Indian Journal of Pathology and Microbiology

: 2021  |  Volume : 64  |  Issue : 4  |  Page : 868--870

Dendritic cell neurofibroma with pseudorosettes: A case report with review of literature

Iffat Jamal1, Punam P Bhadani2,  
1 Department of Hematology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Pathology, AIIMS, Patna, Bihar, India

Correspondence Address:
Iffat Jamal
IGIMS, Sheikhpura, Patna - 800 016, Bihar

How to cite this article:
Jamal I, Bhadani PP. Dendritic cell neurofibroma with pseudorosettes: A case report with review of literature.Indian J Pathol Microbiol 2021;64:868-870

How to cite this URL:
Jamal I, Bhadani PP. Dendritic cell neurofibroma with pseudorosettes: A case report with review of literature. Indian J Pathol Microbiol [serial online] 2021 [cited 2023 Mar 31 ];64:868-870
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Full Text

Dear Editor,

DCNWPR is a rare variant of neurofibroma with only 37 cases being reported so far including our present case.[1] This entity is often misdiagnosed as any other neural tumor. Hence, we report this case to raise awareness about this rare variant. Simultaneously, a PubMed-based review of literature was also made with the keywords dendritic cell, neurofibroma, and pseudorosettes.[2]

A detailed PubMed-based literature search was made to review the number of cases of DCNWPR reported so far so as to emphasize its rare occurrence. Articles between 2001 to 2020, both years inclusive, were searched. Reviewed articles provided additional references. Recent reviews in high-impact journals were given extra weightage. Of the total 110 articles, 16 articles were shortlisted and read by all authors. The keywords were used for PubMed based literature search were dendritic cell, neurofibroma, and pseudorosettes.

 Case Report

A 4-year-old boy presented with a swelling over the mid-back region gradually increasing in size and associated with itching since birth. On clinical examination, a firm compressible swelling was noted of size 12 × 10 × 6 cm. The overlying skin was hyperpigmented [Figure 1]a. MRI revealed an ill-defined, large discrete confluent T2 heterogenous hyperintense lesion of size 22 × 11.8 × 3 cm in the posterior back extending from D3 to L5 vertebral body in subcutaneous plane. A radiological diagnosis of neurofibromatosis was made [Figure 1]b and [Figure 1]c. Debulking of tumor was done and sent for histopathology. Grossly, they were greyish white, multiple-fragmented soft tissue pieces, with the largest measuring 17 × 5 × 1 cm and the smallest measuring 0.5 × 0.5 × 0.3 cm. The outer surface was rough and the cut section was solid, greyish white, and glistening. Microscopically, many tumor nodules were noted that are composed of two main types of cells. One population was smaller in size and lymphocyte like with scant cytoplasm and dark nuclei and the other group of cells were larger in size, with abundant eosinophilic cytoplasm and vesicular nuclei. The larger cells were arranged around smaller cells forming pseudorosettes. No nuclear atypia and mitosis were noted. Immunohistochemistry revealed both small and larger cells to be diffusely positive for S-100 with negative EMA staining in tumor cells and its positivity limited to the perineurium [Figure 2]a, [Figure 2]b and [Figure 3]a, [Figure 3]b.{Figure 1}{Figure 2}{Figure 3}


Michal et al. in 2001 first reported DCNWPR as a distinct and unrecognized neurofibroma variant in 2001.[2] A total of approximately 37 cases have been previously reported so far and most of them were unrelated to neurofibromatosis. Their characteristic features are summarized in [Table 1]. Among these 37 cases, 19 (51.3%) were males and 17 (45.9%) were females with age range being 4–77 years. The lesion was most frequently seen on the trunk (37.8%), followed by the extremities and face that comprise 18.9% each.[3],[4] DCNPWR is mostly solitary but it was associated with neurofibromatosis in our case that made it worth reporting.[5]{Table 1}

Differential diagnoses include plexiform neurofibroma, palisaded encapsulated neuroma, epithelioid schwannoma and Neurothekoma.


DCPWNR is a rare variant of neurofibroma and hence a high degree of clinical and histopathological suspicion is required to diagnose it. Its occurrence in a 4 year old which is quite an uncommon age group, as all the previous studies done so far showed it to be a disease of adults and old age, and its association with neurofibromatosis made this case worth reporting.

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.[10]

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.


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