Good Clinical Response Research Articles

Perimandibularna metastaza karcinoma rektuma - prikaz slučaja

Introduction. Colorectal cancer is the third most common cancer and metastatic disease is an important and frequent clinical problem. Metastases in the oral and maxillofacial region are rare, comprising 1-2% of all malignant lesions, and clinically resemble common benign conditions. Case report. A 59-year-old woman diagnosed with rectal cancer developed perimandibular lesion as a first sign of metastatic process. Metastasis manifested clinically during the curative intent treatment of rectal cancer (preoperative chemo-irradiation) as a rapidly growing tumefaction close to the mandible angle. After the biopsy and histopathological examination of the lesion that clinically resembled abscess, metastasis of rectal cancer was diagnosed. Incidentally, a cerebral metastasis was diagnosed as well. Although it was oligometastatic rectal cancer, surgical treatment was not indicated due to extensive infiltrative characteristics of the perimandibular lesion. The patient was treated with chemotherapy, with good clinical response. Biological therapy was not available at that moment. Cerebral metastasis was treated with stereotactic neuroradiosurgery with gamma knife. Unfortunately, the patient died from cerebrovascular insult. Conclusion. Lesions in the oral and maxillofacial region may be the first manifestation of metastatic disease. Since early diagnosis of metastatic process has prognostic implications, any new formation in the oral and maxillofacial region in a patient with colorectal cancer requires cautious observation and histological examination.

Preferences Regarding Breast Surgery Omission Among Patients With Breast Cancer Who Receive Neoadjuvant Chemotherapy.

Currently, several ongoing prospective studies are investigating the safety of breast surgery omission in patients with breast cancer who are exceptional responders to neoadjuvant chemotherapy. However, there is little information about the preferences of these patients regarding omission of breast surgery. We conducted a questionnaire survey to assess preferences regarding omission of breast surgery among patients with breast cancer who had human epidermal growth factor receptor 2-positive or estrogen receptor-negative tumors and good clinical response after neoadjuvant chemotherapy. Patients' estimation of the risk of ipsilateral breast tumor recurrence (IBTR) after definitive surgery or breast surgery omission was also assessed. Of 93 patients, only 22 (23.7%) said they would omit breast surgery. Under the scenario of omitting breast surgery, the 5-year IBTR rate estimated by patients who said they would omit breast surgery was significantly lower (median, 10%) than the rate estimated by patients who preferred undergoing definitive surgery (median, 30%) (p=0.017). The proportion of our surveyed patients who were willing to omit breast surgery was low. Patients who said they preferred to omit breast surgery overestimated the 5-year IBTR risk.

Brain Abscess after COVID-19: Case Report

As the COVID-19 pandemic progresses, complications and unusual presentations of the disease have been described. Among them, the involvement of distinct parts of the neuroaxis. We report a rare case of brain abscess in elderly after SARS-Cov-2 infection readmitted in our health unit. Patient was treated with ceftriaxone, metronidazole and vancomycin with good clinical and therapeutic response. The satisfactory conduct of the case was only possible by the involvement of a multiprofessional team, which sought early diagnosis, surgical intervention and adequate duration of treatment.

How Can Oncoplastic Breast Surgery Contribute to the Management of Locally Advanced Breast Cancer in Sub-Saharan Africa?

Because of resource constrains in sub-Saharan African countries, breast-conserving surgery (BCS) has no role in patients with locally advanced breast cancer (LABC) following neoadjuvant chemotherapy (NACT), and mastectomy remains the standard surgical treatment for these patients. The first objective of the study was to assess the safety of oncoplastic BCS in patients with LABC who showed good clinical response to NACT in a breast center with enhanced level of resources in Sudan. The second objective was to assess the cosmetic outcome. Two hundred and fifty patients with LABC were treated with NACT at Khartoum Breast Care Center during the period 2013-2019. Out of this, 52 patients were surgically treated with oncoplastic breast-conserving surgery. Kaplan-Meier curve was used to calculate the survival rates. The cosmetic outcome was subjectively assessed by the Harris scale. The median follow-up period was 53 months. The 3- and 5-years distant metastasis-free survival rates were 92.9% and 82.2%, respectively. One patient developed regional recurrence, and 6 patients developed distant metastasis. Eighty percentage of patients were admitted to have good to excellent cosmetic outcome. This Sudanese experience showed that oncoplastic BCS is oncologically safe and aesthetically satisfactory in patients with LABC who demonstrated good clinical response to NACT in a setting with enhanced levels of resources for breast cancer care.

