Lymphoplasmacytic Inflammatory Infiltrate Research Articles

A Case Report of Inflammatory Myofibroblastic Tumor Mimicking Leiomyosarcoma of the Uterus

Abstract Introduction/Objective Inflammatory myofibroblastic tumor (IMT) of the uterus is a rare entity that presents significant diagnostic challenges due to its infrequency and has the potential for being misdiagnosed as a leiomyoma, endometrial stromal tumor, or myxoid leiomyosarcoma, resulting in undertreatment or overtreatment. Methods/Case Report We present a case report of a 50-year-old woman with a history of anemia due to heavy menstruation caused by fibroids, who underwent hysterectomy. The concurrent pelvic wash cytology raised suspicious for myxoid leiomyosarcoma. Results (if a Case Study enter NA) Macroscopic examination revealed both well and poorly circumscribed nodules, ranging upto 4.7 cm in maximum dimension. Cut surfaces exhibited a spectrum of appearances, including tan-pink, whorled and rubbery, as well as soft, hemorrhagic, edematous, and mucinous areas with necrosis. Microscopically, the tumor exhibited myxoid spindle cell proliferation, in the background of lymphoplasmacytic inflammatory infiltrates. She was diagnosed with IMT metastasis to left fallopian tube, ovary, and omentum. Immunohistochemical (IHC) analysis further confirmed the diagnosis, showing strong and diffuse cytoplasmic positivity for ALK (anaplastic lymphoma kinase), caldesmon, and smooth muscle actin. This case underscores the critical importance of accurate diagnosis in guiding appropriate treatment strategies. Moreover, given the morphological overlap with other uterine tumors, the identification of ALK rearrangement emerges as a pivotal diagnostic marker, distinguishing IMT from its mimics such as leiomyoma, leiomyosarcoma and smooth muscle tumor of uncertain malignant potential (STUMP). Enhancing our understanding of the histomorphological features and ALK immunostaining of uterine IMT is essential for optimizing patient management and outcomes. Conclusion IMT in the female genital tract can mimic other more common benign and malignant tumors like leiomyoma, leiomyosarcoma, and endometrial stromal sarcoma. Familiarity with clinical and histologic features and the use of ALK immunostaining can be critical for correct diagnosis.

Inflammatory myofibroblastic tumors: recent progress and future of targeted therapy.

An inflammatory myofibroblastic tumor is a rare component of bone and soft-tissue sarcomas that has distinct pathological features as a lymphoplasmacytic inflammatory infiltrate. As is the case for other non-small round cell sarcomas, surgical resection remains the standard treatment strategy for inflammatory myofibroblastic tumors, but recurrence is possible. Concerning systemic therapy, the available data for conventional chemotherapy (such as those of doxorubicin-based regimens) are limited, and case reports of anti-inflammatory inflammatory myofibroblastic tumor treatments describe some degree of symptom relief and efficacy against tumor progression. However, as more information about cancer genomics accumulates, the potential for molecularly targeted therapies for inflammatory myofibroblastic tumors has become more promising. Approximately half of inflammatory myofibroblastic tumors harbor anaplastic lymphoma kinase (ALK) fusion genes, and the other half could have potentially targetable fusion genes or mutations such as ROS1, NTRK and RET; case reports demonstrating the clinical efficacy of treatments targeted to inflammatory myofibroblastic tumor have been published, as have several prospective clinical trials. Few drugs are approved for the treatment of inflammatory myofibroblastic tumor, and most of them were approved for tumor-agnostic indications. Drugs that could be used for pediatric indications and dosing in inflammatory myofibroblastic tumor have also not been established. To provide effective targeted therapy for rare diseases such as inflammatory myofibroblastic tumor, it is necessary to obtain clinical evidence by designing and performing clinical trials and to find a path toward regulatory approval.

