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Year : 2021  |  Volume : 64  |  Issue : 4  |  Page : 868-870
Dendritic cell neurofibroma with pseudorosettes: A case report with review of literature

1 Department of Hematology, Indira Gandhi Institute of Medical Sciences, Patna, Bihar, India
2 Department of Pathology, AIIMS, Patna, Bihar, India

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Date of Submission06-May-2020
Date of Decision13-Jun-2020
Date of Acceptance24-Jul-2020
Date of Web Publication20-Oct-2021

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;64:868-70

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 20];64:868-70. Available from:

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 Top

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: (a) Clinical preoperative photograph of the patient showing dark brown colored pigmented cutaneous lesions spanning over trunk and abdomen with small nodules on skin surface shown by arrow. (b) X ray chest and abdomen–lateral view showing a soft tissue shadow spanning abdomen and extending to right posterolateral chest wall shown by arrow. (c) MRI showing a large heterogenous mass in posterior back extending from D3 to L5 vertebrae as shown by arrow

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Figure 2: (a) Microphotograph showing tumor cells arranged in nodules of varying sizes by fibrovascular septa (H&E; X40). (b) Microphotograph showing pseudo rosette formation by type II cells. (H&E; X100). (c) Microphotograph showing small dark type I & II cells and large pale staining type II cells exhibiting stellate growth pattern (H&E; X400). (d) Microphotograph showing myxoid changes (H&E; X400)

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Figure 3: (a) Microphotograph showing diffuse cytoplasmic and membranous S-100 positivity in tumor cells (DAB; 100X). Inset shows the same in higher magnification; 400X). (a) Microphotograph showing tumor cells negative for EMA (DAB; 400X). Inset shows EMA positive perineurium, H &E; 100X

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   Discussion Top

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: Showing comparison of PubMed-based literature review of all cases of DCNWPR till date

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

   Conclusion Top

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.

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Conflicts of interest

There are no conflicts of interest.

   References Top

Messina J, Yeh I. WHO Classification of Skin Tumors. Lhyon, France: IARC; 2018. p. 353-6.  Back to cited text no. 1
Michal M, Fanburg-Smith JC, Mentzel T. Dendritic cell neurofibroma with pseudorosettes: A report of 18 cases of a distinct and hitherto unrecognized neurofibroma variant. Am J Surg Pathol 2001;25:587-94.  Back to cited text no. 2
Simpson RH, Seymour MJ. Dendritic cell neurofibroma with pseudorosettes: Two tumors in a patient with evidence of neurofibromatosis. Am J Surg Pathol 2001;25:1458-9.  Back to cited text no. 3
Kazakov DV, Vanecek T, Sima R. Dendritic cell neurofibroma with pseudorosettes lacks mutations in exons 1-15 of the neurofibromatosis type 2 gene. Am J Dermatopathol 2005;27:286-9.  Back to cited text no. 4
Kazakov DV, Mukensnabl P, Zamecnik M. Intraneural dendritic cell neurofibroma with pseudorosettes. Am J Dermatopathol 2004;26:72-5.  Back to cited text no. 5
Petersson F. Dendritic cell neurofibroma sine pseudorosettes: Report of a case with a granulomatous appearance. Am J Dermatopathol 2011;33:742-4.  Back to cited text no. 6
Hsin YH, Wu YW. Dendritic cell neurofibroma with pseudorosettes: One case report and literature review. Dermatol Sinica 2012;30:75-7.  Back to cited text no. 7
Lerman MA, Li CC, Woo SB. Dendritic cell neurofibroma with pseudorosettes: A clinicopathologic and immunohistochemical study of 5 intraoral cases. Oral Surg Oral Med Oral Pathol Oral Radiol 2014;117:221-6.  Back to cited text no. 8
Hattori T, Fukumoto T, Anan T. Four Japanese cases of dendritic cell neurofibroma with pseudorosettes. J Dermatol 2014;41:1077-81.  Back to cited text no. 9
Kubota R, Nishida H. A case of dendritic cell neurofibroma with pseudorosettes. Am J Dermatopathol 2020:1-4. doi: 10.1097/DAD.0000000000001613.  Back to cited text no. 10

Correspondence Address:
Iffat Jamal
IGIMS, Sheikhpura, Patna - 800 016, Bihar
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_475_20

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  [Figure 1], [Figure 2], [Figure 3]

  [Table 1]


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