative treatment will help identify nail-specific neoplasms and prevent the progression of malignant nail tumors.

Nail neoplasms.

Nail neoplasms include all tumors occurring in the nail or periungual apparatus tissue. While some nail tumors can be similar to tumors located on the skin, others are unique. Both benign and malignant lesions can affect the nail apparatus. In particular, early malignant tumors like melanoma and squamous cell carcinoma can present similarly to onychomycosis or benign melanonychia and frequently missed by clinicians. Therefore, physicians should be aware of nail structures and the characteristics of nail tumors. Our review covers the normal nail structure and the most common nail tumors from benign to malignant.

Intraoperative dermoscopic features of onychomatricoma: a review of 10 cases.

Amelanotic nail tumours are difficult to diagnose. Dermoscopy is an accessible tool successfully used in diagnosis of amelanotic or melanotic skin tumours. We have previously shown the usefulness of dermoscopy in the preoperative diagnosis of onychomatricoma (OM). In this study, we completed this work by identifying additional intraoperative criteria to better establish the initial diagnosis of this tumour. Evaluation of intraoperative dermoscopy in a small series of OM cases in order to define relevant diagnostic criteria. In total, 10 patients with OM diagnosed in our centre were enrolled in the study. Six trained dermoscopists individually evaluated each criterion, then the data were compared and a consensus reached after discussion between the observers. For each criterion, we analysed its frequency and its interobserver accordance. We defined three architectural criteria (the 'Sagrada Familia' sign, digitations and the 'mirror sign'), and three vascular criteria (sagittal vessels, dotted vessels and irregular vessels). The Sagrada Familia sign, digitations and mirror sign were found in 100%, 90% and 70% of the cases, respectively, with high interobserver agreement. The vascular criteria were less regularly observed: sagittal, dotted and irregular vessels were respectively found in 80%, 70% and 50% of the OM cases, and were more difficult to assess, as shown by the lower interobserver agreement rates. Intraoperative dermoscopy of the nail matrix and bed offers useful information for the diagnosis and management of OM. Larger comparative studies should be performed to evaluate the true benefit of this approach.