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Indian Journal of Otolaryngology and... Dec 2022: Langerhans cell histiocytosis (LCH) is a rare disease in adults, and it is even uncommon in pediatric populations, affecting only 1-2 people per million. A LCH located...
: Langerhans cell histiocytosis (LCH) is a rare disease in adults, and it is even uncommon in pediatric populations, affecting only 1-2 people per million. A LCH located in the frontal sinus is exceptionally unusual. We present a case of LCH located in the far lateral of the frontal sinus and discuss management as well as surgical techniques to control this far lateral frontal sinus lesion. : A 39 year old female, presented with a history of progressive headaches and purulent nasal discharge for three months. Computed tomography (CT) of the sinuses showed an approximately 15 × 10 mm soft tissue with osteolytic bony changes located in lateral wall of the frontal sinus on the left side. Total tumor removal was achieved by minimally invasive endonasal endoscopic surgery with a periorbital suspension technique. Histopathologic analysis revealed LCH and the patient was referred to a hematologist for further treatment. : LCH is a rare hematological pathology. It should be diagnosed timely and treated with a multidisciplinary approach. To get a definitive diagnosis a biopsy is mandatory. Having LCH in the far lateral frontal sinus can be challenging for a biopsy even for experienced surgeons. Using curved instruments while performing an endonasal periorbital suspension technique makes it possible to access the lateral wall of the frontal sinus safely and provides an effective surgical route similar to traditional open approaches without causing any external scars.
PubMed: 36742520
DOI: 10.1007/s12070-021-02968-0 -
Histopathology Jul 2022The sinonasal tract is a common extranodal site for Rosai-Dorfman disease (RDD). Recently, histiocytes with features of RDD were identified in the clinical setting of...
AIMS
The sinonasal tract is a common extranodal site for Rosai-Dorfman disease (RDD). Recently, histiocytes with features of RDD were identified in the clinical setting of chronic sinusitis. This study evaluates whether this phenomenon should be considered part of the RDD spectrum or classified separately as RDD-like histiocytes.
METHODS AND RESULTS
We prospectively collected 13 cases showing histological features of RDD in chronic sinusitis patients and identified 14 with similar findings (3.5%) via retrospective review of 403 sinus contents over 2 years. All 27 cases displayed nodular aggregates of eosinophilic histiocytes with intermixed lymphoplasmacytic inflammation, prominent eosinophils and emperipolesis. The histiocytes were positive for S100 protein and cyclin D1 and negative for CD1a and CD207. All patients presented with severe chronic sinusitis without tumour formation or systemic symptoms. Twelve patients with follow-up (55%) required repeat sinus surgery compared with just 43 other sinusitis patients evaluated (11%); features of RDD were present in their additional specimens. Two cases that underwent targeted next-generation sequencing (20%) had oncogenic mutations in NF1 and KEAP1.
CONCLUSIONS
Overall, these findings confirm diagnostic histological and immunohistochemical features of RDD in a subset of chronic sinusitis specimens. While patients uniformly lack systemic involvement or tumefactive growth, they have a high risk of recurrent sinus disease. Although the relatively subtle nature of the findings raises consideration of separate classification, the presence of occasional oncogenic mutations and evidence of consistent MAPK/ERK pathway activation via cyclin D1 positivity suggests that this phenomenon represents a unique limited manifestation of RDD.
Topics: Cyclin D1; Histiocytosis, Sinus; Humans; Kelch-Like ECH-Associated Protein 1; NF-E2-Related Factor 2; Sinusitis
PubMed: 35426462
DOI: 10.1111/his.14664 -
Archives of Pathology & Laboratory... Jul 2007This article provides an overview of the major pathologic manifestations of sinus histiocytosis with massive lymphadenopathy, including patient characteristics and... (Review)
Review
CONTEXT
This article provides an overview of the major pathologic manifestations of sinus histiocytosis with massive lymphadenopathy, including patient characteristics and current knowledge about its pathogenesis, with an emphasis on multifocal and extranodal presentation. Sinus histiocytosis with massive lymphadenopathy is a rare, nonneoplastic, idiopathic, proliferative histiocytic disorder; recognition of this disorder is important to avoid misinterpretation and subsequent unnecessary treatment. This is especially true for primary extranodal manifestation of this rare disorder. Although accurate diagnosis of this entity requires a correlation of clinical, radiologic, laboratory, and pathologic studies in most cases, it remains a disorder primarily defined by its histopathologic features and pathologic manifestations, which are key to the diagnosis.
OBJECTIVE
To summarize the scientific literature, provide a concise review, and emphasize the diagnostic histopathologic features of extranodal sinus histiocytosis with massive lymphadenopathy.