Polymyalgia rheumatica presenting as sternoclavicular arthritis: a case report

BackgroundPolymyalgia rheumatica and giant cell arteritis are systemic inflammatory conditions of the elderly. Polymyalgia rheumatica classically presents as a bilateral proximal muscle pain and stiffness syndrome. Biceps tenosynovitis is the commonest pathology in polymyalgia rheumatica. However according to literature, erosive sternoclavicular arthritis is a rare association of polymyalgia rheumatica. Giant cell arteritis is an inflammatory granulomatous arteritis predominantly involving large cerebral arteries. Thus, its classic clinical presentation includes severe headache with scalp tenderness, jaw claudication, and sudden painless loss of vision. Urological manifestations (prostatic vasculitis and epididymo-orchitis) were seldom reported in giant cell arteritis.Case presentationA 53-year-old Sinhalese man presented with progressive right-sided shoulder joint pain and neck pain associated with constitutional symptoms and episodic generalized headache. Examination revealed restricted movements of the right shoulder joint with nontender pulsatile bilateral temporal arteries. Blood testing showed elevated erythrocyte sedimentation rate and C-reactive protein. Color Doppler ultrasound of the superficial temporal artery revealed “halo sign.” The temporal artery showed infiltration of mononuclear cells in the arterial media and adventitia. Computed tomography revealed right sternoclavicular arthritis with incidental finding of ureteric stricture. The patient was treated with high-dose oral prednisolone, and good clinical and biochemical response was observed during follow-up.ConclusionPolymyalgia rheumatica–giant cell arteritis may rarely present as erosive sternoclavicular arthritis as the initial manifestation, mimicking many rheumatological conditions. Urological involvement such as ureteric strictures may be rare associations of primary systemic vasculitis. A high degree of suspicion combined with targeted investigations would allow early identification the polymyalgia rheumatica–giant cell arteritis syndrome in the presence of atypical manifestations, leading to improved patient outcomes.

Autoimmune Encephalitis With Ganglionic a3-AChR Antibodies

Objective To describe a case of autoimmune encephalitis presented as new onset refractory nonconvulsive status epilepticus and autonomic failure caused by alpha-3 ganglionic acetylcholine receptor autoantibodies (a3-AChR Ab). Background Autoimmune autonomic ganglionopathy (AAG) is rare acquired dysautonomia in adults due to a3-AChR Ab. A patient typically presents with subacute autonomic dysfunction, and encephalitis has not been reported yet. Design/Methods NA. Results A 60 years old female presented with progressive worsening of mental status for two weeks. Vital signs were normal. The patient was somnolent, nonverbal, with tonic non-reactive pupils on the exam. CT head showed multiple subcortical hypodensities. MRI brain demonstrated T2-FLAIR hyperintense lesions in bifrontal lobes sparing the U-fibers, with abnormal leptomeningeal/pial perivenular type enhancement. MRI C/T spine, CT chest/Abd/pelvis were negative. Although serum and CSF testing showed inflammatory markers, meningoencephalitis and autoimmune encephalitis panel were negative, except for the serum Paraneoplastic panel was positive with high titer a3-AChR Abs. EEG showed nonconvulsive status epilepticus, which required management with three antiseizure medications. Initially, the patient had little response to empirical pulse methylprednisolone therapy. Later showed good clinical response with plasmapheresis. Later on she presented with nonobstructive small bowel obstruction, resolved with another pulse steroid therapy, discharged on dexamethasone taper. On a three-month follow-up, the patient was in near clinical remission, supported with resolved lesions on a repeat MRI. She never relapsed after two years of follow-up. Conclusions Studies have shown that a3-AChR Abs can result in diverse neurological manifestations, mainly dysautonomia. Our patient presented with encephalitis, dysautonomia, and refractory nonconvulsive status epilepticus. Scans suggested diffuse leukoencephalopathy, patient was found to have high 3-ACHR antibody seropositivity. Initially, the patient was steroid-resistant but showed clinical improvement with plasmapheresis. We have described an interesting case of encephalitis likely associated with a3-AChR Ab that has not been previously described. More studies are required to confirm this association.