Outbreaks of dermatopathy caused by Tephrosia noctiflora intoxication in Brazilian cattle

This study aims to update the knowledge concerning the intoxication by Tephrosia noctiflora in Brazilian cattle herds by reporting new cases of intoxication in lactating cows, their calves and bulls and highlight the epidemiology, clinical signs, pathogenesis, gross, and microscopic lesions. The morbidity and mortality of this intoxication in the farms studied was low. Gross lesions in all affected cattle consisted of dermatitis with hyperpigmentation, crusts, ulceration, erythema, and lichenification in the skin of limbs, ventral abdomen, perianal and perineal areas of lactating calves and adult cattle. Microscopically, the main lesion observed consisted of severe dermatitis with parakeratotic hyperkeratosis, papillated proliferation, and diffuse, accentuated lymphoplasmacytic inflammatory infiltrate in the epidermis and dermis. The presence of skin lesions mainly in the limbs and ventral abdomen of cattle implies the pathogenesis of intoxication is related to a primary contact dermatitis, and the occurrence of similar lesions on the skin of nursing calves reinforces this hypothesis. The putative toxins of T. noctiflora have been thought to be rotenoids. Additional work is needed to define better if these compounds are the main toxin responsible for the dermatopathy observed in these herds.

Leiomyoma-like Morphology in Metastatic Uterine Inflammatory Myofibroblastic Tumors

Uterine inflammatory myofibroblastic tumors (IMTs) are rare mesenchymal neoplasms that frequently harbor ALK gene rearrangements and have a low risk of metastasis. We reported 3 of these tumors mimicking the appearance of leiomyoma in their recurrence. These patients were 34, 43, and 45 years old. Two uterine tumors demonstrated classic morphology, with combined myxoid, compact fascicular, and hyalinized patterns and spindled cells with bipolar cytoplasmic processes, moderate atypia, and lymphoplasmacytic inflammatory infiltrates. The third had a “leiomyoma-like” appearance, with fascicles of plump spindled cells and a sparse lymphoplasmacytic infiltrate. ALK immunohistochemistry was positive in all the tumors, and all demonstrated ALK rearrangements using fluorescence in situ hybridization (n = 2) and/or RNA sequencing (n = 2). Two classic IMTs recurred at 3 and 50 months in the lung and abdomen, respectively, and recurrent tumors had a “leiomyoma-like” appearance, with 0 and 1 mitosis per 10 high-power fields, no inflammation in 1, and a sparse lymphocytic infiltrate in the other. ALK was positive in both tumors; 1 with available tissue showed an IGFBP5::ALK fusion using RNA sequencing. The third patient, who had a “leiomyoma-like” uterine tumor, experienced multiple recurrences, first in the abdomen at 100 months showing a similar appearance. Subsequent recurrence at 105 months showed transmural invasion of the sigmoid colon and a similar microscopic appearance but with the addition of infiltrative borders, moderate cellularity, mild-to-moderate atypia, and 10 mitoses per 10 high-power fields. Both recurrences were positive for ALK, and RNA sequencing revealed the same ACTG2::ALK fusion transcript identified in the primary tumor. The patient was treated with crizotinib, resulting in prolonged clinical remission, with no evidence of disease at 168 months from the initial surgery. Although “leiomyoma-like” uterine IMTs have been recently described, to our knowledge, this is the first report of recurrence of these tumors and the first report of a “leiomyoma-like” appearance in the recurrences of conventional uterine IMTs. A low threshold for performing ALK immunohistochemistry on recurrent uterine tumors can identify patients who may benefit from tyrosine kinase inhibitors.

Síndrome do olho seco em Shih Tzu: aspectos clínicos e morfológicos da superfície ocular nas diferentes fases da ceratoconjuntivite seca

ABSTRACT: This study analyzed clinical and cyto-histomorphological parameters of the ocular surface of Shih Tzu dogs, non-carriers and carriers of quantitative keratoconjunctivitis sicca (KCS) at different stages. Thirty-five eyes from 23 male and female Shih Tzu dogs between two and eight years were evaluated in four groups: control group (CG - without KCS), mild KCS group (KCS1), moderate KCS group (KCS2), and severe KCS group (KCS3). Most clinical variables among KCS carrier groups worsened at the more advanced stages of the disease, with a negative correlation between the Schirmer tear test (STT-1) and tear film break-up time (TBUT). Squamous metaplasia, lymphoplasmacytic inflammatory infiltrate, and decrease in conjunctival goblet cells on histopathological examination comprised disease severity parameters. Quantitative KCS non-carried Shih Tzu dogs have qualitative and tear film distribution changes. The cytomorphological exam is limited to evaluating the inflammatory infiltrate and quantifying conjunctival goblet cells. However, intermediate epithelial cells were higher in healthy eyes compared to eyes with KCS in Shih Tzu dogs. Also, moderate and severe KCS carrier Shih Tzu dogs have qualitative dysfunction of the tear film. KCS in Shih Tzu dogs is common and chronic and may be responsible for the loss of vision of these animals. Early identification of the disease and routine evaluation can improve these dogs’ quality of life and ocular health.

