A 68‐Year‐Old Woman with A Left Orbital and Temporal Mass

Bertero, Luca; Zenga, Francesco; Maletta, Francesca; Senetta, Rebecca; Cassoni, Paola

doi:10.1111/bpa.12580

Cited by 2 publications

(2 citation statements)

References 4 publications

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“…Interestingly, a report published by Bertero et al described a similar case of an orbito-temporal mass ultimately diagnosed as “Rosai-Dorfman meningeal disease with IgG4-related disease histological features”; just as in our case, initial histopathological analysis of was suggestive of IgG4-RD, while further samples exhibited features pathognomonic for RDD [ 29 ]. The histologic overlap between IgG4-RD and RDD in both of these cases may indicate a common pathogenesis underlying at least a subset of these two inflammatory processes.…”

Section: Discussionmentioning

confidence: 52%

Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report

Iyengar¹,

Golub

McQuinn³

et al. 2020

acta neuropathol commun

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Inflammatory orbital lesions include a broad list of diagnoses, many of them with overlapping clinical and radiographic features. They often present a diagnostic conundrum, even to the most experienced orbital specialist, thus placing considerable weight on surgical biopsy and histopathological analysis. However, histopathological diagnosis is also inherently challenging due to the rarity of these lesions and the overlaps in histologic appearance among distinct disease entities. We herein present the case of an adolescent male with a subacutely progressive orbital mass that generated a significant diagnostic dilemma. Early orbital biopsy was consistent with a benign fibro-inflammatory lesion, but corticosteroid therapy was ineffective in halting disease progression. After an initial substantial surgical debulking, histopathological analysis revealed several key features consistent with IgG4-related disease (IgG4-RD), a systemic fibro-inflammatory process typically accompanied by multifocal tumor-like lesions. Surprisingly, within months, there was clear evidence of clinical and radiographic disease progression despite second-line rituximab treatment, prompting a second surgical debulking. This final specimen displayed distinctive features of Rosai-Dorfman disease (RDD), a systemic inflammatory disease characterized by uncontrolled histiocytic proliferation. Interestingly, certain features of this re-excision specimen were still reminiscent of IgG4-RD, which not only reflects the difficulty in differentiating RDD from IgG4-RD in select cases, but also illustrates that these diagnoses may exist along a spectrum that likely reflects a common underlying pathogenetic mechanism. This case emphasizes the importance of surgical biopsy or resection and histopathological analysis in diagnosing—and, ultimately, treating—rare, systemic inflammatory diseases involving the orbit, and, furthermore, highlights the shared histopathological features between RDD and IgG4-RD.

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Section: Discussionmentioning

confidence: 52%

Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report

Iyengar¹,

Golub

McQuinn³

et al. 2020

acta neuropathol commun

View full text Add to dashboard Cite

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“…According to 285 RDD literature reviewed cases [1][2][3][4][5]7,9,10,[12][13][14][15][16][17][18][19][20][21][22][23][24][26][27][28][29][30][31][32][33][34][35][36][37][38][39]41,[44][45][46][47][48][50][51][52][53][54][56][57][58][59][60][61][62]…”

Section: Discussionmentioning

confidence: 99%

Rosai-Dorfman disease mimicking images of meningiomas: Two case reports and literature review

Tatit

Raffa²,

Motta

et al. 2021

Surgical Neurology International

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Background: Rosai-Dorfman disease (RDD) is a rare non-Langerhans cell histiocytic proliferative disorder classically as a massive cervical lymphadenopathy. However, over the years, extranodal locations were confirmed with the central nervous system involvement in less than 5% of cases, which is marked as a significant differential diagnosis of meningiomas, with which they are widely confused due to the similarity of their radiological images. Case Description: We report a 37-year-old man and 45-year-old man who were diagnosed with intracranial RDD but whose radiological images mimic meningiomas, requiring anatomopathological and tumor’s immunohistochemistry for definitive diagnosis. Moreover, a review of 184 publications with 285 cases of intracranial involvement of this disease was also performed, comparing these findings with those brought in the previous studies. Conclusion: Intracranial Rosai-Dorfman tumors should always be remembered as differential diagnosis of meningiomas since they are similar radiologically and macroscopically. Once remembered and diagnosed, the lesion must be treated following the same pattern of resection done in meningiomas and, treatment’s differences will not occur in the surgical excision technique, but in complementary chemotherapy implementation, radiotherapy, and even with radiosurgery aid, depending on the case. Thus, it is possible to obtain better results than with just the isolated surgical procedure.

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A 68‐Year‐Old Woman with A Left Orbital and Temporal Mass

Cited by 2 publications

References 4 publications

Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report

Orbital Rosai-Dorfman disease initially diagnosed as IgG4-related disease: a case report

Rosai-Dorfman disease mimicking images of meningiomas: Two case reports and literature review

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