Non-IgG4-Related Fibrosing Mediastinitis Diagnosed on Needle Biopsy and Treated with Steroids

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S149-S150
Author(s):  
A Wilhelm ◽  
P V Muthukumarana ◽  
H Uddin ◽  
I Okereke

Abstract Introduction/Objective Fibrosing mediastinitis (FM) is a rare and aggressive fibroinflammatory process of the mediastinum. It is a difficult diagnosis, especially on small biopsies as the presentation and the fibrosing background mimics malignancy. Although the exact etiology is unknown, prior fungal infections, certain medications and autoimmune conditions are documented. Significant histopathologic overlap with IgG4-related disease (IgG4-RD) can complicate the diagnosis. An accurate diagnosis is important for treatment as corticosteroids for IgG4-RD is proven to be effective; however, no effective treatment is described for FM. Additionally, immunosuppression would prove harmful in other conditions with fibrosing morphology. We report a very unique case of non-IgG4-related FM that was diagnosed on a needle biopsy and showed marked improvement with corticosteroids. Methods/Case Report A 65-year-old man with no significant medical history presented with syncope and recent weight loss. Imaging revealed a pericardial effusion and a heterogeneous 8.1 cm mediastinal mass encasing the great vessels and aortic arch. Needle biopsy showed fibrosis with keloid-type collagen, mixed inflammatory cells, and few benign lymphoid follicles. No evidence of hematolymphoid malignancy, carcinoma, or infection was seen. Serum and tissue IgG-4 studies were negative for IgG4-RD. A diagnosis of FM was made, and a trial of corticosteroids was initiated. Interestingly, the patient showed improvement with a 2.3 cm decrease in lesion size and near-complete resolution of pericardial effusion after one month of treatment. Results (if a Case Study enter NA) NA Conclusion FM should be included in the differential diagnosis of infiltrative mediastinal masses which presents a diagnostic challenge on limited needle biopsies. Although effective treatment for non-IgG4 related FM is not clearly established, corticosteroids may prove beneficial, as in this patient. Mortality from FM is directly related to the compressive effect on adjacent structures. Early diagnosis and treatment are therefore crucial. Further studies on the pathogenesis are necessary, as well as better directed therapeutic options.

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 18022-18022
Author(s):  
U. Ricardi ◽  
A. M. Priola ◽  
S. M. Priola ◽  
S. Novello ◽  
A. Cataldi ◽  
...  

18022 Background: CT-guided transthoracic needle biopsy (TNB) is commonly used in diagnostic work-up of lung lesions. The availability of a on-site pathologist at the time of the procedure ameliorate its sensitivity, reduce the number of biopsies and false negatives. Methods: 612 procedures (608 patients with a CT-documented central or peripheral pulmonary lesion) performed at S. Luigi Hospital between November 2002 and August 2005 were prospectively analyzed; 66% males, median age was 66 years (range 29–87). Ineligibility criteria for the procedure included severe coagulopathy, previous contralateral pneumonectomy, lesions with a maximum diameter less than 5 mm or the impossibility to understand the procedure or to maintain the clinostatism for the time of the procedure. The on-site pathologist assigned to each specimen a semiquantitative score: 0 for bloody sample without other cells, 1 for aspecific benign or inflammatory cells, 2 for malignant cells without histotype characterization and 3 for well established benign or malignant histotype. Results: Most of the procedures was performed by fine needle aspiration biopsy, while in a minority of cases a tru-cut biopsy was requested. In 57.2% of the cases a single transthoracic access (range 1–4) was used and in 31% the procedure was repeated on the basis of the radiologist/pathologist judgment. In 154 patients a surgical resection was subsequently performed, while 454 were patients non-surgical. A score of 3 was obtained in 71% of cases (88% malignancies), 2 in 12.5%, 1 in 7.5% and 0 in 9%. A definitive diagnosis was made in 83.5% of procedures, while a score of 0–1 was assigned in 101 cases. Among 458 malignancies there were 411 lung cancer, 7 non-epithelial cancers and 40 metastases with only 1 false positive. The diagnostic accuracy for benign and malignant lesions was 67% and 92%, respectively (Pearson’s test p<0.005) with overall diagnostic accuracy of 83.3%. The variables affecting diagnostic accuracy were final diagnosis (benign 67%, malignant 92%, p<0.001) and lesion size (lesion 5 cm 78%, p<0.05). The presence of cavitation or necrotic areas and location of the lesion didn’t affect the diagnostic accuracy. Conclusion: In consecutive cases of CT-guided TNB final diagnosis and lesion size affect diagnostic accuracy. No significant financial relationships to disclose.


