Nodular Areas Research Articles

S1910 A Case Series of Esophageal Granular Cell Tumor in Eosinophilic Esophagitis

INTRODUCTION: Granular cell tumors (GCTs) are rare, soft tissue neoplasms arising from Schwann cells and can be found anywhere in the body. Gastrointestinal GCTs represent 6–10% of all cases with the majority arising in the esophagus. On endoscopy, GCTs appear as white-grey or a yellow subepithelial lesion. Most GCTs are benign, but approximately 0.5–2% of cases progress to malignancy and carry a poor prognosis. Eosinophilic esophagitis (EoE) is a chronic, immune-mediated condition characterized by eosinophil-predominant inflammation of the esophagus. We present three cases of esophageal GCTs diagnosed endoscopically in the setting of EoE. CASE DESCRIPTION/METHODS: Case 1: A 25 year-old female with asthma and environmental allergies presented with chronic dysphagia and intermittent solid food impaction never requiring endoscopy. She underwent EGD which showed mild inflammation. There was also a 5 mm small nodular area with adherent exudate (Figure 1). Biopsies of the proximal and distal esophagus revealed squamous mucosa containing innumerable intraepithelial eosinophils, consistent with EoE. Additionally, the nodule was found to be a GCT. Case 2 :A 38 year-old male with a history of biopsy-proven EoE presented for a follow up EGD after receiving PPI therapy for twelve weeks. EGD revealed a normal esophagus (Figure 2); however, random biopsies revealed incidental GCT in the mid-esophagus. Case 3: A 33 year-old male with a history of childhood environmental allergies presented with chronic dysphagia and intermittent solid food impaction. On EGD, the esophagus appeared normal. However, random biopsies from the mid esophagus showed eosinophilia consistent with EoE as well as a GCT. Therefore, repeat EGD was performed with white light along with endoscopic ultrasound to further evaluate for GCT and revealed a 2 mm submucosal lesion (Figure 3). DISCUSSION: The relationship between GCT and EoE remains unclear. Previously, case reports noted the simultaneous finding of GCT in patients with EoE on EGD. Here, we present three different endoscopic findings of esophageal GCT. Most notably, in two cases GCT was not diagnosed visually, but rather histologically. These cases add to the growing body of literature associating EoE and GCT, though a pathophysiologic relationship has not yet been drawn. Whether the presence of GCT in these cases is completely incidental or immune-mediated is unknown. Thus, as the prevalence of EoE continues to increase, clinicians should be aware of the association between GCT and EoE.Figure 1.: EGD of the esophagus revealing a 5 mm granulosa cell tumor (GCT).Figure 2.: Normal endoscopic appearance of the esophagus without endoscopic appearance of a GCT.Figure 3.: Submucosal GCT seen on white light endoscopy.

Lipoma gigante en la región abdominal del perro: Reporte de un caso

Lipoma is a benign mesenchymal neoplasm of adipocytes, most commonly presented in subcutaneous or dermal forms and its predominant occurrence is linked to the canine species. It is usually characterized by a single, well-circumscribed, subcutaneous, slow-growing mass that can be cured by surgical excision without the need for chemotherapy. And despite the low danger to the life of the animal, the welfare of the animal may be compromised due to its characteristic growth and ulcer formation, with associated pain. During treatment at the University Veterinary Hospital of the Federal University of Piauí, was treated an 8-year-old male dog, with no defined breed, presenting a pendular mass in the abdominal region, measuring approximately 30 cm in length, on palpation was possible, note a small ulcerated nodular areas next to the large mass. To confirm the diagnosis, cytology was performed from fine needle aspiration, which showed the presence of adipocyte clustering. Color Doppler ultrasonography showed no mass vascularization and radiographic exams showed no signs of metastasis. Surgical treatment with extirpation of the affected area was performed. Post-surgical treatment with Maxicam, Veterinary Pentabiotic, Tramadol Hydrochloride, daily local cleaning, Rifocin application and compressive bandage were instituted. The treatment was effective the animal had excellent recovery with no signs of recurrence.