OA16 A great imitator of scleroderma

Introduction/BackgroundEosinophilic fasciitis is a rare disease of idiopathic aetiology, which can often mimic scleroderma in presentation and has been associated with haematological diseases. We present a case of eosinophilic fasciitis where the diagnosis was delayed due to the attribution of skin manifestations to scleroderma. This case highlights the importance of careful history and examination to recognize these differential diagnoses, prevent delay and improve outcome.Description/MethodA 69-year-old gentleman was referred to the Rheumatology clinic with suspected diffuse cutaneous systemic sclerosis. He presented with a 9-month history of skin tightening, pain and swelling affecting his legs, finding it difficult to fully extend at the knees, to drive or walk. His symptoms progressed overtime with skin changes spreading to all four extremities, abdomen and back. He reported weight loss of 12Kg associated with early satiety. He also experienced intermittent numbness of his hands. There were no respiratory symptoms, dysphagia or Raynaud’s phenomenon reported. His medical history includes atrial fibrillation and asbestos exposure. Currently on Apixaban 5mg/daily and Bisoprolol 2.5mg/daily.Physical examination revealed diffuse brown hyperpigmentation, erythema and thickened skin on his upper and lower limbs, abdomen and back, it was difficult to crease the skin and there was ‘peau d’orange’ appearance in his forearms. His face and hands were spared. Phalen's and Tinel’s tests were negative.Prior to clinical review he was investigated for paraneoplastic disease: a CT chest, abdomen and pelvis showed an incidental cyst in the thymus and emphysema with no underlying malignancy. Laboratory tests showed normocytic normochromic anaemia with haemoglobin 112 g/L and eosinophilia 1.5 x 109/L, elevated erythrocyte sedimentation rate 62mm/h, CRP 57mg/L, polyclonal hypergammaglobulinaemia IgA 4.3g/L and IgG 24.85g/L. The myeloma screen were negative.The absence of Raynaud’s, and the distribution of skin changes sparing his face, hands and feet, eosinophilia and raised inflammatory markers, made the diagnosis of eosinophilic fasciitis. Histological analysis of a full thickness skin biopsy confirmed patchy lymphoplasmacytic inflammatory cell infiltrate within the fascia and spilling into subcutis, with eosinophils.Subsequently, he was commenced on prednisolone 40mg/daily reducing regimen and Methotrexate 20mg/weekly, with a good response and regression of the swelling and pain. He is now able to walk without difficulty.Discussion/ResultsEosinophilic fasciitis is rare. Symptoms can be similar to those of scleroderma, sometimes considered a variant. The exact aetiology is unknown; however, the literature mentions a potential paraneoplastic, inflammatory, infectious or physical trigger, leading to an abnormal response with accumulation of eosinophils in different tissues. Consequently, this may release transforming growth factor beta to activate fibroblasts, increasing expression of other factors in the connective tissue, resulting in fibrosis.This case highlights that eosinophilic fasciitis present with hardening and thickening of the skin and surrounding tissue similar to that of scleroderma, however, lacks Raynaud’s phenomenon, sclerodactyly. Another striking features from his examination which helped us distinguishing from scleroderma was that his skin changes spare his face, hands and feet.Carpal tunnel syndrome is described in the literature which can progress if untreated.Typical blood tests findings documented in the literature include eosinophilia in 80% of the cases, elevated ESR and C-reactive protein (CRP), polyclonal hypergammaglobulinaemia, are usually present, however, eosinophilia may be transient, therefore normal eosinophil count does not exclude the disease. ANA and Rheumatoid factor can be positive in up to 10 % of the cases.MRI of the extremities to look for thickening of the fascia has been used in other cases, however, it was not required in our case because our Orthopaedics colleagues helped us with performing an urgent full thickness biopsy.Recommended treatment includes high dose steroids and immunosuppressive medication such Methotrexate is most often used for severe or resistant cases.The prognosis is good, however if treatment is delayed patients can develop joint contractures in 85% of patients and skin hardness may persist.In conclusion, careful history and examination is essential in differentiating scleroderma from the scleroderma-mimics especially when there is an atypical distribution, no sclerodactyly and no autoantibodies.Key learning points/Conclusion1. This case suggest that clinicians should be aware that eosinophilic fasciitis typically spare the hands, feet and face.2. Eosinophilia may be transient, therefore normal count does not exclude the disease.3. Recognizing this rare but great imitator of scleroderma set the stage for prompt therapy in either condition and reduce morbidity.

