Generic selectors
Exact matches only
Search in title
Search in content
Search in posts
Search in pages
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstract
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Addendum
Announcement
Art & Psychiatry
Article
Articles
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Commentary
Conference Oration
Conference Summary
Continuing Medical Education
Correspondence
Corrigendum
Cosmetic Dermatology
Cosmetology
Current Best Evidence
Current View
Derma Quest
Dermato Surgery
Dermatopathology
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
e-IJDVL
Editor Speaks
Editorial
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Errata
Erratum
Focus
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
General
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
History
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL AWARDS 2015
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Index
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
LETTER TO THE EDITOR - LETTERS IN RESPONSE TO PUBLISHED ARTICLES
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Messages
Miscellaneous Letter
Musings
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News
News & Views
Obituary
Observation Letter
Observation Letters
Oration
Original Article
ORIGINAL CONTRIBUTION
Original Contributions
Pattern of Skin Diseases
Pearls
Pediatric Dermatology
Pediatric Rounds
Perspective
Presedential Address
Presidential Address
Presidents Remarks
Quiz
Recommendations
Regret
Report
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Retraction
Review
Review Article
Review Articles
Revision Corner
Self Assessment Programme
SEMINAR
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Studies
Study Letter
Supplement-Photoprotection
Supplement-Psoriasis
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
SYMPOSIUM - VITILIGO
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Tables
Technology
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapeutics
Therapy
Therapy Letter
View Point
Viewpoint
What’s new in Dermatology
View/Download PDF
Quiz
ARTICLE IN PRESS
doi:
10.4103/ijdvl.IJDVL_563_19
CROSSMARK LOGO Buy Reprints
PDF

A red nodule on the tip of the nose in a Chinese girl

Department of Dermatology, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China
Department of Neurosurgery, Xiangya Hospital, Central South University, Changsha, Hunan, China
Department of Pathology, The Second Xiangya Hospital of Central South University, Changsha, Hunan, China
Department of Dermatology, Xiangya Hospital, Central South University, Changsha, Hunan, China
Corresponding author: Dr. Siqi Fu, Department of Dermatology, The Second Xiangya Hospital of Central South University, 139 Ren Min, Middle Road, Changsha 410011, Hunan, China. fusiqi@csu.edu.cn Dr. Guiying Zhang, Department of Dermatology, The Second Xiangya Hospital of Central South University, 139 Ren Min, Middle Road, Changsha 410011, Hunan, China. lindazgy@csu.edu.cn
Co-first author
Licence
This is an open-access article distributed under the terms of the Creative Commons Attribution-Non Commercial-Share Alike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms.

How to cite this article: Fu S, Zeng Y, Zhang J, Zhou Y, Fan S, Chen M, et al. A red nodule on the tip of the nose in a Chinese girl. Indian J Dermatol Venereol Leprol,doi:10.4103/ijdvl.IJDVL_563_19

A 7-year-old girl presented with a red nodule on the apex of the nose for 1 month without any other symptom. Skin examination revealed a well-defined, round, pink nodule measuring 1.2 × 1.5 cm with a hard texture [Figure 1a]. Her past medical history was unremarkable.

An incisional biopsy showed normal epidermis and nests or sheets of small round cells with atypical nuclei and scanty cytoplasm in the reticular dermis. The tumor cells formed alveolar structures. Necrosis, cross striations or obvious mitotic activity were absent [Figures 1b and c]. Immunohistochemical studies demonstrated that the tumor cells were strongly positive for vimentin, myoblast determination protein 1, desmin and myogenin, weakly positive for myoglobin (focal), synaptophysin, cytokeratin and Ki-67 (30% +); and negative for S-100, smooth muscle actin and cluster of differentiation (CD) 3 [Figures 2a-d].

Figure 1a:: Solitary, pink nodule on tip of nose of a 7-year-old girl
Figure 1b:: Nest or sheet-like distribution of tumor cells with atypical nuclei and scanty cytoplasm, with some tumor cells forming alveolar structures. No necrosis, cross striations or obvious mitotic activity was found (hematoxylin and eosin, × 40).
Figure 1c:: Nest or sheet-like distribution of tumor cells with atypical nuclei and scanty cytoplasm, some tumor cells forming alveolar structures. No necrosis, cross striations or obvious mitotic activity was found (hematoxylin and eosin, × 400).
Figure 2a:: Immunohistochemical stain showed reactivity for myoblast determination protein 1 (×400)
Figure 2b:: Immunohistochemical stain showed reactivity for myogenin (×400)
Figure 2c:: Immunohistochemical stain showed reactivity for desmin (×400)
Figure 2d:: Immunohistochemical stain showed reactivity for Ki-67 (30% +) (×400)

