Punctate Calcifications Research Articles

CT and MRI radiographic characteristics of seminal vesicle cysts

PurposeSeminal vesicle cysts are relatively uncommon benign lesions. Early and accurate diagnosis is crucial for guiding appropriate management strategies. The purpose of this study was to investigate the radiographic characteristics of seminal vesicle cysts in order to enhance the diagnostic proficiency of radiologists. MethodsWe conduct a comprehensive descriptive analysis of the CT and MRI findings pertaining to 15 patients with seminal vesicle cyst, complemented by a brief literature review. ResultThe mean age of 15 patients with seminal vesicle cyst was 57.53 ± 17.0 years. Among these patients, 53.33% (8/15) were incidentally detected, while 40% (6/15) presented with symptoms of abdominal or pelvic pain. One patient exhibited hematospermia. In 86.67% (13/15) of cases, the cysts were unilateral, while the remaining 13.33% (2/15) were bilateral. Radiological assessment comprised CT scans for 7 patients, MRI scans for 12 patients, and a combination of both modalities for 4 patients. Imaging characteristics revealed 11 cases of unilocular cysts and 4 cases of multilocular cysts, with the largest measuring approximately 16.0 × 9.8 cm. CT imaging distinctly demonstrated punctate or nodular calcification along the cyst wall in 3 cases. MRI, on the other hand, facilitated the visualization of cystic contents, including mucinous protein in 9 cases and hemorrhagic components in 2 cases. ConclusionThe radiographic manifestations of seminal vesicle cysts exhibit distinct characteristics, CT and MRI imaging modalities are instrumental in facilitating their accurate diagnosis.

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.

To describe the clinical and ultrasonographic features of uterine perivascular epithelioid cell tumor (PEComa) using standardized terminology. This was a retrospective analysis of patients with uterine PEComa diagnosed and confirmed by pathology and immunohistochemistry at West China Second University Hospital, Sichuan University, Sichuan, China, between January 2010 and September 2023. The Morphological Uterus Sonographic Assessment (MUSA) consensus and the International Endometrial Tumor Analysis (IETA) consensus were utilized for the standardized description of the sonographic characteristics of uterine PEComa. We summarized the clinical and ultrasound features of uterine PEComa in cases from our center and those found in a review of the literature conducted using PubMed from 1 January 2013 to 30 September 2023 (inclusive). Five patients, aged 33-57 (median, 52) years with a total of six uterine PEComa lesions were included in our cohort. All cases had complete ultrasonographic and pathological images. None of the patients had a history of tuberous sclerosis complex. Two patients had malignant PEComa (one patient had two lesions) and three had benign PEComa, originating from the cervix, myometrium or uterine cavity. Patients presented with symptoms including increased vaginal discharge, vaginal bleeding and pelvic or abdominal pain. The three patients with benign PEComa underwent total hysterectomy and bilateral adnexectomy, tumor excision and conservative management, respectively, while both malignant cases underwent total hysterectomy and bilateral adnexectomy followed by chemotherapy. Regular follow-up (from 6 to 24 months) revealed recurrence in one case. Two lesions were misdiagnosed as uterine fibroids, two as cervical cancer, one as metastatic cervical cancer (with myometrial invasion) and one was indeterminate. Ultrasound examination showed that most lesions displayed regular round or ovoid shapes (66.7%), uniform echoes (66.7%) and hypoechogenicity (66.7%), with one (16.7%) malignant PEComa showing cystic areas and one (16.7%) benign PEComa showing punctate calcifications. All lesions lacked shadowing and the majority showed moderate to abundant vascularity (color score of 3-4, 83.3%). The color score was 2-4 in the periphery in 100% of cases and internally in 83.3% of cases. The three benign PEComas showed similar characteristics in vascular distribution, with scattered internal vessels and peripheral vessels exhibiting a circular pattern. The literature search identified 11 articles describing the ultrasonographic appearance of 18 cases of uterine PEComa, with similar characteristics to those in our cohort. The sonographic features of uterine PEComa include a uniform or non-uniform hypoechogenic mass, typically round or ovoid with regular margins, occasionally containing cystic areas or calcifications, lacking shadowing and often showing moderate to abundant vascularity. Although the preoperative ultrasound diagnosis of uterine PEComa remains challenging, particularly given the non-specific nature of the sonographic characteristics described here, dispersed intratumoral vessels and a peripheral circular vascular distribution may serve as diagnostic clues for uterine PEComa, but more cases are needed for confirmation. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Extranodal natural killer/T-cell lymphoma with tonsil involvement: a case report