SCLEROSING POLYCYSTIC ADENOSIS OF THE MINOR SALIVARY GLANDS: A CASE REPORT

Sclerosing polycystic adenosis is a rare benign salivary gland lesion that more commonly involves the parotid. A 65-year-old male patient, without systemic changes, presented swelling in the lower lip for 18 months. Clinical examination revealed a painless submucosal nodule in the lower labial mucosa with a 5-mm diameter. The diagnostic hypotheses were mucocele and hyperplastic minor salivary gland, and total surgical excision was performed. Histopathologic examination showed a well-circumscribed lobular proliferation of ectatic ductal structures with cystic dilatation and periductal sclerosis. The ductal spaces were lined by flattened to cuboidal epithelium containing squamous and apocrine-like metaplasia and occasional mucin. The perilesional region showed minor salivary glands with an infiltration area along with focal lymphoplasmacytic inflammatory infiltrate. The final diagnosis of sclerosing polycystic adenosis was made based on clinical and histopathologic examination. The patient has been followed up for 6 months and is currently free of the lesion.

OSTEOMYELITIS WITH PROLIFERATIVE PERIOSTITIS ASSOCIATED WITH A SECOND PRIMARY MOLAR: CASE REPORT

Osteomyelitis with proliferative periostitis, also called Garre's osteomyelitis, is a rare chronic process characterized by periosteal proliferation reactive to the presence of inflammation. A 4-year-old male patient presented with facial asymmetry on the right side with 3 months of evolution and diffuse pain. Clinical examination revealed an indurated and hyperemic facial tumor and severe trismus, in addition to a decayed second primary molar. Imaging exams revealed periosteal thickening and subperiosteal new bone formation with radiopaque lamination associated with tooth 85. Treatment involved antibiotic therapy, drainage of purulent secretions, and removal of the decayed tooth. After incisional biopsy, microscopic examination showed interconnected bone trabeculae, arranged in parallel and surrounded by osteoblasts. Multinucleated giant cells similar to osteoclasts, fibrous connective tissue with focal of lymphoplasmacytic inflammatory infiltrate, and scattered polymorphonuclear cells confirmed the diagnosis of osteomyelitis with proliferative periostitis. After 7 days there was a decrease of the edema and recovered mouth opening.

Patologia da periodontite crônica em ovinos

ABSTRACT: Periodontitis is an inflammatory process of infectious origin affecting the teeth and their supporting structures, causing significant economic losses and reducing animal welfare. Bacteria in the gingival biofilm are one of the main factors in initiating inflammatory lesions. Bacteria act directly on tissues or indirectly through substances that cause tissue damage. Studies on the etiopathogenesis of periodontitis in Brazilian sheep herds are scarce. The present study aimed to characterize histologically periodontal lesions of culled sheep from the Brazilian breed, Santa Inês. Periodontal lesions, such as periodontal pockets containing plant tissue and bacteria, replacement of the periodontal ligament by connective tissue and inflammatory cells, superficial pustules, hydropic epithelial degeneration, and epithelium hyperplasia, were observed. Submucosal changes were characterized by granulation tissue, edema, swelling of the endothelial cells, bacteria, and predominantly perivascular lymphoplasmacytic inflammatory infiltrate. In the alveolar bone, osteoclastic resorption and bone apposition were observed. This study revealed subacute to chronic inflammation, alveolar bone resorption, and cortical bone apposition in ovine periodontitis. Thus, these findings can contribute to the evolution of knowledge about the etiopathogenesis of ovine periodontitis and, possibly, the development of measures to control the disease.