DATA SOURCES
A comprehensive literature review was undertaken to summarize the clinical and pathologic features of this disorder.
CONCLUSIONS
Sinus histiocytosis with massive lymphadenopathy is characterized by a rare, acquired, nonmalignant proliferation of distinctive histiocytes that present with lymphadenopathy or extranodal disease, primarily in children and young adults. It exhibits a broad range of clinical presentations, thus eliciting a wide differential diagnosis. The diverse clinical manifestations and frequent association with subtle or severe immunologic abnormalities suggest an immune-mediated cause. Additional studies are needed to characterize the interplay between death receptors and cytotoxic mediators and to further elucidate the loss of immune hemostasis that may underlie idiopathic histiocytic proliferations such as this.
Topics: Antigens, CD; Bone and Bones; Histiocytes; Histiocytosis, Sinus; Humans; Lymphatic Diseases; Radiography
PubMed: 17617001
DOI: 10.5858/2007-131-1117-MESHWM -
Journal of Research in Medical Sciences... Mar 2012Rosai-Dorfman disease is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is...
Rosai-Dorfman disease is a benign lymphohistiocytosis that often involve lymph nodes and present as massive lymphadenopathy with sinus histiocytosis. The disease is rarely associated with intracranial involvement. Herein, we report a 33-years-old man with recent onset of unconsciousness. According to his past medical history, he was suffering from frontal headache, ataxia and dizziness with no sensory or motor defect since August 2010. At initial work up, MRI showed infiltrating mass in the left parietal region. Microscopically, the mass consisted of infiltration of abundant lymphoplasma cells, neutrophils and some histiocytes scattered in fibrotic background. Emperipolesis (lymphocytophagocytosis) of histiocytic cells made the diagnosis of Rosai-Dorfman disease. Rosai-Dorfman disease should be added in the list of differential diagnosis for a dural mass mimicking meningioma or cerebral mass mimicking glioma, therefore, immunohistochemical staining for EMA, S100 and CD1a should be performed to rule out the differential diagnosis.
PubMed: 23267385
DOI: No ID Found -
European Journal of Case Reports in... 2017Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in...
UNLABELLED
Sinus histiocytosis with massive lymphadenopathy (SHML) is a rare and benign cause of lymphadenopathy and was first described in 1969. This disease commonly presents in children and young adults with supra-diaphragmatic lymphadenopathy mainly at cervical nodal sites, composed of a population of histiocytes. Since its description, over 400 cases have been described, but there are few reports in the literature on its association with lymphomas. This case report describes a case with co-existing SHML and anaplastic large cell lymphoma (ALCL). The diagnosis of SHML in our patient did not alter the clinical outcome and the patient responded well to treatment for ALCL. Clinicians should maintain a high index of suspicion in cases of infra-diaphragmatic SHML for the presence of occult lymphoma.
LEARNING POINTS
A rare presentation of sinus histiocytosis with massive lymphadenopathy (SHML) with anaplastic large cell lymphoma in the same lymph node at the same time is described.SHML has no proven clinical significance.A diagnosis of SHML in the presence of infra-diaphragmatic lymphadenopathy should raise a high index of suspicion for lymphoma.
PubMed: 30755945
DOI: 10.12890/2017_000605 -
Annals of Surgery Open : Perspectives... Dec 2023In this review, we aim to provide an overview of literature on lymph node (LN) histomorphological features and their relationship with the prognosis in colorectal cancer... (Review)
Review
OBJECTIVE
In this review, we aim to provide an overview of literature on lymph node (LN) histomorphological features and their relationship with the prognosis in colorectal cancer (CRC).
BACKGROUND
Lymph nodes play a crucial role in the treatment and prognosis of CRC. The presence of LN metastases considerably worsens the prognosis in CRC patients. Literature has shown that the total number of LNs and the number negative LNs (LNnegs) has prognostic value in CRC patients. In esophageal carcinoma, LN size seems to be surrogate of the host antitumor response and a potentially clinically useful new prognostic biomarker for (y)pN0 esophageal carcinoma.
METHODS
A comprehensive search was performed in Pubmed, Embase, Medline, CINAHL, and the Cochrane library in March 2021. The PRISMA guidelines were followed. Only studies focusing on histomorphological features and LN size and their relation to overall survival were selected.
RESULTS
A total of 9 unique articles met all inclusion criteria and were therefore included in this systematic review. Six of these studies investigated HMF (eg, paracortical hyperplasia, germinal center predominance, and sinus histiocytosis) and 4 studies LNneg size and their relationship with overall survival. The presence of paracortical hyperplasia and an increased number of large LNnegs is related to a more favorable prognosis in CRC.