BackgroundExtranodal natural killer/T-cell lymphoma (ENKTL) with tonsil involvement is not common, especially in children.Case presentationA 13-year-old girl presented with an unexplained sore throat for more than 2 months, together with intermittent fever and suppurative tonsilitis. Nasopharyngoscopy revealed a pharyngeal mass. Enhanced computed tomography (CT) scan showed tonsillar hypertrophy and punctate calcification. Chronic pyogenic granulomatous inflammation with pseudoepithelial squamous epithelial hyperplasia was observed in left tonsil, and pyogenic granulomatous inflammation and a small number of T-lymphoid cells were detected in the right tonsil. The immunohistochemical results showed CD2+, CD3+, CD4+, CD5+, CD8+, granzyme B+, and TIA-1+. The Ki-67 proliferation index was 20%. The case showed T cell receptor gene rearrangement. Finally, the case was diagnosed as ENKTL of stage II with tonsil involvement. The patient received 6 cycles of chemotherapy with SMILE regimen, and showed complete response with no recurrence in the follow-up.ConclusionWe presented a rare case of ENKTL with tonsil involvement in a child. The patient showed complete response to the SMILE chemotherapy with no recurrence.

Benign Brenner tumor of the ovary: two-dimensional and contrast-enhanced ultrasound features-a retrospective study from a single center.

Benign Brenner tumor (BBT) is a rare ovarian tumor, and there are few discrete reports about its manifestation in an ultrasound. This study sought to investigate the two-dimensional (2D) and contrast-enhanced ultrasound (CEUS) features of this entity. This is a retrospective single-center study. The clinical manifestations, laboratory examination, and ultrasound data of 25 female patients with BBT were confirmed by pathology when they underwent 2D and/or CEUS examination at Ningbo First Hospital from January 2012 to June 2023. The ultrasound findings of the patients were analyzed using the terminology of the International Organization for the Analysis of Ovarian Tumor and were read by two senior sonographers who reached an agreement. Among the all 25 patients, most of them were unilateral, and only one patient was bilateral. Thus, 26 lesions were found: 44.0% (11/25) were in the left and 52.0% (13/25) were in the right. Moreover, 53.84% (14/26) were solid lesions, 15.38% (4/26) were mixed lesions, and 26.92% (7/26) were cystic lesions. Among the solid-type patients, 42.85% (6/14) of the cases were with calcification. Upon laboratory examination, 12.0% (3/25) of the patients had high carbohydrate antigen 125 (CA-125) level, and 19.04% (4/21) of the patients had an elevated carbohydrate antigen724 (CA-724) level in the serum tumor markers. In the hormone test, 14.28% (3/21) were found to have a high postmenopausal estrogen level and 14.28%(3/21) were found to have a high level of follicle-stimulating hormone (FSH). One patient with complex manifestations and three with solid manifestations were examined by CEUS to observe the microcirculation perfusion of the tumor. One with solid and cystic separation was rapidly hyperenhanced and cleared, and the filling subsided faster than the uterus. The postoperative pathological diagnosis was benign Brenner tumor with mucinous cystadenoma. The other three cases were solid adnexal lesions, which showed isoenhancement on CEUS and disappeared slowly, synchronizing with the uterus. The CEUS results were considered as benign tumors and confirmed by pathology. BBT can show ovarian cystic, mixed cystic and solid type, and solid echo in 2D ultrasound. Unilateral ovarian fibrosis with punctate calcification is an important feature of BBT in 2D ultrasound. However, for solid adnexal masses and mixed cystic and solid masses with unclear diagnosis, if CEUS shows isoenhancement or hyperenhancement, the possibility of BBT cannot be excluded.

An important finding for differential diagnosis in thymic cyst mimicking pericardial cyst: punctate calcification.

Mediastinal cysts are usually congenital but present in adulthood. A pericardial cyst is usually localized in the right cardiophrenic region. Thymic cysts are less common and are located in the cervical region or anterior mediastinal region. While thoracoscopic excision or aspiration can be applied in pericardial cysts, excision is recommended in thymic cysts. We present a case of a thymic cyst located in the localization of the pericardial cyst and radiologically containing wall punctate calcification.