Answer

Alveolar rhabdomyosarcoma

Discussion

Rhabdomyosarcoma is the most common soft tissue sarcoma among children.1 Rhabdomyosarcoma is classified into four types based on histological features, namely, alveolar rhabdomyosarcoma, embryonal rhabdomyosarcoma, spindle cell rhabdomyosarcoma and pleomorphic rhabdomyosarcoma. The most common site of occurence of rhabdomyosarcoma is the head and neck region. Almost all rhabdomyosarcomas occur in the deep soft tissue, on the trunk and extremities.2 It rarely occurs on the skin and superficial dermis, as primary sites.3

Rhabdomyosarcoma in the head and neck region can be divided into three subgroups: orbital, parameningeal and nonorbital nonparameningeal.2 Almost all nonorbital nonparameningeal rhabdomyosarcomas are reported to occur in deeper tissue. Rhabdomyosarcoma in the nose usually appears in the nasal cavity and rarely occurs on the skin as the primary site (comprising less than 1% of all rhabdomyosarcoma).3 Based on histologic features, alveolar rhabdomyosarcoma and embryonal rhabdomyosarcoma are common subtypes. Usually, alveolar rhabdomyosarcoma shows more aggressive clinical behavior with a worse prognosis than embryonal rhabdomyosarcomas.3 Till now, very few cases of superficial primary cutaneous alveolar rhabdomyosarcoma have been reported on the nasal skin.4

Histopathologically, the present case exhibited nests and sheets of small round tumor cells in the superficial dermis. Focal alveoli-like structures were observed, which is an important histological feature of primary cutaneous rhabdomyosarcoma.3 These morphological features mimic melanocytic as well as epithelial tumors. The tumor cells were strongly positive for vimentin, myoblast determination protein 1, desmin, myogenin and negative for S-100, smooth muscle actin, leukocyte common antigen and CD3. The strong expression of myogenin and myoblast determination protein 1, the two most reliable markers for rhabdomyosarcoma, helped us reach a diagnosis of primary cutaneous superficial alveolar rhabdomyosarcoma and excluded malignant melanoma, Spitz nevus, vascular tumors, squamous cell carcinoma, lymphoma, leukemia, Merkel cell carcinoma, leiomyosarcoma and hematological malignancies.

The optimal treatment of rhabdomyosarcoma includes wide local excision with lymph node dissection, along with postoperative radiation and chemotherapy.5 However, the optimal width and impact of the margin of resection have not been well-defined. The following factors have contributed to patients’ outcomes, such as tumor size, histological subtype, invasion to the adjacent tissues, the involvement of regional lymph nodes and distant metastases. Due to financial difficulties, the patient did not undergo postoperative radio-chemotherapy and died of cervical metastasis a year later.

In summary, this case had an unusual presentation that could be easily misdiagnosed as Spitz nevus or another cutaneous neoplasm. The correct diagnosis of alveolar rhabdomyosarcoma was reached based on the histopathological features and immunochemistry findings. The patient’s general condition rapidly worsened due to regional metastasis and died a year after the initial presentation, underlying the importance of awareness of such a disease presentation and prompt diagnosis. Optimal treatment is yet to be defined and should be based on clinical analysis of more such cases.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that child’s names and initials will not be published and due efforts will be made to conceal the identity but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

  1. , , , , . Rhabdomyosarcoma of the maxillofacial region in children and adolescents: Report of 9 cases and literature review. J Craniomaxillofac Surg. 2017;45:831-8.
    [CrossRef] [PubMed] [Google Scholar]
  2. , . Head and neck rhabdomyosarcoma: A critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg. 2011;145:967-73.
    [CrossRef] [PubMed] [Google Scholar]
  3. , , , . Primary cutaneous rhabdomyosarcoma: Case report and review of published work. J Dermatol. 2015;42:1014-5.
    [CrossRef] [PubMed] [Google Scholar]
  4. , , , . Primary cutaneous rhabdomyosarcoma: A clinicopathologic review of 11 case. J Cutan Pathol. 2012;39:987-95.
    [CrossRef] [PubMed] [Google Scholar]
  5. , , , . Rhabdomyosarcomas of the nose and paranasal sinuses: Treatment results in 15 cases. Otolaryngol Head Neck Surg. 2005;133:42-50.
    [CrossRef] [PubMed] [Google Scholar]

Fulltext Views
3132

PDF downloads
131
View/Download PDF
Download Citations
BibTeX
RIS
Show Sections