CONCLUSION
The results of this systematic review seem to support the hypothesis that there is a relationship between the host antitumor response reflected in different histomorphological reaction patterns visible in LNnegs and LNneg size related to survival in CRC patients.
PubMed: 38144501
DOI: 10.1097/AS9.0000000000000336 -
Journal of Clinical Pathology Nov 1992To determine the prevalence and pathology of intramammary lymph nodes in breast specimens.
AIMS
To determine the prevalence and pathology of intramammary lymph nodes in breast specimens.
METHODS
All breast specimens examined by a single pathologist over 70 months in a large teaching hospital were studied retrospectively. All the surgical pathology reports were reviewed. Relevant glass slides from cases in which intramammary lymph nodes were identified were also reexamined.
RESULTS
Breast specimens (n = 682) were examined. Seven lymph nodes were found in five patients. The specimens comprised 533 biopsy specimens, 29 segmental resections, 22 reduction mammoplasties, 77 modified radical mastectomies and 20 gynecomastia mastectomies. No clinically relevant microscopical abnormalities were found in four lymph nodes and slight sinus histiocytosis was seen in two nodes. One node contained metastatic adenocarcinoma and benign glandular epithelial inclusions.
CONCLUSION
Although rare, intramammary lymph nodes may be detected by careful gross examination of breast specimens even in the absence of clinical identification. They can occur in any quadrant of the breast and can display a variety of pathological conditions. Pathologists should be alert to the existence and potential importance of these lymph nodes.
Topics: Adenocarcinoma; Adolescent; Adult; Aged; Biopsy; Breast; Female; Humans; Lymph Nodes; Lymphatic Metastasis; Mammaplasty; Mastectomy; Mastectomy, Modified Radical; Mastectomy, Segmental; Middle Aged; Prevalence; Retrospective Studies
PubMed: 1452776
DOI: 10.1136/jcp.45.11.1023 -
BMC Ophthalmology Nov 2021To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review.
BACKGROUND
To provide basic demographic information and clinicopathologic features of ophthalmic Rosai-Dorfman disease (RDD) with a literature review.
METHODS
A multi-centre retrospective case series reviewing all patients with histopathologically confirmed ophthalmic RDD at three tertiary eye care centres between January 1993 and December 2018.
RESULTS
Eleven eyes of eight patients with histopathologically confirmed ophthalmic RDD were included, with equal numbers of males and females. The median age was 40.25 years (range: 26.6-72.4). Two patients had familial RDD. The orbit was the most commonly involved site (90.9% eyes). One patient (one eye) presented with a scleral nodule, anterior uveitis and cystoid macular oedema. Visual acuity ranged from 20/25 to light perception. Six patients had an extra-nodal ophthalmic disease, and the remaining two had an associated submandibular lymphadenopathy (nodal RDD).
CONCLUSIONS
Ophthalmic RDD can be the only manifestation of this systemic disease, with the orbit being the most commonly involved site, exhibiting bone destruction, intracranial and/or sinus involvement and variable degree of visual loss. Ophthalmic familial RDD represent a severe form with a malignant course. Steroid monotherapy may be inadequate to control orbital RDD; thus, combined treatment is usually necessary. A comprehensive approach to assessment and management is recommended.
Topics: Adult; Female; Histiocytosis, Sinus; Humans; Macular Edema; Male; Retrospective Studies; Vision Disorders; Visual Acuity
PubMed: 34814862
DOI: 10.1186/s12886-021-02173-1 -
Radiology. Imaging Cancer Jun 2022
Topics: Diagnosis, Differential; Histiocytosis, Sinus; Humans; Sciatic Nerve
PubMed: 35748761
DOI: 10.1148/rycan.220046 -
Cureus Apr 2017Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the...
Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy) is a rare benign proliferative disorder of histiocytes that typically involves the lymph nodes and can also involve extranodal sites. Rosai-Dorfman disease confined to the breast is extremely rare, but important to recognize as it can mimic malignancy. We present the case of a 63-year-old woman who presented with a palpable breast lump that was highly suspicious for malignancy based on mammogram and ultrasound appearance. Biopsy revealed inflammatory tissue with lymphoplasmacytic and histiocytic predominance. The diagnosis of Rosai-Dorfman was made based on characteristic staining of histiocytes with S-100 and the presence of emperipolesis. Early recognition of this benign disease entity spared the patient further investigation and surgical intervention.
PubMed: 28503389
DOI: 10.7759/cureus.1153