Epstein-Barr Virus Negative Lymphoepithelioma-Like Cholangiocarcinoma in a Patient of European Descent: A Report of a Case

Abstract Lymphoepithelioma-like carcinoma in the setting of cholangiocarcinoma in the absence of underlying Epstein-Barr virus is an incredibly rare entity. However, all of the reports that exist, which are few and far between, describe this vignette in patients of Far Eastern origin. We present the first case report of Epstein-Barr virus–negative lymphoepithelioma-like carcinoma–associated cholangiocarcinoma occurring in a patient of European descent. The 62-year-old woman described here presented with nonspecific symptoms of lethargy, low-grade pyrexia, and raised white blood cell count. Subsequent investigations revealed a large hepatic mass with subsequent hepatectomy yielding a large tumor containing poorly differentiated adenosquamous cholangiocarcinoma alongside a rich infiltrate of lymphocytes and lymphoplasmacytic inflammatory infiltrate. Epstein-Barr virus in situ hybridization was negative in both the malignancy and surrounding tissues. Reflecting its rarity, the pathogenesis of this specific malignancy is poorly understood, as also are the treatment and prognosis.

Histological Lesions and Replication Sites of PCV3 in Naturally Infected Pigs.

Simple SummaryDiagnosing porcine circovirus type 3 (PCV3) is a challenge in pig production. Although the virus has been recently isolated, the patterns of PCV3-associated histological lesions are still to be elucidated. The present study describes the association of PCV3 mRNA by in situ hybridization within histological lesions and PCV3 DNA detected by real-time PCR in naturally infected pigs. The main histologic lesions associated with PCV3 mRNA detection were lymphoplasmacytic myocarditis and lymphoplasmacytic interstitial pneumonia, in heart and lung, respectively. Our findings offer robust guidance of microscopic lesions associated with PCV3, which may have a key role in PCV3 diagnosis.Porcine circovirus type 3 (PCV3) has been recently described as a potential cause of abortions and systemic vasculitis in pigs. Although the virus has been detected by real-time PCR in several porcine tissues from countries worldwide, PCV3-associated diseases have not been satisfactorily clarified. The objective of this study was to investigate the association between the presence of PCV3 mRNA detected by in situ hybridization (ISH) within histological lesions and PCV3 DNA detected by real-time PCR in naturally infected pigs. A total of 25 PCV3 PCR-positive cases were analyzed. Formalin-fixed tissues from these cases were evaluated for histologic lesions and for ISH-RNA positive signals for PCV3. The most frequent tissue type with histopathologic lesions was heart, 76.2%, with lymphoplasmacytic myocarditis and epicarditis as the most frequent lesions observed. Lymphoplasmacytic interstitial pneumonia was also a frequent finding, 47.6%. There were also lesions in kidney, liver, spleen and lymph nodes. PCV3-ISH-RNA positive signals were mostly observed in association with lymphoplasmacytic inflammatory infiltrate in various tissues, including arteries. Based on our results, the minimum set of specimens to be submitted for histopathology and mRNA in situ hybridization to confirm or exclude a diagnosis of PCV3 are heart, lung and lymphoid tissues (i.e., spleen and lymph nodes), especially for differential diagnosis related with PCV2-associated diseases.

Distinct Histopathologic Features of Complicated Sinusitis.