Imaging features and deep learning for prediction of pulmonary epithelioid hemangioendothelioma in CT images.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular tumour, and its early diagnosis remains challenging. This study aims to comprehensively analyse the imaging features of PEH and develop a model for predicting PEH. Retrospective and pooled analyses of imaging findings were performed in PEH patients at our center (n=25) and in published cases (n=71), respectively. Relevant computed tomography (CT) images were extracted and used to build a deep learning model for PEH identification and differentiation from other diseases. In this study, bilateral multiple nodules/masses (n=19) appeared to be more common with most nodules less than 2 cm. In addition to the common types and features, the pattern of mixed type (n=4) and isolated nodules (n=4), punctate calcifications (5/25) and lymph node enlargement were also observed (10/25). The presence of pleural effusion is associated with a poor prognosis in PEH. The deep learning model, with an area under the receiver operating characteristic curve (AUC) of 0.71 [95% confidence interval (CI): 0.69-0.72], has a differentiation accuracy of 100% and 74% for the training and test sets respectively. This study confirmed the heterogeneity of the imaging findings in PEH and showed several previously undescribed types and features. The current deep learning model based on CT has potential for clinical application and needs to be further explored in the future.

FRI557 Don’t Forget The Incidental Thyroid Nodule In Graves’ Disease. A Case of Marine-Lenhart Syndrome

Abstract Disclosure: K.N. Andino-Lebrón: None. A. Martinó-Morales: None. J.M. Colón-Castellano: None. Marine-Lenhart syndrome (MLS) was initially described by Charkes in 1972 as he observed patients with Graves’ disease (GD) and incidental functioning thyroid nodules. He named the disease Marine-Lenhart syndrome based on a paper published by Marine and Lenhart in 1911. MLS is a variant of GD now described as the coexistence of GD and autonomous functional thyroid nodules (AFTN); thus, patients present with thyrotoxicosis due to GD and AFTN. MLS is very rare, and prevalence is estimated to be around 0.8%-2.7%. Here, we present a case of MLS in a 79-year-old female. A 79-year-old Hispanic woman with history of breast metaplasia s/p chemoprevention with tamoxifen for 5 years, atrial premature contractions on metoprolol, among other medical conditions was referred to Endocrinology clinics by her primary care physician (PCP) because of abnormal thyroid function tests and ultrasound. She reported feeling anxious with associated palpitations, she denied weight loss. Physical examination was remarkable for regular rate and rhythm, no goiter, no palpable nodules, no proptosis, and deep tendon reflexes +1. Laboratory testing revealed a suppressed TSH of 0.224 uIU/mL, normal free T4 at 1.17 ng/dL, normal total T3 at 142.5 ng/dL, and positive thyroid stimulating immunoglobulins at 345%. Thyroid ultrasound showed a normal size gland with multiple complex nodules. In the right lobe there was a mixed cystic and solid nodule, measuring 1.48x1.21x1.71cm, wider than tall, with internal vascularity and punctate calcifications. In the left lobe there was an isoechoic and solid nodule measuring 0.7x0.5x0.7cm, wider than tall, without calcifications. Thyroid uptake scan with SPECT-CT demonstrated a focus of increased tracer uptake on the upper pole of the right thyroid lobe that corresponds to the one found on CT scan and a diminished and nonuniform uptake of the tracer in the reminders of the thyroid gland. The 24-hour radioactive iodine uptake was 17.5%. Diagnosis of MLS was made, and the patient was started on methimazole 5mg daily, which she has been tolerating well. Follow up laboratories show a biochemically euthyroid state. Marine-Lenhart syndrome is a rare entity of Graves’ disease which is currently defined as GD and accompanying functioning nodule(s). To establish the diagnosis, it is important the evaluation with scintigraphy and autoimmune testing. Multiple treatment options are available and patients with GD should be evaluated for the presence of thyroid nodules and their functional status to achieve optimal therapy. Patients with GD have a chance of remission when treated with antithyroid drugs. Due to the pathophysiology of AFTN, patients with MLS (concurrent GD and AFTN) have lower remission rates with antithyroid drugs and may require definitive management therapy with radioactive iodine or surgery. Presentation: Friday, June 16, 2023