Successful DWEK in persistent corneal edema secondary to subacute unexplained endotheliitis: 6‐month follow‐up

AbstractPurpose: To present a case of persistent corneal edema after endothelial disease of unknown cause and with a severe decrease in the endothelial count and clinical improvement after DWEK (descemetorhexis without endothelial keratoplasty).Methods: A 61‐year‐old immunocompetent male patient, with no relevant ophthalmological or systemic history, who after multiple episodes of subacute endotheliitis with retrokeratic precipitates and diffuse iridian atrophy in his right eye, showed central persistent corneal edema, secondary to a marked decrease in endothelial count and severe decrease in best‐corrected visual acuity (0.2) after several months of inflammatory stability.Results: During flare‐ups of inflammation, treatment with corticosteroids, topical ganciclovir and oral acyclovir was prescribed, with a good clinical response. Aqueous humour samples were taken during the outbreaks without yielding results for herpesviruses. The endothelial count remained low at the central level several months later, although the peripheral count was good. The fellow eye showed a normal count. It was decided to perform DWEK in the endothelial zone of retrokeratic precipitates as a diagnostic measure. The analyses of the endothelial sample FOR THE HERPESVIRIDAE FAMILY do not show results, however, a progressive improvement in corneal edema and visual acuity was observed from the first month after DWEK. He is currently stable, with mild paracentral edema and a best‐corrected visual acuity of 0.7.Conclusions: DWEK is reserved for cases of slightly advanced endothelial dystrophy, in which the central endothelial denudation causes a centripetal migration of the peripheral endothelial reserve. In this case, the clinical findings suggest a viral aetiology of the endothelial loss, therefore, DWEK was considered as a diagnostic procedure, to obtain samples for PCR and as a first step prior to DMEK. The improvement after DWEK in the area of retrokeratic precipitates without the need for a subsequent transplant suggests localized endothelial involvement in cases of endothelial loss associated with localized inflammatory precipitates.

Clinical and Molecular Features of FTL3 non Canonical and Activation Loop Mutations

Activating mutations of FLT3 (FLT3MT) gene are one of the most frequent pathogenic lesions in AML. As a consequence, tailored therapeutics have been developed in this setting. For instance, protein tyrosine kinase (PTK) domain is a target of inhibitors, which have already entered clinical practice. Overall, FLT3MT occur in about 30% of AML cases, with the internal tandem duplication (ITD) being by far the most common mutation. Indeed, FLT3ITD account for about 83% of all FLT3MT cases and are associated with dismal prognosis. In addition to FLT3ITD, distinct point mutations and deletions within the tyrosine kinase domain (FLT3TKD) and more specifically its activation loop (FLT3AL) have been described and can be also targeted by PTK inhibitors. Nonetheless, these latter mutations are less common, making up approximately 7-10% of all AML cases. Besides these "canonical” mutation types, point mutations outside of the FLT3AL, or "non-canonical” FLT3MT (FLT3NC) have also been observed in AML and other related myeloid neoplasms (MN). However, the prognostic impact and clinical relevance of such molecular alterations are not fully elucidated. This study was inspired by a case diagnosed with CMML harboring a FLT3NC mutation at the amino acid position 592 substituting an alanine for a valine. This spot is immediately located at the starting of the region (591-597) and is important for maintaining the normal kinase in an inactive state in absence of the ligand. Of note is that treatment with the highly-selective, second generation PTK inhibitor, gilteritinib resulted in a good clinical response. Therefore, we reviewed NGS sequencing results of 5,308 patients with MN in search for cases carrying similar lesions. A total of 248 patients with somatic FLT3MT were identified with 152 FLT3ITD, 66 FLT3AL, and 30 FLT3NC hits (Fig.1). The ITD subgroup included 144 AML, 4 MDS, 2 MPN, and 2 MDS/MPN overlap cases. Within cases with FLT3AL mutations, diagnoses consisted of 61 AML, 2 MDS, and 3 MDS/MPN. Most importantly, we also found 30 cases with FLT3NC mutations including 19 AML, 3 MDS, 1 MPN, 4 MDS/MPN, and notably 3 MDS cases with an antecedent history of aplastic anemia. While all the FLT3AL were predicted to be pathogenic based on the ACMG criteria, the FLT3NC consisted of 7 VUS, 10 likely pathogenic, and 9 known pathogenic variants with one biallelic, two recurrent variants in 2 patients and one (N676K) recurring in 4 patients. These atypical FLT3MT mapping to non-canonical locations were found more commonly in non-AML entities (37% in MDS/MPN, MDS, MPN vs. 63% AML) vs.FLT3AL (92% in AML; P< .001) and FLT3ITD (95% AML; P<.001). Co-mutational burden was evaluated with an NGS panel of 51 commonly mutated genes in MN. As expected, NPM1MT was significantly more likely to be associated with cases carrying FLT3ITD (46%) than FLT3NC (20%) (P= .008). Additionally, DNMT3A was found to be recurrently associated to cases with both FLT3ITD (36%) and FLT3AL (30%) as compared to FLT3NC (10%) (P= .005, P= .023, respectively). Median VAFs of both FLT3ITD and FLT3AL were not different compared to FLT3NC (21 vs. 31%; P= .08). Analysis of clinical phenotypes revealed that FLT3AL was more associated with high-grade diseases compared to FLT3NC (94% vs. 67%; P= .001). However, there was no difference in overall survival (OS) between FLT3AL and FLT3NC in AML (23 vs. 36 months; P= .70). In terms of treatment, 17 patients: 12 FLT3AL and 5 FLT3NC (4 AML, 1 MDS/MPN) received TKI inhibitors. In this limited group, no OS differences between AML patients treated with PTK inhibitors (13 cases receiving midostaurin, 3 gilteritinib and 1 sorafenib) and non-treated patients were observed in either FLT3AL or FLT3NC. However, all 5 patients harboring FTL3NC responded to PTK inhibitors. In sum, the well-studied FLT3ITD and NPM1 association does not extend to point mutations outside the FLT3AL. In addition, although differences were noted in less advanced diseases, once patients progressed to AML no specific features, clinical parameters or prognosis differed between cases with FLT3 point mutations/deletions within the activation loop, which appeared to be similar to patients with other types of point mutations outside this region. We also report on the use of PTKs in FLT3NC, whose therapeutic response in association with such FLT3 molecular patterns should warrant further studies. Figure 1View largeDownload PPTFigure 1View largeDownload PPT Close modal