C-KIT oncogene expression in PRKAR1A-mutant adrenal cortex.

Protein kinase A (PKA) regulatory subunit type 1A (PRKAR1A) defects lead to primary pigmented nodular adrenocortical disease (PPNAD). The KIT protooncogene (c-KIT) is not known to be expressed in the normal adrenal cortex (AC). In this study, we investigated the expression of c-KIT and its ligand, stem cell factor (SCF), in PPNAD and other cortisol-producing tumors of the adrenal cortex. mRNA and protein expression, by qRT-PCR, immunohistochemistry (IHC) and immunoblotting (IB), respectively, were studied. We then tested c-KIT and SCF responses to PRKAR1A introduction and PKA stimulation in adrenocortical cell lines CAR47 and H295R, which were also treated with the KIT inhibitor, imatinib mesylate (IM). Mice xenografted with H295R cells were treated with IM. There was increased c-KIT mRNA expression in PPNAD; IHC showed KIT and SCF immunoreactivity within certain nodular areas in PPNAD. IB data was consistent with IHC and mRNA data. PRKAR1A-deficient CAR47 cells expressed c-KIT; this was enhanced by forskolin and lowered by PRKAR1A reintroduction. Knockdown of PKA's catalytic subunit (PRKACA) by siRNA reduced c-KIT levels. Treatment of the CAR47 cells with IM resulted in reduced cell viability, growth arrest, and apoptosis. Treatment with IM of mice xenografted with H295 cells inhibited further tumor growth. We conclude that c-KIT is expressed in PPNAD, an expression that appears to be dependent on PRKAR1A and/or PKA activity. In a human adrenocortical cell line and its xenografts in mice, c-KIT inhibition decreased growth, suggesting that c-KIT inhibitors may be a reasonable alternative therapy to be tested in PPNAD, when other treatments are not optimal.

SUN-047 Cosmetic Injection of Silicone in a Transgender Person Leading to Granulomatous Disease with Hypercalcemia and Terminal Kidney Failure

Background: Granulomatous disease secondary to cosmetic injection of silicone is an uncommon cause of hypercalcemia. Transgender persons with limited access to appropriate surgery commonly use this procedure as an alternative, which can cause serious complications. Clinical Case: Forty year old transgender women with a 7-year history of nephrolithiasis treated with lithotripsy, pyelotomy and requiring the installation of a pig tail. She is admitted at the emergency room due to renal failure. Non-contrast CT rules out acute obstruction and lab tests conclude terminal kidney failure so she is started with dialysis and discharged. Two months later she returns with fever, she is diagnosed with central venous catheter-related bloodstream infection associated to the dialysis catheter and is started with antibiotics. Physical exam reveals pigmented, indurated nodular areas in her buttocks, hips and legs. The patient is re-interrogated and admits to have had injections of industrial silicone at the age of 22 in the described nodular areas. Regarding her transition process she had a vaginoplasty at 25 years old and was started with estrogens at that time but abandoned controls thereafter. Laboratory: Calcium 10, 8 (8,6-10,2 mg/dl), P 6 (2,5-4,5 mg/dl), PTH 17 (12-88 pg / ml), 25OHVD 3 (30ng / ml). Abdominal and thorax CT showed multiple pulmonary nodules, hilar, axillar and retroperitoneal adenopathies, hepatosplenomegaly, and subcutaneous granulomas with calcification in the buttocks and lumbar areas. HVB, HVC, VDRL and HIV serologies were negative as were ANA, pANCA and Rheumatoid Factor. Myelogram was normal. Biopsy of the involved skin and axillary lymph nodes revealed foreign body granulomatous reaction. Real Time PCR determination of the CYP27B1 mRNA showed a positive expression of this gene in the lesions confirming increased 1 alpha-hydroxylase activity as the cause of hypercalcemia. Tc-99m MDP scintigraphy showed increased activity in the soft tissue of the hips, buttocks and legs. Currently she maintains dialysis with normal values of calcium and is following regular controls in our gender program. Conclusion: Granulomatous disease due to cosmetic injection of silicone is a cause of hypercalcemia that should be suspected in the appropriate context. It is important to educate the transgender community about the possible severe adverse effects of these not authorized procedures.