Kronik Piyelonefritin Nadir Bir Nedeni: Ksantogranulomatöz Piyelonefrit

Xanthogranulomatous pyelonephritis (XGP) is a chronic, destructive, granulomatous inflammation of the renal parenchyma leading non-functioning kidney. Contrast-enhanced computed tomography is the most useful diagnostic method. A 6-year-old female patient admitted to us with complaints of abdominal pain, fever, fatigue, weakness. Recurrent urinary tract infection was present in her history. The patient’s urine specimen revealed leukocyturia, hematuria, positive for nitrite and bacteria, white blood cell: 12.7x103 /microl, hemoglobin:6.2 g/dl and CRP: 101 mg/dl. In the urinary system USG imaging, the contours of the left kidney were irregular and lobulated, and medullary punctate echogenicity and calcifications were present. Contrast-enhanced abdominal computed tomography showed increased left kidney size and opacities suggestive of stone in the collecting system; pararenal area was heterogeneous. Although the patient's gentamicin treatment was completed in 7 days and meropenem treatment in 14 days, acute phase reactants did not regress. No activity uptake was observed in the left kidney lodge in renal cortical scintigraphy. Left total nephrectomy was performed with the diagnosis of non-functioning left kidney and chronic pyelonephritis. Kidney biopsy material were reported as XGP. XGP is a rare and aggressive cause of chronic pyelonephritis with serious consequences such as nephrectomy requirement.

UNUSUAL PRESENTATION OF MALIGNANT THYMOMA ASSOCIATED NEPHROTIC SYNDROME WITH FOCAL SEGMENTAL GLOMERULOSCLEROSIS: A CASE REPORT

Paraneoplastic syndromes (PS) are the consequences of hormones or immune cross-reactivity produced by a tumor. Nephrotic syndrome (NS) is an extremely rare PS resulting from a thymoma. Here, the case of a 55-year-old woman presenting progressive generalized edema and foamy urine is reported. The patient’s chest CT scan showed anterior mediastinum with intramural punctate calcification size 8.6x7.0x10.2 cm. The case was reviewed at the multi-dispensary team conference, and the clinical diagnosis was an unusual presentation of malignant thymoma known as NS. After that, a thymectomy and kidney biopsy was performed. Histopathologic examination showed Thymoma type B and focal segmental glomerulosclerosis, respectively. Therefore, we considered it paraneoplastic nephrotic syndrome (PNS), without glucocorticoids, immunosuppressants or other drugs to treat NS. After the thymectomy, her clinical spontaneous resolved at the first follow-up, proposing a causative relationship between the two conditions.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: Radiological Findings and Radiological-pathological Correlation

The objective of this study was to describe the CT and MRI features of sclerosing angiomatoid nodular transformation (SANT) of the spleen with pathologic correlation. Ten patients with surgically resected and pathologically confirmed SANTs were included. Clinical history was reviewed, and gross pathologic, histologic, and immunohistochemical findings were recorded. CT and MRI examinations were evaluated by two radiologists. Patients included seven men and three women, with a mean age of 42.9±16.7 years. Pathologic features of SANTs involved multiple angiomatous nodules in a radiating pattern with a central stellate fibrous scar and evidence of hemosiderin deposition. 9 cases showed a lobulated demarcated margin, 8 cases a slight hypoattenuating, 1 isoattenuating, and 1 case with two lesions demonstrated a slight hyperattenuating margin, respectively. Multiple scattered punctate calcifications were involved in 2 cases. 5 cases manifested hypointensity on in-phase imaging, 1 iso-intensity, and 4 iso-hypointensity on out-of-phase imaging. Progressive and centripetal enhancement were exhibited in 10 cases, spoke-wheel pattern in 3 cases, and nodular enhancement in 4 cases, respectively. The central fibrous scar was identified in 8 cases during delayed enhancement. Characteristics of SANTs on CT/MRI reflected the underlying pathology. Hypointensity on DWI and T2WI, and change of signal on T1 chemicalshift imaging were found to be due to hemosiderin deposition and fibrous tissue. Typical feature was a solitary, round, lobulated mass with a fibrous scar. Progressive and centripetal enhancement, spoke-wheel pattern, nodular enhancement, and delayed enhancement of central fibrous scar were observed.