Deteriorating Sprue: A New Frontier for Gastrointestinal Ultrasound

Question: A 73-year-old Australian man of Indian background presented with a 6-month history of worsening steatorrhea, malodorous flatulence and weight loss of 15 kg (body mass index 18 kg/m2). He had a 20-year history of celiac disease treated with a strict gluten-free diet with good clinical and histologic response. He also had hypertension previously treated with candesartan, which had been ceased years before the onset of these symptoms. He was on no medications, was a nonsmoker, and did not consume alcohol.

Takayasu’s Arteritis Associated with Crohn’s Disease and Autoimmune Thyroiditis: An Unusual Association with Diagnostic and Therapeutic Difficulties

The coexistence of Crohn's disease (CD) and Takayasu's disease (TD) does not seem to be accidental and does not seem to modify the natural course of the 2 pathologies. The granulomatous character of the inflammatory infiltrate of the vascular wall appears to be the result of the pro-inflammatory effect of several cytokines, such as TNF and the IL-6, -8, -12 and -18 common to both entities. The literature reports more than 40 cases of Takayasu's disease associated with Crohn's disease. However, the association of these two pathologies with autoimmune thyroiditis has never been described before in the literature. In a case hospitalized in the gastroenterology department of the Mohammed VI University Hospital in Marrakech, of a woman with autoimmune thyroiditis treated by total thyroidectomy and hormone replacement therapy, Crohn's disease and Takayasu's disease for which she was treated by antiTNF treatment and the evolution was marked by a good clinical response.

PO41 Presentation Time: 4:45 PM: Initial Experience with the New Commercial Aarhus MR Compatible Hybrid Interstitial Brachytherapy Applicator in Indian Setting

<h3>Purpose -</h3> We have been using CT based intracavitary applicator in our clinic for cervical cancer. The advent of new hybrid applicator and short sequence cube MRI sequence opened up opportunity to do advanced MRI based brachytherapy in our clinic. We present the initial clinical experience of Aarhus ring and tandem MR compatible hybrid interstitial applicator with both straight and oblique needles and no ring channel (Varian may need to add TM) which was recently approved for use in a patient with bulky cervical cancer. <h3>Material and methods -</h3> A 45 years old lady, with initial diagnosis of carcinoma cervix, stage 1B3, completed External beam radiotherapy to a total dose of 45Gy in 25 Fractions, with a good clinical response, was taken up for further treatment with brachytherapy to a total dose of 28 Gy in 4 fractions. Brachytherapy was split into two applications one week apart and two treatment fractions delivered with each application, with 8 hours gap between each fraction.We used 60 degree Aarhus applicator set with Intrauterine tandem of 40 mm length and outer ring diameter of 32 mm. The Ring includes 19 guide holes for plastic interstitial needles arranged in 2 circles, 9 in inner circle for oblique needles and 10 in outer circle for straight needles.After external radiation patient underwent MRI pelvis with only T2 weighted axial and Cube sequences, which showed residual disease limited to cervix on the right side. The depth of needle insertion into the cervix was measured on MRI to be 3cms. Procedure performed under short general anaesthesia and 4 straight plastic needles through the ring were inserted with two on either side along with 4 cm central Tandem. CT and MR performed for both applications and fused. GTV-primary and HR CTV delineated on MRI, while OARs were delineated on CT. A dose of 7 Gy per fraction prescribed to HR CTV. An optimised plan was generated on eclipse treatment planning system with repeat CT planning for remaining fractions. <h3>Results -</h3> Treatment delivery went uneventful with very minimal blood loss. Average Dose per fraction received by 90 % of volume (D90) of HR CTV was 7.617 Gy and D98 of GTV primary was 10.35 Gy, delivering a total EQD2 of 89 Gy to HR CTV and at the same time delivering 115 Gy to the GTVp. OAR doses were well constrained with Bladder 2cc receiving 79.25 Gy, Rectum 2cc constrained to 53.48 Gy, sigmoid 2cc to 58.99 Gy and small bowel to 51.98 Gy. <h3>Conclusions -</h3> We successfully implemented MRI based planning with new Aarhus applicator in our clinic. Procedure was uneventful with excellent dosimetry. We will continue to use it in our clinic for patients with locally advanced cancer.