2018 ◽  
Vol 32 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Shahine Goulam-Houssein ◽  
Jeffrey L Grenville ◽  
Katerina Mastrocostas ◽  
David G Munoz ◽  
Amy Lin ◽  
...  

IgG4-related disease (IgG4-RD) is a multi-organ chronic inflammatory process caused by infiltration of IgG4-positive plasma cells in one or more organs. Intracranial involvement has only recently become better recognized. Our case series adds to the growing literature on the varying presentations of intracranial IgG4 by describing the clinical and imaging findings of three patients who presented to our institution with intracranial involvement. Our first patient presented with a mass-forming IgG4 pachymeningitis mimicking a sphenoid wing meningioma, which is to our knowledge the largest mass-forming pachymeningitis published in the literature. Our second case depicts another presentation of extensive IgG4 pachymeningitis involving both cavernous sinuses and surrounding Meckel’s caves. The third case describes a patient with presumed lymphocytic hypophysitis, which was later determined to be IgG4-related hypophysitis with concomitant pachymeningitis and perineural spread along the optic nerves. The delayed diagnoses in our cases illustrates the diagnostic challenge that clinicians face in differentiating intracranial IgG4-RD from other infiltrative diseases such as sarcoidosis, granulomatous disease, tuberculosis and lymphoma. Earlier consideration of IgG4-related hypophysitis and hypertrophic pachymeningitis in the differential diagnosis can prevent significant morbidity including unnecessary surgical intervention and organ failure secondary to extensive fibrosis.


2021 ◽  
Vol 23 (1) ◽  
Author(s):  
Yanying Liu ◽  
Fei Yang ◽  
Xiying Chi ◽  
Yuxin Zhang ◽  
Jiangnan Fu ◽  
...  

Abstract Objective The growing utilization of needle biopsy has challenged the current pathology consensus of IgG4-related disease (IgG4-RD). The aims of this study were to identify the histological characteristics of needle biopsy and surgical specimens and evaluate the ability of needle biopsy in histological diagnosis of IgG4-RD. Methods Biopsies from patients who were referred to as IgG4-RD by the 2019 ACR/EULAR IgG4-RD classification criteria in Peking University People’s Hospital from 2012 to 2019 were re-evaluated. Typical histological features and diagnostic categories were compared between needle biopsy and surgical biopsy. Results In total, 69 patients met the 2019 ACR/EULAR classification criteria and 72 biopsies of them were re-evaluated. All cases showed lymphoplasmacytic infiltrate, while storiform fibrosis and obliterative phlebitis were only present in 35 (48.6%) and 23 (31.9%) specimens, respectively. Storiform fibrosis was more likely to be seen in retroperitoneum lesion (P = 0.033). Surgical biopsy showed significantly higher IgG4+ plasma cells/high-power field (IgG4/HPF) count (P < 0.01) and higher proportion of IgG4/HPF > 10 (P < 0.01). No significant difference was observed with regard to the ratio of IgG4+ plasma cells/IgG+ plasma cells (IgG4/IgG) (P = 0.399), storiform fibrosis (P = 0.739), and obliterative phletibis (P = 0.153). According to the 2011 comprehensive diagnostic criteria, patients who performed a needle biopsy were less likely to be probable IgG4-RD (P = 0.045). Based on the 2011 pathology consensus, needle biopsy was less likely to be diagnosed as IgG4-RD (P < 0.01), especially to be highly suggestive IgG4-RD (P < 0.01). Only 1/18 (5.6%) needle salivary specimens fulfilled the cutoff of IgG4/HPF > 100, which was significantly less than 15/23 (65.2%) of surgical ones (P < 0.01). Conclusions Needle biopsy shows an inferiority in detecting IgG4/HPF count but not in IgG4/IgG ratio, storiform fibrosis, and obliterative phlebitis. Compared with surgical samples, needle biopsy is less likely to obtain a histological diagnosis of IgG4-RD. A different IgG4/HPF threshold for needle biopsy of the salivary glands may be considered.


Breast Care ◽  
2018 ◽  
Vol 13 (5) ◽  
pp. 364-368 ◽  
Author(s):  
Doris Leithner ◽  
Benjamin Kaltenbach ◽  
Petra Hödl ◽  
Volker Möbus ◽  
Volker Brandenbusch ◽  
...  