In vivo effect of minocycline alone and in combination with immunotherapy against pythium insidiosum

This study examined the effect of minocycline alone and in combination with immunotherapy against pythiosis. Twenty rabbits, aged three months old and subcutaneously inoculated with Pythium insidiosum zoospores were divided into four groups (n = 5): treated with minocycline (10 mg/kg/day twice daily), treated with immunotherapy (34 mg subcutaneously every 14 days), treated with minocycline plus immunotherapy, and untreated (control group). The treatments were started 30 days after inoculation and continued for 70 days. The subcutaneous nodular injury areas in infected groups were measured every seven days after the beginning of treatment. Only the rabbits that developed lesions were selected for this study. When compared with the control group over 70 days, the minocycline and minocycline plus immunotherapy groups of rabbits with pythiosis showed significantly reduced injuries. The histopathology showed the presence of inflammation, macrophages and eosinophils. Grocott’s staining revealed irregular hyphae-like structures that were ramified and occasionally septate. Our results suggest that minocycline has fungistatic activity and that the combination of minocycline and immunotherapy is more effective than the individual therapies tested.

Lung-Digit Syndrome Related to an Adenosquamous Feline Lung Carcinoma

Background: The lung-digit syndrome is a rare syndrome characterized by the presence of a primary pulmonar neoplasia manifesting metastasis to the digit, occurring exclusively in felines. The diagnosis is based on the clinical signs, associated with radiographic and histopathological exams. There are no therapeutic protocols well-established, and surgical excision is considered controversial due to high recurrence and metastasis rates. The prognosis is considered poor, with low survival rates. The aim of this paper is to report a case of lung-digit syndrome attended in Rio de Janeiro.Case: A 12-year-old female cat, no defined race, was attend with the complaint of weight loss and injury in the right thoracic limb, already having histopathological diagnosis of squamous differentiation adenocarcinoma through biopsy. Physical examination showed no other clinical signs at first, including no signs of respiratory disease. Laboratory and imaging exams were performed, and the radiographic examination showed alterations in the pulmonary parenchyma showing a nodular area of increased radiographic density. Followed up by the manifestation of breathing noise at rest, as the first respiratory signs. The association of the clinical evaluation, medical history, and histopathological report from the limb lesion, lead to the suspicion diagnostic of lung-digit syndrome. The owners decide for the palliative treatment with chemotherapy, using carboplatin and prednisolone. Only the first session was performed, with the animal being euthanized due to clinical worsening 48 days after the initial manifestation of clinical signs. The diagnosis was confirmed by post mortem exams, and the pulmonary nodule were diagnosed as adenocarcinoma with squamous differentiation in concordance with the limb lesion diagnosis.Discussion: The lung-digit syndrome is still poorly studied in the veterinary medicine, with few reports published. Epidemiological data shows major incidence in elderly cats, with no racial or sexual predisposition, occurring in a 12-year-old cat in the present case. There are few theorys to explain the etiopathogenesis of the syndrome, however, none have been fully comproved. The clinical symptomatology varies from asymptomatic animals to those manifesting nonspecific systemic signs and respiratory signs. In this case the animal initially manifested only the presence of the digit injury and weight loss, with respiratory signs manifesting later. The digital lesion local signs usually include swelling, ulceration, purulent discharge, nail loss, and pain, occurring more frequently in the thoracic limbs, involving weight-bearing digits, as in the present report that occurred in the right thoracic limb, with involvement of the first and third digits. Radiographic findings may help in the diagnosis suspicion, having the confirmation by histopathological examination. The most frequent histological types involved in the syndrome are the adenocarcinoma, bronchoalveolar carcinoma, squamous cell carcinoma and adenosquamous carcinoma. The squamous-differentiated adenocarcinoma or adenosquamous carcinoma, as described in the histopathological report in the present case, is the least frequent. The treatment protocol is not well-established with no proven effective treatment. Surgical excision is not recomended as a palliative method, and the chemotherapy and radiotherapy sucess rates are still unknown. The prognosis is considered extremely unfavorable and early diagnosis represents the best attempt to control the disease.