Systemic lupus erythematosus with Fahr’s syndrome: A case report with review of literature

Systemic lupus erythematosus (SLE) is a multisystem disease with autoimmune etiology. The neurological manifestations of lupus are diverse. We introduce a case of a 40-year-old male who presented with constitutional symptoms like low-grade fever, myalgia, and easy fatigability for one month. On examination, he had icterus, hepatosplenomegaly, and spasticity. Lab tests were suggestive of autoimmune hemolytic anemia with ANA positivity and low complements. Diagnosis of SLE was certain. CT brain showed bilateral symmetrical dense radial and punctate calcifications involving bilateral cerebral and cerebellar hemispheres suggestive of Fahr’s syndrome.

Acetabular Rim Ossification Variants Are Found in Almost 20% of Patients Presenting With Hip Pain

To determine the prevalence of 4 different types of acetabular rim ossifications, including partial labral ossification or punctate calcification, true os acetabuli, acetabular rim stress fracture, and complete labral ossification, and to determine whether different types of periacetabular ossifications are linked to demographic or radiological factors. We retrospectively reviewed the medial records of patients presenting for hip-related complaints at 2 sports medicine practices from September 2007 to December 2009. An anteroposterior radiograph of both hips and a lateral radiograph of each hip was obtained for all patients and reviewed for findings of cam and pincer femoroacetabular impingement, degenerative changes (Tönnis grade), and periacetabular calcifications for both hips. These parameters were also evaluated with respect to symptoms, sex, and age. Four hundred ninety-one consecutive patients (982 hips) presented to 2 orthopaedic surgeons at 2 centers for "hip"-related complaints. There were 223 males and 268 females (age 39 ± 14 years). The overall prevalence of periacetabular calcifications in hips was 17.6%, with 56.6% of calcifications in the symptomatic hip and 43.4% in the contralateral hip. Four basic patterns of calcification were identified: punctuate calcifications within the labrum (8.0% hips), large rounded calcifications (os acetabuli) (4.2% hip), large fragments with a vertical line of the superior-lateral acetabular rim, consistent with healed or non-healed stress fracture (2.0% hips), and complete ossification of the labrum (3.4% hips). Overall, male sex (P= .002), increased lateral center-edge angle (P= .046), and higher Tönnis grade (P < .001) statistically predicted the presence of periacetabular ossification. Punctate calcifications were more prevalent in males (P= .002). Higher Tönnis grade (P=.029) and increased alpha angle (P= .046) were more prevalent with os acetabuli. Younger age (P= .001), male sex (P= .048), increased alpha angle (P= .012), and increased lateral center-edge angle (P < .001) were more prevalent in acetabular rim fractures. No factors were statistically significant at predicting the presence of an ossified labrum. Periacetabular calcifications are not uncommon. Four particular patterns of calcification are identified: punctate labral calcifications (8%), larger rounded calcifications (i.e., os acetabuli) (4.2%), acetabular rim stress fractures (2%), and complete ossification of the labrum (3.4%) for a combined prevalence of 17.6% in patients presenting to an orthopaedic surgeon with "hip"-related complaints. Nearly half were in the asymptomatic hip. Male sex had a higher prevalence of periacetabular calcifications. An increased lateral center edge angle and higher Tönnis grade also had a higher prevalence of periacetabular calcifications. Younger male patients are more likely to have acetabular rim stress fractures. Patients with an increased alpha angle have a higher prevalence of os acetabuli and rim stress fractures. This study aims to identify, quantify, and categorize periacetabular calcifications about the hip. Their clinical relationships and relevance have been discussed, but no study has distinctly categorized the various types and their prevalence. This study provides a framework for identification and categorization.

Diagnostic accuracy of dual-energy computed tomography-based nomogram for differentiating papillary thyroid microcarcinomas from micronodular goiters.