ODP059 Torn Between Two Adrenal Cancers: A Case Report

Abstract The adrenals are affected by Non-Hodgkin's lymphoma (NHL) in only 1 percent of cases. We report a rare case of NHL presenting as a large irregular mass at the adrenal gland in an elderly male. A 62-year-old male with hypertension and diabetes presented with 6-month history of vague right sided abdominal pain with recent extension to the left flank accompanied by body malaise and unintentional weight loss. Abdominal ultrasound revealed a 9-cm left suprarenal mass in addition to bilateral nephrolithiases and prostate enlargement. There was also left-sided minimal pleural effusion. CT scan and MRI contrast studies both confirmed a large left adrenal mass with infiltration of the spleen, pancreatic tail, and left kidney. High-Resolution CT scan of the chest confirmed left-sided pleural effusion with subsegmental atelectasis. Hormonal tests showed normal 24-hour urinary metanephrines, 24-hour urinary free cortisol, aldosterone-renin ratio, and serum DHEAS. Intact PTH and calcium were also normal. Multidisciplinary consultations involving endocrinology, medical oncology, hematology, pulmonology, surgical oncology, urology, thoracic cardiovascular surgery, anesthesiology, radiology, and pathology were done. He underwent exploratory laparotomy with resection of left adrenal mass and en bloc left nephrectomy, splenectomy and distal pancreatectomy. Histopathologic findings and immunostaining were consistent with diffuse large B-cell lymphoma. He completed R-CHOP chemotherapy and adjuvant radiotherapy with good clinical response and he has been on remission for two years. Presentation: No date and time listed

Evaluation of the Diagnostic Accuracy of Non-Specific Bronchial Provocation Tests in the Diagnosis of Asthma: A Randomized Cross-Over Study

IntroductionThe role of bronchial provocation tests in the diagnosis of asthma remains to be fully explored. We aimed to evaluate methacholine and mannitol challenge testing, and explore the factors associated with this broncoprovocation response. MethodsObservational, cross-over, randomized trial evaluating adult cases with suspected asthma, naïve to treatment, with normal pre-bronchodilator spirometry, and negative bronchodilator test. Patients were randomized to start with methacholine or mannitol. The diagnosis of bronchial asthma was confirmed if there was a good functional and clinical response to one month with twice daily formoterol/budesonide 9/320. The diagnostic profile and the concordance were calculated. Factors associated with a positive provocation test were entered into a multivariate binomial logistic regression analysis, and classification trees were created for both tests. ResultsThe study included 108 cases (50.0% diagnosed with asthma and 51.9% cases starting with methacholine). The percentage of cases positive to methacholine and mannitol were 30.6% and 25.0% respectively. Kappa values were 0.40 (p<0.001). The diagnostic profile for methacholine was sensitivity 59.3% and specificity 98.1%, while for mannitol it was sensitivity 48.1% and specificity 98.1%. Variables associated with a positive methacholine response included sex, atopy, FEV1, FEV1/FVC and FENO, whereas they were FEV1/FVC and FENO for mannitol. A FENO value>26ppb, FEV1≤103.3% and female sex correctly classified 78.7% of methacholine responders. FENO value>26ppb was enough to correctly classify 81.5% of mannitol responders. ConclusionsOur study confirms the diagnostic profile of methacholine and mannitol challenge tests and describes the variable associated to their positivity with new proposed cutoff values.