Sinusitis complicated by intracranial or orbital extension can be life-threatening and require emergent intervention. Histologic features of complicated sinusitis have yet to be determined and may have significant implications for understanding pathophysiology. A structured histopathology report was utilized to analyze sinus tissue extracted during functional endoscopic sinus surgery (FESS). A total of 13 histopathology variables were compared between patients with complicated sinusitis (CS), CRS without nasal polyps (CRSsNP), and CRS with nasal polyps (CRSwNP). About 24 CS, 149 uncomplicated CRSsNP, and 191 uncomplicated CRSwNP patients were analyzed. Nasal tissue from CS and CRSwNP patients demonstrated similar levels of overall inflammation (66.7% vs. 69.6% with moderate/severe inflammation, P = .466). Relative to CRSsNP, CS patients showed significantly greater overall inflammation (66.7% vs. 41.6%, P = .019). CS patients demonstrated significantly fewer eosinophils per high power field (eos/HPF) and eosinophil aggregates compared to CRSwNP patients (20.8% vs. 70.7% with 5+eos/HPF, P < .0001; 4.2% vs. 33.5%, P < .0001). Relative to CRSsNP patients, CS patients demonstrated enhanced neutrophil infiltration (45.8% vs. 28.0%, P = .011). About 91.7% of CS patients demonstrated a lymphoplasmacytic predominant inflammatory infiltrate, compared to 69.8% of CRSsNP and 62.8% of CRSwNP patients (P < .0001). Significant histopathological differences were evident in patients with CS, CRSsNP, and CRSwNP. CS patients did not perfectly fit either a CRSsNP or CRSwNP profile, underscoring the importance of delineating the histopathological features of CS. This study offers insight into the histologic aspects of CS, providing initial evidence that it is an aggressive neutrophilic inflammatory process.

Ivermectin reduces in vivo coronavirus infection in a mouse experimental model

The objective of this study was to test the effectiveness of ivermectin for the treatment of mouse hepatitis virus (MHV), a type 2 family RNA coronavirus similar to SARS-CoV-2. Female BALB/cJ mice were infected with 6,000 PFU of MHV-A59 (group infected, n = 20) or infected and then immediately treated with a single dose of 500 µg/kg ivermectin (group infected + IVM, n = 20) or were not infected and treated with PBS (control group, n = 16). Five days after infection/treatment, the mice were euthanized and the tissues were sampled to assess their general health status and infection levels. Overall, the results demonstrated that viral infection induced typical MHV-caused disease, with the livers showing severe hepatocellular necrosis surrounded by a severe lymphoplasmacytic inflammatory infiltration associated with a high hepatic viral load (52,158 AU), while mice treated with ivermectin showed a better health status with a lower viral load (23,192 AU; p < 0.05), with only a few having histopathological liver damage (p < 0.05). No significant differences were found between the group infected + IVM and control group mice (P = NS). Furthermore, serum transaminase levels (aspartate aminotransferase and alanine aminotransferase) were significantly lower in the treated mice than in the infected animals. In conclusion, ivermectin diminished the MHV viral load and disease in the mice, being a useful model for further understanding this therapy against coronavirus diseases.

The type of interstitial inflammatory cell infiltrate and the severity of glomerular injury; an underestimated morphologic correlation

Introduction: The interrelation between the type of interstitial inflammatory cells and the severity of glomerulonephritis was not considered in most of the relevant medical literature. Objectives: To investigate the relationship between the type of interstitial cell infiltrate and the morphological severity of glomerular injury in different types of proliferative glomerulonephritides. Patients and Methods: We retrospectively reviewed 138 native kidney biopsies and assessed the relationship between the type of interstitial inflammatory cell infiltrate and the severity of glomerular injury in the form of cellular crescents and fibrinoid necrosis. Results: The predominant type of interstitial inflammatory cell infiltrate was lymphocytic, noted in more than half of the cases. Lymphoplasmacytic inflammatory cell infiltrate was the second most common type which observed. Fifty-five of patients had inflammation in areas of fibrosis. Cellular/fibrocellular crescents were observed in 44% of cases, and fibrinoid necrosis in 30% of cases. As compared to the ‘lymphocytic’ group, patients in the ‘lymphoplasmacytic’ group had ~3 times higher probability of presenting with crescents and fibrinoid necrosis. Conclusion: Our study highlights the significance of morphological correlations that may predict the severity of glomerular injury. Such findings would be helpful in limited or inadequate renal biopsy samples where the pathologist can alert the clinician, in the appropriate clinical context, to the possibility of having crescents and/or necrotizing lesions in the unsampled glomeruli.