The misdiagnosis of papillary thyroid microcarcinoma (PTMC) and micronodular goiter (MNG) may lead to overtreatment and unnecessary medical expenditure by patients. This study developed and validated a dual-energy computed tomography (DECT)-based nomogram for the preoperative differential diagnosis of PTMC and MNG. This retrospective study analyzed the data of 366 pathologically confirmed thyroid micronodules, of which 183 were PTMCs and 183 were MNGs, from 326 patients who underwent DECT examinations. The cohort was divided into the training (n=256) and validation cohorts (n=110). The conventional radiological features and DECT quantitative parameters were analyzed. The iodine concentration (IC), normalized iodine concentration (NIC), effective atomic number, normalized effective atomic number, and slope of the spectral attenuation curves in the arterial phase (AP) and venous phase (VP) were measured. A univariate analysis and stepwise logistic regression analysis were performed to screen the independent indicators for PTMC. A radiological model, DECT model, and DECT-radiological nomogram were constructed, and the performances of the 3 models were assessed using the receiver operating characteristic curve, DeLong test, and a decision curve analysis (DCA). The IC in the AP [odds ratio (OR) =0.172], NIC in the AP (OR =0.003), punctate calcification (OR =2.163), and enhanced blurring (OR =3.188) were identified as independent predictors in the stepwise-logistic regression. The areas under the curve with 95% confidence intervals (CIs) of the radiological model, DECT model, and DECT-radiological nomogram were 0.661 (95% CI: 0.595-0.728), 0.856 (95% CI: 0.810-0.902), and 0.880 (95% CI: 0.839-0.921), respectively, in the training cohort; and 0.701 (95% CI: 0.601-0.800), 0.791 (95% CI: 0.704-0.877), and 0.836 (95% CI: 0.760-0.911), respectively, in the validation cohort. The diagnostic performance of the DECT-radiological nomogram was better than that of the radiological model (P<0.05). The DECT-radiological nomogram was found to be well calibrated and had a good net benefit. DECT provides valuable information for differentiating between PTMC and MNG. The DECT-radiological nomogram could serve as an easy-to-use, noninvasive, and effective method for differentiating between PTMC and MNG and help clinicians in decision-making.

A clinico-pathological study of thyroid nodules and correlation among ultrasonographic, cytologic, and histologic findings

Background: Nodular thyroid is a common occurrence affecting 5% of the population. Aims and Objectives: To find the prevalence of thyroid nodules, to study the clinico-biochemical profile of the patients with thyroid nodules and to establish a correlation between pre-operative ultrasonography and fine-needle aspiration cytology (FNAC) with post-operative histopathologic findings. Materials and Methods: A prospective observational study was conducted in a tertiary care hospital in Kolkata over 1.5 years. Patients with clinically detectable nodules posted for surgery were evaluated clinically, biochemically, ultrasonographically (USG), and cytologically with FNAC. Histopathologic examination (HPE) was done post-surgery. Comparison was done between those with benign and malignant nodules. Correlation was done between pre-operative USG, FNA, and post-operative HPE of the nodules. Results: Out of 322 cases during the study period, the prevalence of clinically detectable thyroid nodule posted for surgery was 39 (12.58%). Most of the patients were euthyroid (94.9%), had nodules in left lobe (41.5%). Most of the nodules were heteroechoic (56.5%) and majority were THY3 nodules (48.7%) on FNAC. On HPE, malignancy was found in 20.5% of the nodules. Compared to benign nodules, malignant nodules were more commonly larger, on the left lobe, solid, hypoechoic, with spiculated margins, punctate calcification, and having associated neck nodes (pall &lt; 0.03). Out of 11 cases, which were radiologically predicted to be malignant, 10 had THY ≥3 on FNA, and 8 were malignant on HPE. USG had a sensitivity, specificity, positive, and negative predictive value of 87.5%, 87.1%, 63.63%, and 96.43% in predicting malignancy. Conclusion: Pre-operative ultrasonography for thyroid nodules can predict malignancy with sufficient sensitivity and specificity and can obviate the need for unnecessary FNAC in many cases and also suggest imminent need for surgery rather than FNAC in very high risk cases.