ODP295 An Unusual Case of Isolated IgG4 Related Hypophysitis

Abstract Introduction Hypophysitis, characterized by chronic inflammation of the pituitary, is a histological diagnosis, secondary to lymphocytic, granulomatous, plasmacytic, necrotizing, or xanthomatous etiology. Infiltration of the IgG4 (Immunoglobulin G4) positive plasma cells, lymphocytes, and subsequent fibrosis lead to IgG4 related hypophysitis. This is typically a part of a multifocal systemic disease called IgG4 related diseases and isolated pituitary involvement is uncommon. Here, we present a case of isolated IgG4 related hypophysitis. Case A 34-year-old woman presented with secondary amenorrhea and galactorrhea. Hyperprolactinemia (137 ng/mL) was noted with no associated pituitary hormone deficits. MRI pituitary showed a markedly enlarged, heterogeneous, diffusely enhancing pituitary gland measuring 13.6×12.8×15.2 mm and a small, non-enhancing focus on the left, abutting the chiasm with mass effect and mild displacement of the stalk. Cabergoline was started with the resolution of galactorrhea and normalization of prolactin levels (&amp;lt;1 ng/mL). MRI pituitary after 18 months of treatment showed enlarging pituitary hyperplasia (11.3×17×14.1 mm). The patient developed worsening headaches and diplopia due to 6th cranial nerve palsy. Infiltrative disorders like sarcoidosis and hemochromatosis were ruled out. She denies any history of radiation therapy, CNS infections, or familial history. Lumbar puncture showed elevated proteins (80 mg/dL) and positive oligoclonal bands of IgG. Transsphenoidal resection of the pituitary mass revealed a fibrous lesion. Pathology revealed lymphoplasmacytic infiltrate consistent with IgG4 hypophysitis. She did not have systemic evidence of IgG4 related diseases. A six-month follow-up MRI showed a recurrent 12×14×13 mm pituitary lesion with a mass effect on the optic chiasm. Prednisone 40 mg daily was started. She developed TIA followed by a stroke within two weeks. Autoimmune labs were positive for speckled ANA 1: 160. High dose methylprednisolone was given for 5 days followed by treatment with 2 doses of rituximab and 4 doses of cyclophosphamide. She showed good clinical response with resolution of the pituitary mass. Discussion IgG4 related hypophysitis refers to non-hormone secreting pituitary inflammation and enlargement that shares clinical and radiological pictures like nonfunctioning pituitary adenomas. Male preponderance is noted with a presentation in the 5 th to 7 th decades of life. The prevalence of IgG4 related hypophysitis is around 30% of hypophysitis but only 1.3% of primary hypophysitis cases with no systemic involvement. Autoimmunity and infectious agents are potential inciting factors in the pathogenesis of IgG4 related diseases. Simple observation is generally recommended if the disease is asymptomatic. If the disease is symptomatic, high-dose glucocorticoids are the first choice in the absence of visual compromise requiring immediate surgical decompression and have shown a favorable response. For steroid-refractory cases, treatment options include Rituximab, azathioprine, cyclophosphamide, surgery, or radiation. Further literature study is required to identify this uncommon etiology. Presentation: No date and time listed

PSAT196 Monostotic Paget's disease of the bone involving the calcaneus

Abstract Introduction Paget's disease of bone (PDB) is a chronic bone disorder that is fairly common in older age. It is characterized by an initial increase in osteoclastic activity followed by excessive osteoblastic activity with disorganized bone architecture. PDB may involve any bone and may be monostotic or polyostotic. Herein, we report the rare occurrence of monostotic Paget's involving the calcaneus. Case Description A 71-year-old white male with PMH of prostate cancer status post radical prostatectomy presented with acute onset severe left heel and ankle pain. Pain was localized to the retrocalcaneal area and heel pad limiting his physical activity. He denied recent trauma. Physical exam was significant for swelling and tenderness of the left heel. Left foot X-rays showed a slightly enlarged calcaneus with very coarse trabeculae consistent with PDB. A total body bone scan done previously because of the prostate cancer showed intense uptake in the left calcaneus but no significant uptake elsewhere. Laboratory workup showed an elevated total serum alkaline phosphatase of 164 U/L (40-129 U/L) and bone specific alkaline phosphatase 65 ug/L (0.0-20 ug/L). He was treated with a single infusion of Zoledronic acid with good clinical response evidenced by improvement in pain and swelling as well as a significant decrease in bone specific alkaline phosphatase to 10 ug/L six months post-infusion. Discussion Monostotic PDB only represents about 20% of PDB cases. The pelvis is the most common location followed by the femur as described by Bachiller-Corral et al (Bone, 2013). In our review of the English literature, we found only nine cases of monostotic PDB involving the calcaneus. The mean age of presentation was 60 years old and most of the cases presented as chronic heel pain not responding to conservative pain alleviating measures. Conventional radiography showed the characteristic features of PDB but the changes observed were difficult to differentiate from that of a malignant process. That led to further imaging in most cases by either CT, MRI scan or SPECT scan. Three out of nine patients were reported to receive bisphosphonate therapy with successful remission with a long duration of disease suppression. Good response to therapy highlights the importance of proper diagnosis. Presentation: Saturday, June 11, 2022 1:00 p.m. - 3:00 p.m.

