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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
IMAGES IN CLINICAL PRACTICE
Year : 2021  |  Volume : 26  |  Issue : 1  |  Page : 63-64
 

Multiple Cutaneous Malignancies in a Child with Xeroderma Pigmentosa


1 Indira Gandhi Medical College and Research Institute, Puducherry, India
2 Department of Pediatrics, All India Institute of Medical Sciences (AIIMS), Mangalagiri, Andhra Pradesh, India

Date of Submission07-Apr-2020
Date of Decision13-Aug-2020
Date of Acceptance22-Sep-2020
Date of Web Publication11-Jan-2021

Correspondence Address:
Dr. Thirunavukkarasu Arun Babu
Department of Pediatrics, All India Institute of Medical Sciences (AIIMS), Mangalagiri, Andhra Pradesh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_97_20

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How to cite this article:
Balakrishnan P, Babu TA. Multiple Cutaneous Malignancies in a Child with Xeroderma Pigmentosa. J Indian Assoc Pediatr Surg 2021;26:63-4

How to cite this URL:
Balakrishnan P, Babu TA. Multiple Cutaneous Malignancies in a Child with Xeroderma Pigmentosa. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2021 Jun 13];26:63-4. Available from: https://www.jiaps.com/text.asp?2021/26/1/63/306710




A 11-year-old boy with xeroderma pigmentosa (XP) presented with multiple ulcerated nodules in the right cheek, dorsum of the nose, forehead, right ear lobe and ulceroproliferative like growth over the left nasolabial fold [Figure 1]a and ulceroproliferative nodule over the right leg [Figure 1]b. Histopathology sections from skin lesions confirmed the simultaneous occurrence of three cutaneous malignancies, namely squamous cell carcinoma, basal cell carcinoma, and malignant melanoma [Figure 2]a,[Figure 2]b,[Figure 2]c.
Figure 1: (a) Multiple ulcerated nodules in the right cheek, dorsum of nose, forehead, right ear lobe, and ulceroproliferative like growth over the left nasolabial fold. (b) Ulceroproliferative nodule over the right leg

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Figure 2: (a) Sections from nodules over the right cheek and dorsum of nose showed features of basal cell carcinoma. (b) Sections from ulceroproliferative growth over the left cheek showed invasive islands of pleomorphic squamous epithelial cells with keratin pearls infiltrating the underlying stroma with focal collections of lymphocytes. (c) Sections from ulceroproliferative growth over the right leg showed surface stratified squamous keratinized epithelium with invasive islands of polygonal tumor cells with prominent nucleoli and intracellular melanin pigment. (H and E, ×400)

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XP is a rare genetically inherited disorder associated with oculocutaneous premalignant and malignant lesions and progressive neurodegeneration predominantly occurring during childhood and adolescence.[1] It is an autosomal recessive disorder caused by the impaired repair mechanism of ultraviolet-B rays-induced DNA damage to skin and eyes.[2] The main cutaneous clinical features include photosensitivity, pigmentary changes, premature skin aging, actinic keratosis, and development of multiple cutaneous malignancies, more commonly basal cell carcinoma, squamous cell carcinoma, malignant melanoma, and fibrosarcoma.[1],[2]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Shankar R, Qureshi SS, Sugoor P, Kembhavi S, Yadav PS, Mukta R. Colossal squamous cell carcinoma of the face in a child with xeroderma pigmentosum. J Indian Assoc Pediatr Surg 2014;19:185-6.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Singh AP, Ansari M, Shukla AK. Basal cell carcinoma with xeroderma pigmentosum in an 8-year-old girl. J Indian Assoc Pediatr Surg 2019;24:314-6.  Back to cited text no. 2
[PUBMED]  [Full text]  


    Figures

  [Figure 1], [Figure 2]



 

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