Background: The management of intraductal papilloma without atypia (IDP) in breast needle biopsy remains controversial. This study investigates the upgrade rate of IDP to carcinoma and clinical and radiologic features predictive of an upgrade. Methods: Patients with a diagnosis of IDP on image-guided (mammography, ultrasound, magnetic resonance imaging) core needle or vacuum-assisted biopsy and surgical excision of this lesion at a certified breast center between 2007 and 2017 were included in this institutional review board-approved retrospective study. Appropriate statistical tests were performed to assess clinical and radiologic characteristics associated with an upgrade to malignancy at excision. Results: For 60 women with 62 surgically removed IDPs, the upgrade rate to malignancy was 16.1% (10 upgrades, 4 invasive ductal carcinoma, 6 ductal carcinoma in situ). IDPs with upgrade to carcinoma showed a significantly greater distance to the nipple (63.5 vs. 36.8 mm; p = 0.012). No significant associations were found between upgrade to carcinoma and age, menopausal status, lesion size, microcalcifications, BI-RADS descriptors, initial BI-RADS category, and biopsy modality. Conclusion: The upgrade rate at excision for IDPs diagnosed with needle biopsy was higher than expected according to some guideline recommendations. Observation only might not be appropriate for all patients with IDP, particularly for those with peripheral IDP.


2020 ◽  
pp. 003693302094126
Author(s):  
Nives Jonjić ◽  
Toni Valković ◽  
Danijela Veljković Vujaklija ◽  
Koviljka Matušan Ilijaš

Hodgkin lymphoma (HL) is an uncommon malignancy usually limited to the lymph nodes and lymphatic system while extranodal involvement is much less common than in non-Hodgkin lymphoma. The current report presents an unusual case of primary classical HL (cHL), nodular sclerosis type with mixed cellularity in buttock soft tissue of 78-year old man. Primary lymphoma of the gluteal muscle is a rare disease and primary cHL is even rarer. In addition, to this unusual extranodal presentation, the present case highlight a diagnostic challenge in fine-needle biopsy masquerading a low grade sarcoma, primarily myxoinflammatory fibrosarcoma or an inflammatory lesion. However, surgical biopsy and immunohistochemistry guided correct diagnosis that was of major interest for further successful treatment.


2020 ◽  
Vol 56 (9) ◽  
pp. 597-599
Author(s):  
Mónica Tello-Sánchez ◽  
María Soledad Rodríguez-Duque ◽  
Claudia Loidi-López ◽  
Juan Martín-Arroyo ◽  
Miguel Ángel González-Gay ◽  
...  

2000 ◽  
Vol 13 (4) ◽  
pp. 662-685 ◽  
Author(s):  
Stephen A. Klotz ◽  
Christopher C. Penn ◽  
Gerald J. Negvesky ◽  
Salim I. Butrus

SUMMARY The unique structure of the human eye as well as exposure of the eye directly to the environment renders it vulnerable to a number of uncommon infectious diseases caused by fungi and parasites. Host defenses directed against these microorganisms, once anatomical barriers are breached, are often insufficient to prevent loss of vision. Therefore, the timely identification and treatment of the involved microorganisms are paramount. The anatomy of the eye and its surrounding structures is presented with an emphasis upon the association of the anatomy with specific infection of fungi and parasites. For example, filamentous fungal infections of the eye are usually due to penetrating trauma by objects contaminated by vegetable matter of the cornea or globe or, by extension, of infection from adjacent paranasal sinuses. Fungal endophthalmitis and chorioretinitis, on the other hand, are usually the result of antecedent fungemia seeding the ocular tissue. Candida spp. are the most common cause of endogenous endophthalmitis, although initial infection with the dimorphic fungi may lead to infection and scarring of the chorioretina. Contact lens wear is associated with keratitis caused by yeasts, filamentous fungi, and Acanthamoebae spp. Most parasitic infections of the eye, however, arise following bloodborne carriage of the microorganism to the eye or adjacent structures.


1974 ◽  
Vol 2 (5) ◽  
pp. 366-369 ◽  
Author(s):  
T Kobayashi ◽  
Y Amenomori

In an open controlled trial, 100 patients with vaginitis were treated with clotrimazole vaginal tablets. Ninety-four patients were infected with Candida and six patients with trichomonas. Therapy was continued for six days in 80% of patients and for up to twelve days in 18%. Judged on clinical and mycological results at the end of therapy, 57 patients were cured and 25 patients improved (90·1% effective). No patients were made worse and side-effects were minimal. The trial showed that clotrimazole was a fast, safe and effective treatment for candida vaginitis. Insufficient patients had trichomonas vaginitis for a clear conclusion to be drawn.


2015 ◽  
Vol 16 (3) ◽  
pp. 231-235
Author(s):  
Roshan K. Verma ◽  
Sourabha Kumar Patro ◽  
M.R. Shivaprakash ◽  
Debajyoti Chaterjee ◽  
Naresh K. Panda

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