EWSR1-SMAD3 positive fibroblastic tumor: a clinicopathological analysis

Objective: To investigate the clinicopathological features, immunophenotypes and molecular genetics of EWSR1-SMAD3 positive fibroblastic tumor (ESFT) with an emphasis on differential diagnosis. Methods: The clinicopathological data, immunohistochemical profiles and molecular profiles of 3 ESFT cases diagnosed at the Department of Pathology, Fudan University Shanghai Cancer Center from 2018 to 2021were analyzed. The related literature was also reviewed. Results: There were two males and one female. The patients were 24, 12 and 36 years old, respectively. All three tumors occurred in the subcutis of the foot with the disease duration of 6 months to 2 years. The tumors were presented with a slowly growing mass or nodule, accompanied with pain in 1 patient. The tumors ranged in size from 0.1 to 1.6 cm (mean, 1.0 cm). Microscopically, the tumors were located in the subcutaneous tissue with a nodular or plexiform growth pattern. They were composed of cellular fascicles of bland spindle cells with elongated nuclei and fine chromatin. One of the tumors infiltrated into adjacent adipose tissue. There was no nuclear atypia or mitotic activities. All three tumors showed prominent stromal hyalinization with zonal pattern present in one case. Focal punctate calcification was noted in two cases. The immunohistochemical studies showed that tumor cells were diffusely positive for ERG and negative for CD31 and CD34, with Ki-67 index less than 2%. Fluorescence in situ hybridization on the two tested cases identified EWSR1 gene rearrangement. The next generation sequencing analysis demonstrated EWSR1-SMAD3 fusion in all three cases. During the follow up, one patient developed local recurrence 24 months after the surgery. Conclusions: ESFT is a benign fibroblastic neoplasm and has a predilection for the foot, characterized by ERG immunoreactivity and EWSR1-SMAD3 fusion. Local recurrence might occur when incompletely excised. Familiarity with its clinicopathological features is helpful in distinguishing it from other spindle cell neoplasms that tend to occur at acral sites.

Layered enhancement with punctate foci of calcifications: A unique computed tomographic feature of mucinous adenocarcinoma of stomach

Calcifications are rare in gastric adenocarcinoma and all reported cases correspond to a mucinous adenocarcinoma. Mucinous gastric adenocarcinoma (MGC) is a rare histological variety representing approximately 2.6%–6.6% of gastric cancers, sometimes associated with punctate and miliary calcification. We describe one such case having punctate calcifications with characteristic contrast-enhanced computed tomography showing hypoenhancing middle and outer layer of stomach suggestive of extracellular mucin pool, primarily in submucosa and deeper layer and thin enhancing inner layer suggestive of normal mucosa, referred to as layered enhancement pattern. We also describe the radiopathological correlation of MGC with brief review of literature.

Clinics in diagnostic imaging (216).

Clinics in diagnostic imaging (216).

Computed Tomography and Magnetic Resonance Imaging Features of a Huge Cavernous Hemangioma in the Pleural Space: A Case Report

A 44-year-old male with a huge left pleural mass was admitted due to chest discomfort. Chest computed tomography showed a large pleural mass with punctate calcification and serpentine-shaped enhancement in the left pleural space. Magnetic resonance imaging showed markedly high signal intensity with septa on fat-suppressed T2-weighted imaging (WI) and gradual peripheral delayed enhancement on dynamic-enhancing T1-WI. With hemangioma as the suspected diagnosis, the entire mass was surgically removed carefully and the final histopathological diagnosis was confirmed to be a cavernous hemangioma. Cavernous hemangioma of the pleural space is a rare benign vascular tumor. Precise radiologic diagnosis is important before the surgical operation of hemangiomas because of risk of massive hemorrhage requiring meticulous bleeding control.

ODP020 A Challenging Case of Poorly Differentiated Carcinoma with Neuroendocrine Differentiation and Adrenal Mass

Abstract Background Neuroendocrine tumors (NETs) account for about 0.5% of all newly diagnosed malignancies. The most frequent primary sites are the gastrointestinal tract or pancreatic (62%-67%) and the lung (22%-27%). All poorly differentiated NETs have a Ki-67 index of greater than 20% and WHO grade of 3. The determination of the site of origin can be challenging in widely metastatic poorly differentiated NETs. Clinical Case A 51-year man with a history of hypertension and type 2 diabetes mellitus presented with angioedema while on lisinopril for years. Apart from a month's history of bilateral flank pain, he denied diarrhea, flushing, stridor, or shortness of breath. CT abdomen showed an 8.2×8. 0×7.9 cm right adrenal mass with a few punctate calcifications, hepatomegaly with multiple liver metastases, multiple retroperitoneal, hepatoduodenal, retrocrural adenopathy, and T4 metastatic lesion. CT chest showed bulky mediastinal and supraclavicular lymph nodes with trachea narrowing. All those lesions were FDG avid on PET/CT scan. Core biopsy of right supraclavicular lymph node revealed a poorly differentiated carcinoma with neuroendocrine differentiation and high proliferative index (25-50%). On cell block, it was negative for (AE1/AE3 (-), CAM5.2 (-), CK7, CK20, vimentin, desmin, CEA, EMA, CD45, CD20, and TTF-1 but positive for CK903 (focal +), synaptophysin (+), chromogranin (+), calretinin (focally +), inhibin (focally +). Based on the immunohistochemical stain, it was concluded as a metastatic small cell carcinoma of unknown origin. He was treated with carboplatin, etoposide, and atezolizumab and received 5 cycles. Endocrine was consulted two months after his initial presentation. Urine normetanephrine was &amp;gt; 4-fold elevated. Blood pressure was controlled on Doxazosin at 4 mg bid. The 10 gene-panel for hereditary pheochromocytoma and Paraganglioma (PGL) was negative. There was a progression of mediastinal adenopathy while on treatment with narrowing of proximal intrathoracic trachea requiring elective tracheostomy. Based on imaging findings and elevated normetanephrine, we believe he has metastatic pheochromocytoma and PGL. His chemo regimen was switched to Cyclophosphamide, Vincristine, and Dacarbazine (CVD). He finished 8 cycles of CVD with an overall reduction in the size of lymph nodes (both above and below the diaphragm) and right adrenal mass. His plasma normetanephrine level was also significantly improved. Presentation: No date and time listed