Type D lymphomatoid papulosis with pityriasis lichenoides et varioliformis acuta-like features in a child with parvovirus infection: a controversial diagnosis in the spectrum of lymphoid proliferations: case report and literature review

BackgroundLymphomatoid papulosis (LyP) is a rare condition in pediatrics; LyP histological type D has been reported in only 7 children. The differential diagnosis of LyP in the spectrum of lymphoid proliferation remains controversial.Case presentationA 6-year-old boy presented to Emergency Department with a 3-week history of an erythematous papulo-vesicular itchy eruption over the submandibular regions, trunk and extremities. History, symptoms and laboratory tests were unremarkable. SARS-CoV-2 antigen was negative. The clinical suspicion of pityriasis lichenoides et varioliformis acuta (PLEVA) was posed, and topical steroids were introduced. One week after, he returned with an extensive painful scaly papulo-erythematous rash, with some ulcerated and necrotic lesions, and fever; therefore the child was hospitalized. Biochemical results were within reference limits, except for high level of C-reactive protein, aspartate aminotransferase, alanine transaminase and bilirubin. Due to a persistently high fever, systemic corticosteroid treatment was administered, with a good clinical response and an improvement of the skin lesions. Anti-PVB-19 Immunoglobulin M was detected. Elevated levels of IL-6, IL-10 and IFN-γ were also recorded. Five days post-admission, most of the lesions had cleared, and the child was discharged. Methotrexate was started, with a positive response. At skin biopsy a “PLEVA-like” pattern was apparent, with a dense, wedge shaped lymphoid infiltrate featuring epidermotropism and morphologically comprising pleomorphic and blastic cells. The pattern of infiltration was highlighted by immunohistochemical stains, which prove the process to feature a CD8+/CD30 + phenotype, the latter being intense on larger cells, with antigenic loss. Polymerase chain reaction for T-cell receptor gamma (TCRG) chain clonality assessment documented a monoclonal peak. A diagnosis of LyP type D was favored.ConclusionThe reported case encompasses most of the critical features of two separated entities—PLEVA and LyP—thus providing further support to the concept of them representing declinations within a sole spectrum of disease. Studying the role of infectious agents as trigger potential in lymphoproliferative cutaneous disorders and detecting novel markers of disease, such as cytokines, could have a crucial impact on pathogenic disease mechanisms and perspective therapies.

Clinical and biochemical predictors of tumor response after neoadjuvant therapy in rectal cancer.

Patients who have a good clinical and/or pathologic response to neoadjuvant chemoradiotherapy (nCRT) for rectal cancer have better long-term outcomes and can potentially be spared morbid surgery. This study aimed to identify pretreatment clinical and biochemical predictors of response to neoadjuvant treatment for rectal cancer. Patients undergoing neoadjuvant therapy for rectal cancer between 2007 and 2022 were retrospectively included. Those patients who achieved a complete clinical response were offered a nonoperative management strategy and the remaining patients underwent surgical resection. The primary endpoint was tumor regression grade (TRG) based on radiological imaging (mrTRG) or pathology (pTRG). Patient response was classified as good (mrTRG 1-2 or pTRG 0-1) versus poor (mrTRG 3-4 or pTRG 2-3). Logistic regression was performed to determine predictors of TRG. A total of 984 patients with rectal cancer were identified of which 274 met the inclusion criteria. Of 274 patients, 228 (83%) underwent surgical resection. A good TRG response was observed in 119 (41%) patients, and a complete response was achieved in 53 (17%) patients. On univariable and multivariable logistic regression, clinical T2 stage and body mass index of ≥25kg/m2 were significant predictors of a good TRG. Clinical T2 stage and a personalised total neoadjuvant therapy regimen were significant predictors of complete response. Clinical T2 stage and a BMI≥25kg/m2 were predictors of good response to neoadjuvant therapy for rectal cancer. Future prospective studies are required to confirm these findings and evaluate their potential use in better targeting of nCRT.

Dupilumab dose spacing after initial successful treatment or adverse events in adult patients with atopic dermatitis: A retrospective analysis.

