Background:
Histopathology is a cornerstone of diagnostics in Sjögren`s syndrome (SjS). The current widely accepted standard, minor salivary gland (lip) biopsy, is a surgical procedure with several ...disadvantages including permanent sensory loss of the lips as a well-known complication (reviewed in 1). Moreover, lymphoma of salivary glands cannot be excluded. Ultrasound-guided core needle biopsy (CNB) is an excellent diagnostic tool with good safety (2) and encouraging results as compared to open biopsy (3).
Objectives:
To retrospectively analyze safety and diagnostic outcome of ultrasound-guided CNB in patients with known or suspected SjS.
Methods:
A retrospective analysis of a case series of four patients with known or suspected SjS. All patients were treated at the outpatient clinic of the Department of Rheumatology & Immunology, and the Department of Otorhinolaryngology. All patients underwent ultrasound-guided CNB: We clean and disinfect the ultrasound transducer and the skin of the patient before and after the procedure. We use a sterile probe cover and gloves. Only sterile ultrasound gel or ultrasound compatible disinfectant should be used. Core-needle biopsy was performed using a 20 g needle (Bard). A Logiq S8 GE ultrasound device with a 6-15 MHz matrix linear transducer was used. We performed a local anesthesia at the entry point and a 2mm skin incision. The sampling length was set on 20mm. The CNB was performed at the most suspicious focal sonographic lesion. 2-3 needle passes were carried out through the same skin access.
Results:
Representative histopathological samples were obtained from all patients. In patient 1 (62y) with known SjS and parotid swelling, mucosa-associated lymphoid tissue (MALT) lymphoma was diagnosed (previous lip biopsy with no proof of malignancy). Also in patient 2 (35y) with known SjS and a 20-years history of parotid swelling, MALT lymphoma was diagnosed. In this patient a lip biopsy was performed in the previous year supporting the diagnosis of SjS, but without proof of malignancy. In patient 3 (64y) with SSc, anti-Ro/SSA positivity and dry eyes and mouth, the biopsy established the diagnosis of SjS. In patient 4 (59y) with SSc, negative anti-Ro/SSA antibodies and dry eyes/mouths, SjS could be excluded. In the corresponding ultrasound, all patients showed hypoechogenic lesions and inhomogeneous parenchyma of major salivary glands reflecting OMERACT grade II-III SjS ultrasound score (4). No safety signals were observed. Patients with prior lip biopsies perceived ultrasound-guided CNB as preferable.
Conclusion:
This pilot study suggests that ultrasound-guided CNB in SjS is a safe procedure with an excellent diagnostic yield allowing the diagnosis of lymphoma of the salivary glands, which is superior to lip biopsy. Given these encouraging results, we will now increase patient numbers for further validation.
References:
1Pijpe, J., et al.,
Parotid gland biopsy compared with labial biopsy in the diagnosis of patients with primary Sjogren’s syndrome.
Rheumatology (Oxford), 2007.
46
(2): p. 335-41.
2Kim, H.J. et al.,
Ultrasound-guided core needle biopsy in salivary glands: A meta-analysis.
Laryngoscope, 2018.
128
(1): p. 118-125.
3Zabotti, A. et al.,
Ultrasound-guided core needle biopsy compared with open biopsy: a new diagnostic approach to salivary gland enlargement in Sjögren’s syndrome? Rheumatology (Oxford). 2020.
17
:keaa441. Epub ahead of print.
4Jousse-Joulin, S., et al.,
Video clip assessment of a salivary gland ultrasound scoring system in Sjogren’s syndrome using consensual definitions: an OMERACT ultrasound working group reliability exercise.
Ann Rheum Dis, 2019.
78
(7): p. 967-973.
Disclosure of Interests:
Urs Borner: None declared, Lluis Nisa Hernandez: None declared, Adela-Cristina Sarbu: None declared, Lisa Christ Shareholder of: Roche, Grant/research support from: Gilead, Roche, Pfizer, Yara Banz: None declared, Britta Maurer Speakers bureau: Boehringer-Ingelheim, Grant/research support from: Abbvie, Protagen, Novartis Biomedical Research and congress support from Pfizer, Roche, Actelion, mepha, and MSD. BM has a patent for mir-29 for the treatment of systemic sclerosis issued (US8247389, EP233143)., Marco D. Caversaccio Grant/research support from: Medel, Cochlear, Oticon, Storz, Florian Kollert Shareholder of: Roche, Consultant of: Actelion, BMS, Boehringer-Ingelheim, Pfizer, Grant/research support from: Gilead, Pfizer, Employee of: In the past at Novartis, currently at Roche
Zusammenfassung
Die frühe Diagnose und Behandlung einer Vaskulitis ist entscheidend, um schwere, lebensalterierende oder -bedrohliche Komplikation zu vermeiden, insbesondere bei Vaskulitiden, die ...primär vitale Strukturen wie die Aorta oder Blutgefäße befallen, die wichtige Organe versorgen, wie zum Beispiel die Temporalarterie und ihre Abgänge. Die Anamnese und klinische Präsentation können den Kliniker dazu leiten, zusätzliche Untersuchungen durchzuführen, um die Diagnose der Vaskulitis zu bestätigen. Die histopathologische Untersuchung wird immer noch als „Goldstandard“ in der Vaskulitisdiagnostik angesehen. Obwohl der positive prädiktive Wert im Allgemeinen hoch ist, schließt ein negatives Resultat in der Biopsie noch keineswegs eine Vaskulitis zuverlässig aus. Die Entzündung der befallenen Blutgefäße ist oft sowohl räumlich als auch zeitlich heterogen. Die Spezifität der Biopsie wird dabei häufig durch viele Faktoren beeinflusst – so zum Beispiel die Biopsiegröße beziehungsweise Länge des befallenen Gefäßes oder die Expertise des Chirurgen sowie des Pathologen, der die Biopsie interpretiert. Zudem können störende Faktoren, wie vorangegangene Therapien, Atherosklerose und andere nicht vaskulitische Gefäßveränderungen die Korrektheit der Diagnose beeinflussen. Es ist deshalb essenziell, dass alle Personen, die an die Behandlung des Patienten beteiligt sind – sowohl der Chirurg als auch der Internist und Pathologe – eng miteinander kollaborieren, um eine optimale Behandlung zu garantieren. Des Weiteren ist eine kontinuierliche Fortbildung („continuous medical education“) der in der Behandlung des Patienten mit Vaskulitis involvierten Ärzten sowie des technischen Personals unerlässlich. Zukünftige diagnostische Fortschritte sollten in den kommenden Jahren berücksichtigt werden. So zum Beispiel die Bestimmung sog. Biomarker, um die Patientenbetreuung optimieren und individualisieren zu können.