Atypical parasitic lesions in slaughtered cattle in Southern Brazil.

In this study, we described the morphological features and unusual presentations of hydatidosis, fasciolosis, and eosinophilic myositis caused by Sarcocystis species diagnosed at the slaughterhouse lines. Twenty-seven samples of atypical parasitic lesions from distinct cattle were evaluated, of which 12 corresponded to hydatidosis, 11 to fasciolosis, and 4 to eosinophilic sarcocystosis. Hydatid cysts were observed mainly in the heart, with all cases involving the left ventricle. Fasciolosis lesions involved mainly the lungs, and were characterized by a focal nodular elevated area involving the edges of the lobes. Intralesional trematodes were observed in three cases. Sarcocystosis lesions were observed in four cases, and lesions were primarily located in the skeletal and cardiac muscles. Grossly, they presented as focal or focally extensive streaks, patches, or numerous nodules that were greenish to mildly yellowish. Histologically, all the cases had intralesional ruptured cysts of Sarcocystis that were associated with severe eosinophilic myositis and myocarditis. Parasitic lesions in atypical locations and/or with aberrant responses should be promptly identified because it may mistakenly diagnosed as other potentially zoonotic diseases, such as cysticercosis and tuberculosis.

Clincial Response of Central Nervous System Melanoma to Anti-PD1 Therapy in 2 Melanoma Patients

The first patient is a 76-year-old man who presented in June 2015 with aphasia and right hemiparesis. Imaging revealed a 3.5 × 3 × 1.3 cm left parietal mass. The patient underwent a craniotomy and pathology confirmed BRAF V600E mutant melanoma. He did not have a previous diagnosis of a primary melanoma. Subsequent PET imaging in August showed no evidence of systemic disease. Surveillance brain MRI in November showed several new nodular areas of enhancement along the dura and inter hemispheric falx, with CT of the body again not demonstrating other sites of disease. He was treated with whole brain radiation in December of 2015 (37.5 Gy, 15 fractions). In late January 2016 an MRI was done demonstrating mild progression of the anterior falcine dural metastatic disease. Given the progression was not significant and the patient was asymptomatic a watchful waiting approach was planned with a close follow-up MRI. Repeat MRI in March showed stable changes, thus surveillance continued.

FIBROMATOSIS: A RARE BENIGN TUMOR IN THE FACE

The 40-year-old white female patient complained of increased volume on face with no relevant past medical history. Extraoral physical examination showed painless volume increase in the right hemiface with 2 years of evolution, involving the parotid and masseter region, which was firm to palpation extending to the oral cavity without causing changes in the mucosa. Computed tomography scan of the face showed a calcified, multilocular, expansive lesion with precise limits invading chewing space on the right side causing erosion in the mandibular ramus. Incisional biopsy was performed, and the histologic slices in hematoxylin and eosin showed cellular proliferation infiltrating mucosa and skeletal striated muscle with nodular areas. Immunohistochemical analysis was positive for Ki-67, smooth muscle actin, and P16 and was negative for P63, ER, caldesmon, CD34, S100, desmin, and β-catenin. With the laboratory findings the diagnosis of fibromatosis was reached. Because of the impossibility of surgical resection, chemotherapy was started using tamoxifen, and the patient is being followed up. The 40-year-old white female patient complained of increased volume on face with no relevant past medical history. Extraoral physical examination showed painless volume increase in the right hemiface with 2 years of evolution, involving the parotid and masseter region, which was firm to palpation extending to the oral cavity without causing changes in the mucosa. Computed tomography scan of the face showed a calcified, multilocular, expansive lesion with precise limits invading chewing space on the right side causing erosion in the mandibular ramus. Incisional biopsy was performed, and the histologic slices in hematoxylin and eosin showed cellular proliferation infiltrating mucosa and skeletal striated muscle with nodular areas. Immunohistochemical analysis was positive for Ki-67, smooth muscle actin, and P16 and was negative for P63, ER, caldesmon, CD34, S100, desmin, and β-catenin. With the laboratory findings the diagnosis of fibromatosis was reached. Because of the impossibility of surgical resection, chemotherapy was started using tamoxifen, and the patient is being followed up.