PSUN236 Previously Undiagnosed Hereditary Pancreatitis Presenting as New Onset Type 3c Diabetes Mellitus

Abstract Background Type 3c Diabetes Mellitus (DM3c) or Pancreatogenic Diabetes is defined as diabetes secondary to pancreatic disease. The most common cause of Type 3c Diabetes is chronic pancreatitis, accounting for 80% of cases.1 Hereditary pancreatitis, with mutations such as SPINK1, CFTR, CTRC, is an underdiagnosed cause of chronic pancreatitis leading to Type 3c Diabetes Mellitus.1 The typical disease course for hereditary pancreatitis is recurrent episodes of acute pancreatitis that leads to chronic pancreatitis.1 The average time between hereditary pancreatitis diagnosis and development of DM3c has been shown to be 80 +/- 24 months.1 We present a case of previously undiagnosed hereditary pancreatitis presenting as new onset Type 3c Diabetes Mellitus. Clinical Case A 33-year-old male presents with a two month history of diarrhea, epigastric pain, and weight loss. Vital signs on presentation were blood pressure 105/74, heart rate 59, BMI 13. Physical exam revealed a cachectic appearing male with epigastric tenderness. Lab-work showed blood glucose 819 mg/dL, SOsm 313 mOsm/kg, lipase 21 U/L, bicarbonate 33 mmol/L. A computed tomography (CT) of the abdomen revealed punctate pancreatic parenchymal calcifications, suggestive of chronic pancreatitis. He was admitted to general medicine for management of HHS, where he was treated with IV hydration and insulin therapy. Workup for new onset diabetes revealed a negative anti-GAD Ab &amp;lt;5.0 U/mL, and C peptide level 0.5 ng/mL which suggested β cell dysfunction and decreased endogenous insulin secretion. With these lab values, along with radiographic findings consistent with chronic pancreatitis, the likely driving factor for his diabetes was pancreatic disease and he was diagnosed with DM3c. Severe pancreatic disease was further shown by a reduced fecal elastase level of &amp;lt;50, suggesting exocrine pancreatic insufficiency. Investigating the etiology of his chronic pancreatitis, history revealed no prior episodes of acute pancreatitis or abdominal pain, and no family history of pancreatitis. Workup for common causes of pancreatitis, including alcohol use, hypertriglyceridemia, and gallstones was negative, and genetic workup was pursued. The patient was found to be heterozygous for a SPINK1 mutation, which is a known mutation in hereditary pancreatitis.1 Therefore, hereditary pancreatitis was determined to be the likely cause of his chronic pancreatitis which led to Type 3c Diabetes Mellitus. He was started on daily insulin therapy and pancreatic enzymes for his endocrine and exocrine pancreatic dysfunction respectively, with improvement in symptoms. Conclusion This is a unique case of hereditary pancreatitis presenting as new onset Type 3c Diabetes Mellitus with no prior episodes of acute pancreatitis or established diagnosis of chronic pancreatitis. References Ramalho, G. X., &amp; Dytz, M. G. (2020). Diabetes of the exocrine pancreas related to hereditary pancreatitis, an update. Current Diabetes Reports, 20(6). Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.