Abstract Antrochoanal polyps are hyperplasias of the nasal mucosa, which have their origin in the maxillary sinus and extend through the nasal cavity and the choanae into the naso- and oropharynx. In ...children antrochoanal polyps represent one of the more frequent manifestations of paediatric nasal polyposis. Most studies on antrochoanal polyps in children report only on nasal obstruction, hyponasal speech and snoring, which are also encountered in the most common cause of obstructive sleep apnoea syndrome; i.e. adenoid or tonsillar hyperplasia. Only very few studies report on additional health hazards by antrochoanal polyps ranging from obstructive sleep apnoea syndrome to swallowing disorders and cachexia. We present the case of an 8 year old girl with a bicycle accident caused by excessive daytime sleepiness and obstructive sleep apnoea syndrome due to an extensive antrochoanal polyp. After a transnasal polypectomy and meatotomy type II the obstructive sleep apnoea and day time sleepiness resolved completely. Awareness of this additional health hazard is important and correct evaluation and timely diagnosis of a potential antrochoanal polyp is mandatory because minimally invasive rhinosurgery is highly curative in preventing further impending problems.
The effect of hydrostatic infiltrations for subperichondrial dissection is controversial. Classical textbooks promote it as the "key step in elevating the flaps" or consider its practicability "a ...mere fable". Moreover, case reports describe fatal side effects. Up to now, experimental tests are missing.
Experimental study.
Three surgeons simulated subperichondrial hydrodissection with 20 mineral salt fixed human cadaver heads. One ml lidocaine 5% with 1:105 adrenaline and India ink was infiltrated. Each septum was examined histologically using serial 3 microm sections in 150 microm intervals. Tissue cleavage containing the ink deposits with minimal distance to the proposed subperichondrial zone, intravasal spread and tissue deposition were analyzed.
Every injection produced a physical dissection (n = 20). However, dissected planes were localized mostly in the supra-perichondrial connective tissue (n = 8) or within the perichondrium (n = 4). Only five cases showed the propagated correct dissection in a subperichondrial zone. Three anomalous septa were excluded from quantitative analysis. Infiltrated matter did not only accumulate within the dissection plane but also penetrated the surrounding vessels of the septal intumescentia (n = 8).
Hydrostatic infiltrations represent an unreliable surgical technique for dissection of an anatomical correct subperichondrial plane but can be useful for anesthesia and hemostasis, however, using high pressure and high volume infiltrations might foster serious side effects.
Study Design
Case report.
Objectives
With only two previously reported cases, localized amyloidosis of the sacrum is extremely rare. Here we report a 64-year-old woman with a large osteolytic lesion ...accompanied by weakness and paresthesia of the right leg and difficulties in bladder control.
Methods
Fine needle biopsy and standard staging procedures revealed a primary solitary amyloidoma that was treated with intralesional resection, lumbopelvic stabilization, and consolidation radiotherapy.
Results
Clinical follow-up revealed the diagnosis of multiple myeloma 9 months after initial treatment. At 12 months, no local recurrence has occurred, the neurologic symptoms have resolved, and the systemic disease is in remission.
Conclusions
Intralesional resection with adjuvant radiotherapy of the amyloidoma achieved good local tumor control with limited morbidity.
Summary
Follicular lymphoma (FL) constitutes a significant proportion of lymphomas and shows frequent relapses. Beyond conventional chemotherapy, new therapeutic approaches have emerged, focussing on ...the interplay between lymphoma cells and the microenvironment. Here we report the immunophenotypic investigation of the microenvironment of a clinically well‐characterized prospective cohort (study SAKK35/10, NCT01307605) of 154 treatment‐naïve FL patients in need of therapy, who have been treated with rituximab only or a combination of rituximab and the immunomodulatory drug lenalidomide/Revlimid® A high ratio of CD4‐ to CD8‐positive T cells (P = 0·009) and increased amounts of PD1+ tumour‐infiltrating T cells (P = 0·007) were associated with inferior progression‐free survival in the whole cohort. Interestingly, the prognostic impact of PD1+ T cells and the CD4/CD8 ratio lost its significance in the subgroup treated with R2. In the latter group, high amounts of GATA3+ T helper (Th2) equivalents were associated with better progression‐free survival (P < 0·001). We identified tumour microenvironmental features that allow prognostic stratification with respect to immuno‐ and combined immuno‐ and immunomodulatory therapy. Our analysis indicates that lenalidomide may compensate the adverse prognostic implication of higher amounts of CD4+ and, particularly, PD1+ T cells and that it has favourable effects mainly in cases with higher amounts of Th2 equivalents.
Correction added on 11 February 2020, after online publication: The NCT‐trial number was previously incorrect and has been updated in this version.