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Year : 2020  |  Volume : 63  |  Issue : 3  |  Page : 511-512
Catechism (Quiz 9)

Department of Surgical Pathology, Tata Memorial Hospital (TMH), HBNI University, Mumbai, Maharashtra, India

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Date of Submission22-Apr-2020
Date of Acceptance24-Apr-2020
Date of Web Publication7-Aug-2020

How to cite this article:
Rekhi B. Catechism (Quiz 9). Indian J Pathol Microbiol 2020;63:511-2

How to cite this URL:
Rekhi B. Catechism (Quiz 9). Indian J Pathol Microbiol [serial online] 2020 [cited 2022 Jan 26];63:511-2. Available from: https://www.ijpmonline.org/text.asp?2020/63/3/511/291730

A 3 year-old male child presented with pain and difficulty in holding neck of 1 month duration. He underwent radiological examination that revealed a lesion measuring 3.5 cm in the largest dimension. Subsequently, he underwent a biopsy for a erosive lesion in the C1-C2 spinal level.

   Microscopic Features Top

Review of biopsy sections revealed a cellular tumor composed of polygonal cells, exhibiting moderate to focally marked degree of nuclear pleomorphism, arranged in a non-descript manner, containing granular, eosinophilic to vacuolated cytoplasm, at places, along with discernible nucleoli and interspersed mitotic figures and chronic inflammatory cells [Figure 1]a, [Figure 1]b, [Figure 1]c.
Figure 1:

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Immunohistochemical expression of pan cytokeratin (AE1/AE3) is shown in [Figure 1]d.


Q1. What is the diagnosis?

Q2. What is the likely (reasonably specific) immunohistochemical marker shown in [Figure 2]a?

Q3: Which is the likely immunohistochemical marker and its interpretation shown in [Figure 2]b, in context of the diagnosis?
Figure 2:

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   Answers of Catechism (Quiz 8) Top

  1. Cytology smear was highly cellular with many histiocytes containing intact lymphocytes and plasma cells, many of which were surrounded by a halo [Figure 1]a and [Figure 1]b. Histiocytes had a bland nucleus with abundant pale vacuolated cytoplasm [Figure 1]c and [Figure 1]d. Few binucleated and multinucleated histiocytes were also seen. Background showed lymphocytes and plasma cells. No eosinophils or atypical cells were seen. The diagnosis offered was sinus histiocytosis with massive lymphadenopathy (Rosai–Dorfman disease) and was subsequently confirmed on biopsy.
  2. S100 and CD68.

   Discussion Top

Sinus histiocytosis with massive lymphadenopathy (SHML), also called Rosai–Dorfman disease, is a rare self-limiting benign histiocytic proliferative disorder of unknown etiology that affects all age groups.[1] The main clinical presentation is painless bilateral cervical lymph node enlargement.[1] Other sites of involvement in order of frequency include axillary, inguinal, para-aortic, and mediastinal lymph node.[2] Extranodal manifestation, such as the skin and soft tissue, CNS, bone, eyes, upper respiratory tract, breast, heart, thyroid, etc., has also been described.[2] It is usually associated with fever, leukocytosis, and raised erythrocyte sedimentation rate (ESR).[2] FNAC is a fast and reliable diagnostic modality as the cytological features of SHML can be distinctive and may give a clue on the excisional biopsy findings.[3]

The characteristic cytological features of SHML include the presence of numerous large histiocytes with abundant pale vacuolated cytoplasm and bland nuclei.[3] The cytoplasm of these histiocytes typically shows intact lymphocytes and plasma cells and less often, neutrophils and red blood cells.[4] Sometimes, a halo can be observed around the engulfed cells.[4] This phenomenon of the presence and movement of one cell in the cytoplasm of another is called emperipolesis and differs from phagocytosis. The cells that are taken up in emperipolesis are not attacked by enzymes and remain intact within the cytoplasm, unlike phagocytosis.[4] Histiocytes with superimposed lymphocytes can also be mistaken for emperipolesis but the presence of a halo around the cells in emperipolesis helps differentiate the two. A few reports describe that the halo is better appreciated on histopathology as compared to cytology as it arises due to fixation artefact. However, it was distinctly seen in the described case and thus helped in making the diagnosis. Occasional cells with emperipolesis can also be seen in Non-Hodgkin's and Hodgkin's Lymphoma too, but the presence of numerous large histiocytes with emperipolesis that dominate the smear and absence of atypical cells rules out lymphoma.[5] SHML should be differentiated from other histiocytic lesions like reactive sinus histiocytosis and Langerhans cell histiocytosis (LCH).[5] Both lack diffuse emperipolesis and LCH shows numerous eosinophils, giant cells, and histiocytes with typical grooved or folded nuclei.[5] Histiocytes of SHML show positive immunostaining for S100, CD11c, CD14, CD33, and CD68.[5]

The standard treatment for this disease is not established and the clinical course of SHML, especially the cases with localized nodal disease is self-limiting with spontaneous remission.[4]

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Conflicts of interest

There are no conflicts of interest.

   References Top

Das DK, Gulati A, Bhatt NC, Sethi RG. Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Report of two cases with fine needle aspiration cytology. Diagn Cytopathol 2001;24:42-5.  Back to cited text no. 1
Kumar B, Karki S. Paudyal P. Diagnosis of sinus histiocytosis with massive lymphad enopathy (Rosai-Dorfman disease) by fine needle aspiration cytology. Diagn Cytopathol 2008;36:691-5.  Back to cited text no. 2
Deshpande AH, Nayak S, Munshi MM. Cytology of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease). Diagn Cytopathol 2000;22:181-5.  Back to cited text no. 3
Sall A, Touré AO, Ndiaye FS, Sène A, Sall FB, Faye BF, et al. Rosai Dorfman disease diagnosed by fine-needle aspiration cytology in a young man with HIV infection. Clin Case Rep 2015;3:879-83.  Back to cited text no. 4
Kushwaha R, Ahluwalia C, Sipayya V. Diagnosis of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman Disease) by fine needle aspiration cytology. J Cytol 2009;26:83-5.  Back to cited text no. 5
[PUBMED]  [Full text]  

Correspondence Address:
Bharat Rekhi
Room Number 818, Department of Surgical Pathology, 8th Floor, Annex Building, Tata Memorial Hospital, Dr E.B. Road, Parel, Mumbai - 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/IJPM.IJPM_436_20

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