An Overview of Immunoglobulin G4-related Ophthalmic Diseases Accompanied by a Case Report

Immunoglobulin G4-related diseases (IgG4-RDs) are immune-mediated, fibroinflammatory conditions that are characterised by affected organ enlargement, lymphocyte, plasma cell infiltration (determined with IgG4-positive plasma cells) and serum IgG4 level elevation. When the disease affects ocular adnexal tissues (such as the lacrimal gland, extraocular muscles, trigeminal nerve branches and orbital fat), it is called IgG4-related ophthalmic disease (IgG4-ROD). The diagnosis of IgG4-ROD is made by physical examination, biochemical findings and histopathological evaluation. It is characterized by elevated serum IgG4 levels and distinctive histopathological features, including IgG4+ plasma cell infiltration, the presence of fibrosis in the storiform pattern and the presence of a dense lymphoplasmacytic inflammatory infiltrate, including eosinophils. It is rare in the ocular region and cause diagnostic confusion. There is a high probability of misdiagnosis when the disease is not recognized. Here, a rare case of eye involvement is presented and IgG4-RDs are discussed with the literature.

1546. Update in Syphilis Proctitis: Insights from a Case Series and Literature Review

BackgroundIn the US, syphilis infections have increased 71% since 2014. Proctitis is a rare manifestation of early syphilis transmitted through anal intercourse. We suspect that its misdiagnosis results from physician under-awareness and thus we present the largest case analysis to date of syphilis proctitis.MethodsWe searched PubMed and Scopus for articles describing cases of proctitis in which Treponema pallidum was a likely causative pathogen based on serologies, and/or organism-specific staining of anorectal biopsy specimens. Furthermore, we conducted chart review to identify cases of syphilis proctitis diagnosed within our health center from 2011-2019. Pertinent data were extracted from the articles and medical records and analyzed to provide a summative account.Results53 cases of syphilis proctitis were identified in 38 articles. 7 additional cases were diagnosed at our institution, totaling 60 cases. All cases of syphilis proctitis were described in individuals of male sex assignment at birth. The age at diagnosis ranged from 15 to 73 years (average 39 years). In 48 cases (92%) men endorsed sex with men. In 27 cases (56%) individuals were HIV co-infected. Syphilis proctitis presented most commonly with hematochezia (68%) and anal pain (48%). The most common physical exam findings were rectal mass (38%), lymphadenopathy (33%), and rash (31%). Non-treponemal titers averaged 1:60 (range 1:2-1:1024). Endoscopy was performed in 52 cases and most commonly showed anorectal mass (42%) and anorectal ulcer (35%). In 38 cases (68%), histopathology revealed a chronic lymphoplasmacytic inflammatory infiltrate, and in 14 of these cases (37%), prominent plasma cells were described. In 24 cases (77%), treponema immunohistochemical stain revealed spirochetes.ConclusionSyphilitic proctitis should be suspected in boys and men presenting with lower gastrointestinal symptoms. Histopathology, while suggestive, is not pathognomonic, and serology and specific tissue staining are required to make the diagnosis. Given overlapping symptoms and histology with inflammatory bowel disease, the diagnosis may be delayed resulting in personal and public health consequences. A sexual history should be routinely elicited and further testing for syphilis pursued if exposure is suspected.Disclosures All Authors: No reported disclosures

S2995 Stomach This: A Novel Type of Gastritis

INTRODUCTION: Atrophic gastritis is often due to autoimmune (AIG) and environmental causes (especially H. Pylori infection).1 AIG typically inflames the stomach body, fundus and spares the antrum while environmental causes affect the antrum.2 We present a novel type of gastritis, autoimmune atrophic pangastritis (AIAP); only 15 cases have been reported since being formally recognized in 2006.1 CASE DESCRIPTION/METHODS: A 76 year old woman with recurrent basal cell carcinoma, hypothyroidism and rosacea presents with 2 months of nausea, vomiting, and 10 pound weight loss. Physical exam showed mild epigastric tenderness. CBC, CMP, and a lipase are unrevealing. See Figures for endoscopy and biopsy findings. DISCUSSION: Autoimmune atrophic pangastritis (AIAP), a rare entity with only 15 cases reported since being formally described in 2006, is primarily diagnosed on characteristic histology: 1) intense mucosal lymphoplasmacytic inflammatory infiltrates, persisting even to severe glandular atrophy, 2) pangastric distribution involving both body and antrum, 3) no H. pylori association, and 4) lack of neuroendocrine hyperplasia unlike traditional forms of AIG which stems from hypergastrinenemia from parietal cell loss.1 Clinical presentations range from nonspecific symptoms of nausea, vomiting and abdominal pain to diarrhea, fat malabsorption and malnutrition. Endoscopically, patients vary from normal mucosa to multiple ulcers.1 Risk factors include female sex, systemic autoimmune, and connective tissue diseases.2 Though the etiology is unclear, the involvement of multiple cell lineages gives a theoretical risk of neoplasia, particularly gastric adenocarcinoma.3 Thus, early identification and treatment with immune suppression is key, often resolving symptoms and can lead to complete histologic resolution.3 The patient was started on prednisone and low dose azathioprine and responded with improved appetite and weight gain. Prednisone was tapered off and she continued azathioprine monotherapy. The patient’s course was complicated by recurrent basal cell carcinoma, which she had prior to initiating thiopurines. Azathioprine was continued for four years total before weaning off due to long-standing clinical remission. The patient’s AIAP remains in clinical remission off all therapy after two years. There is a plan to repeat an upper endoscopy this year.Figure 1.: Endoscopy showed erythema, congestion, friability, and diffuse atrophic mucosa throughout the stomach (Figure 1).Figure 2.: Gastric body biopsies also had chronic inflammation with glandular dropout on H&E stain (Figure 2).Figure 3.: No H. Pylori or neuroendocrine hyperplasia identified on the gastric body biopsies with IHC stain and no neuroendocrine hyperplasia on synaptophysin stain (Figure 3).