SQUAMOUS CELL CARCINOMA ASSOCIATED WITH WHITE LESIONS

An 81-year-old white female, with no history of smoking or drinking, reported persistent pain and increase in lesion size in the last 3 months. Intraoral exam showed an exophytic nodular and ulcerated lesion in the region of left labial commissure, 1.5 cm in size and of soft consistency; next to the lesion, there was an extensive white plaque. A white plaque with striated appearance was also observed on the right buccal mucosa. An incisional biopsy was performed in the nodular area with the histopathologic diagnosis of squamous cell carcinoma. The patient was referred to the head and neck service for treatment. This carcinoma case may have been preceded by oral lichenoid lesions and proliferative verrucous leukoplakia and these lesions should be detected during clinical exam and followed closely, as they carry a higher risk for malignant transformation and carcinoma arising from them and may show better prognosis if detected early. An 81-year-old white female, with no history of smoking or drinking, reported persistent pain and increase in lesion size in the last 3 months. Intraoral exam showed an exophytic nodular and ulcerated lesion in the region of left labial commissure, 1.5 cm in size and of soft consistency; next to the lesion, there was an extensive white plaque. A white plaque with striated appearance was also observed on the right buccal mucosa. An incisional biopsy was performed in the nodular area with the histopathologic diagnosis of squamous cell carcinoma. The patient was referred to the head and neck service for treatment. This carcinoma case may have been preceded by oral lichenoid lesions and proliferative verrucous leukoplakia and these lesions should be detected during clinical exam and followed closely, as they carry a higher risk for malignant transformation and carcinoma arising from them and may show better prognosis if detected early.