Strategies on long-term management of patients affected by atopic dermatitis (AD) undergoing treatment with dupilumab achieving good clinical response (GCR) or experiencing dupilumab-related adverse events (AEs) are scant. Data of patients who implemented longer than scheduled dupilumab dosing interval due to GCR (at least 52 weeks of treatment and controlled AD activity [Eczema Area Severity Index ≤7 and Dermatology Life Quality Index ≤5 for at least 6months]) or AEs (dupilumab-related and treatment-resistant conjunctivitis) were retrospectively collected. Dupilumab was tapered to Q3W or Q4W based on physician-patient shared decision. At baseline (T0) and each follow-up (week 16 [T1] and week 32 [T2]) disease severity was assessed. A total of 59 patients implemented longer than scheduled dosing interval (44 GCR, 15 AEs). Among these, 50 (35 GCR and 15 conjunctivitis) patients switched to 300 mg Q3W, while nine GCR subjects to Q4W. In the GCR group Q3W, 34 and 31 patients maintained clinical response at T1 and T2, whereas eight and seven Q4W subjects maintained clinical response at the same timepoints, respectively. No significant differences in AD severity were observed between T1 and T2 in both groups. Contrariwise, one Q3W and one Q4W patients at T1, and three Q3W and one Q4W subjects at T2, returned to dupilumab labeled dosage due to AD worsening. In conjunctivitis group, dupilumab Q3W was maintained in eight and four patients at T1 and T2, respectively. Three patients at T1 and three at T2 subjects returned to the labeled interval due to conjunctivitis remission. Four patients at T1 and four subjects at T2 interrupted dupilumab due to the persistence of conjunctivitis. A longer dupilumab dosing interval may be a valuable option in patients with a GCR and may be a useful strategy to reduce treatment-related conjunctivitis, also with pharmacoeconomic benefit.

Infectious Pulmonary Artery Pseudoaneurysm That Resolved with Conservative Treatment

Pulmonary artery pseudoaneurysms (PAPs) are rare but can cause massive hemoptysis if they rupture. Infectious PAPs are often treated by surgery or transcatheter embolization and are rarely treated conservatively with antibiotics. We herein report a case of PAP treated conservatively in a 21-year-old woman with lung abscess. Except for one massive hemoptysis early in the course, the patient responded well to the empirical therapy with ampicillin/sulbactam and systemic hemostatic agents. After six weeks of antibiotics, the pseudoaneurysm disappeared. Conservative therapy with careful observation can be considered in small infectious PAPs when there is a good clinical response to initial conservative therapy.

50 The spectrum of pediatric lupus: data from the Kenya Pediatric Rheumatology Registry (KAPRI)

BackgroundPediatric rheumatic diseases are chronic illnesses that impart a significant disease burden upon children and their families (1–3). Furthermore, they have the potential to cause physical disability, diminished quality of life and significant direct and indirect costs (1–3). Determination of the burden and clinical characteristics of pediatric lupus is a critical first step to improving access to care and optimizing use of existing health systems for the well-being of these patients (4). Childhood-onset systemic lupus erythematosus (cSLE) is a prototype autoimmune condition characterized by simultaneous or sequential organ and system involvement, with unpredictable flare and high levels of morbidity and mortality (5). A pediatric rheumatology registry is critical in defining the spectrum of pediatric lupus within the region, the clinical characteristics of the disease, the outcomes and responses to various interventions. The Kenya Pediatric Rheumatology Registry (KAPRI) registry offers a unique opportunity to pioneer and spearhead a systematic and organized format in collecting pertinent clinical information that will offer information on current clinical scenarios and offer a platform to conduct other research projects to improve pediatric lupus healthcare in sub-Sahara Africa. Our objective was to determine the clinic-epidemiological profile of paediatric lupus patients by describing their baseline patient characteristics and clinical features at the Aga Khan University Medical College East Africa who were enrolled into the KAPRI registry from inception in March 2019 to December 2021.MethodsAll patient records were selected from the KAPRI registry database using the ICD 10 code M32 that denotes Systemic Lupus Erythematosus (SLE) Age, gender, laboratory, clinical features at diagnosis, treatment options offered at time of diagnosis were extracted from the database.ResultsAmong the 207 patients enrolled thus far in the registry, 7 had a diagnosis of cSLE (3.4%). The commonest symptom among the patients were joint pain, fever and rash. Other clinical features and outcomes are highlighted in the table 1 below;ConclusionOver a 2-year period, 57% of our cSLE patients (4 of 7) achieved remission while 43% (3of 7) were lost to follow up. Further studies are required to elucidate predictors of good clinical response and reasons for loss to follow up among our cSLE patients.