18F-FDG PET/CT in Hyalinized Cholecystitis.

Hyalinizing cholecystitis is a rare type of chronic cholecystitis. Moderate patchy transmural especially perivascular lymphoplasmacytic inflammatory cell infiltration is observed in the gallbladder wall. We present the 18F-FDG PET/CT and MRI findings of this rare subtype of chronic cholecystitis. Hyalinizing cholecystitis should be kept in mind in the differential diagnosis of gallbladder wall thickening with intense 18F-FDG uptake.

The histopathological spectrum of Melkersson-Rosenthal syndrome: Analysis of 47 cases.

Melkersson-Rosenthal syndrome (MRS) is a rare disease characterized by the triad of recurrent orofacial edema, relapsing facial paralysis and plicated tongue. Histopathological features of MRS have not been extensively analyzed. This study investigated the histopathological aspects of oral lesions from 47 MRS patients. The most common biopsy site was the upper lip, followed by the lower lip, gingiva and palate. The most important findings were ill-defined and well-formed granulomas. Lymphoplasmacytic inflammatory infiltrate was seen in early and late stages of MRS. Edema, fibrosis, vasodilatation and congestion were the most common finding in the lamina propria. Gingival and palate exams also demonstrated granulomatous infiltrates. Regarding the evolution time of the disease, we demonstrated that, in initial phases, there is a lymphoplasmacytic inflammatory infiltrates, followed by a granulomatous infiltrate and, subsequently, fibrosis. Histopathological examination of oral lesions is helpful for the diagnosis of MRS; the absence of granulomatous inflammation does not exclude the diagnosis of syndrome. Clinical and histopathological analysis of the rare gingival and palate lesions is important, since all histopathological findings of the disease were detected in these sites.

Calcifying Fibrous Tumor of the Jejunum in a 27-year-old Primigravid: A Case Report

The most common mesenchymal tumors of the gastrointestinal tract are gastrointestinal stromal tumors (GIST) and smooth muscle neoplasms; however, other soft tissue tumors may also present in the intestines and cause diagnostic dilemmas. We report the case of a 27-year old primigravid, with no known complications, who underwent cesarean section for cephalopelvic disproportion. Intraoperatively, a well-demarcated, solid mass measuring 1.5 x 1.0 x 0.7 cm was noted at the jejunum. The patient underwent segmental resection of the mass. Microscopic examination of the mass reveals a non-encapsulated, solid mass composed of bland spindle cells and dense, hyalinized collagen in whorls and bundles. Dystrophic calcifications and a lymphoplasmacytic inflammatory infiltrate are seen within the collagen bundles. Immunohistochemical staining with desmin, CD117, and DOG-1 was done, which are all negative. The case was signed out as calcifying fibrous tumor (CFT). Inclusion of CFTs in the differential diagnoses for mesenchymal tumors of the gastrointestinal tract is important, as these neoplasms are benign and have an excellent prognosis.