Gastrointestinal Stromal Tumor Associated with Hypertrophic Osteopathy in a Dog

Background: Gastrointestinal stromal tumor (GIST) is a malignant mesenchymal neoplasm rarely described in the veterinary routine. The aim of this study was to report a case of GIST accompanied by a periosteal reaction, suggestive of hypertrophic osteopathy, in a dog.Case: An 11-year-old male dog had a history of progressive weight loss, difficulty in locomotion, and dyspnea. During clinical care, increased bone volume was observed. Blood samples were collected for a complete blood count and biochemical analysis. The dog also underwent thoracic radiography and abdominal ultrasonography. The test results revealed anemia, leukocytosis, hypocalcemia, hypoalbuminemia, and hypocholesterolemia. The radiographic images of the limbs showed a generalized periosteal reaction, and thoracic radiography indicated changes compatible with mild chronic lung disease. Ultrasonographic findings indicated a neoformation in the intestinal loop of the right mesogastric region and increased volume in the left testicle, both of which were indicative of neoplasia. Therefore, the dog was referred for surgery, wherein the intestinal mass and both testes were removed; the intestinal mass and left testicle were subjected to histopathological diagnosis. The results of the biopsies confirmed that the testicular neoplasm was a seminoma, whereas the intestinal nodule was compatible with GIST, and immunohistochemical analysis was necessary to confirm the diagnosis. On the basis of positive labeling for the antibodies vimentin, desmin, S100, and c-kit, the diagnosis of GIST was confirmed. Therefore, the animal underwent metronomic chemotherapy with 12 mg/m2 cyclophosphamide every 24 h for 3 months, and thereafter every 48 h for 6 months. Moreover, the dog was periodically monitored via imaging (radiography of the anterior and posterior limbs, abdominal ultrasonography, and computed tomography). A few months after the surgical resection of the intestinal nodule, radiography revealed that the periosteal reactions had disappeared, but ultrasonography revealed nonspecific alterations of mild thickening and enlargement of the intestinal loops. Computed tomography revealed two nodular areas of soft-tissue attenuation in the right mesogastric region. Although the possibility of tumor recurrence was raised, the animal’s owner chose only to perform palliative treatment. After 4 months, ultrasonography revealed a neoformation in the right mesogastric region. On July 29, 2018 the animal had a worsening of its clinical condiction, with tumor recurrence by ultrasound exam. The owners didn´t accept returning to the oncologist and made an option for a conservative treatment with tramadol (3 mg/kg/TID), dipirone(25 mg/kg/TID), and vitaminic supplement based on docosahexaenoic acid plus eicosapentaenoic acid (30 mg/kg, once daily) On August, 28, 2018 he was euthanized. The dog’s postsurgical survival time was 15 months.Discussion: The dog described in the present report was large and elderly, both of which are predisposing factors for GIST development. Although non-specific, progressive weight loss may be associated with intestinal neoplasia, owing to the impairment of digestion and nutrient absorption, because a part of the duodenal mucosa was compromised by neoplasia. The difficulty in locomotion, which did not respond to treatment, was attributed to the periosteal reaction confirmed by radiographic exam. This, in turn, was associated with hypertrophic osteopathy secondary to neoplasia, because the condition regressed after the removal of the intestinal nodule. The histopathological findings related to intestinal neoplasia were suggestive of GIST; positive immunoblotting for c-kit, vimentin, S100, and desmin confirmed the diagnosis. Although the veterinarian alerted the owner to the suspicion of recurrence and suggested continuing treatment, the owner chose not to.

Cerebral Cavernous Hemangioma in a Dog

Background: Cerebral cavernous hemangioma is a rare neoplasm of vascular origin in the brain, characterized by abnormally dilated vascular channels surrounded by endothelium without muscle or elastic fibers. Presumptive diagnosis is performed by magnetic resonance or computed tomography (CT) scanning and can be confirmed by histopathology. The prognosis of intracranial cavernous hemangioma is poor, with progression of clinical signs culminating in spontaneous death or euthanasia. The purpose of this paper is to report a case of cerebral cavernous hemangioma in a dog, presenting the clinical findings, tomographic changes, and pathological findings.Case: This case involved a 2-year-old medium sized mixed breed female dog presenting with apathy, hyporexia, ataxia, bradycardia, dyspnea, and seizure episodes for three days. Hemogram and serum biochemistry of renal and hepatic function and urinalysis did not reveal any visible changes. CT scanning was also performed. The scans revealed a hyperdense nodule of 15.9 x 14 mm, with well defined borders, and a hypodense halo without post-contrast enhancement and mass effect in the right parietal lobe was observed in both transverse and coronal sections. Based on the image presented in the CT scans, the nodule was defined as a hemorrhagic brain lesion. The animal died after a seizure. The right telencephalon was subjected to necropsy, which revealed a reddish-black wel-defined nodule 1.7 cm in diameter extending from the height of the piriform lobe to the olfactory trine at the groove level and extending towards the lateral ventricle, with slight compression and deformation of the thalamus but no other macroscopic alterations in the other organs. The histopathology indicated that this nodular area in the encephalus contained moderate, well-delimited but unencapsulated cellularity, composed of large vascular spaces paved with endothelial cells filled with erythrocytes, some containing eosinophilic fibrillar material (fibrin) and others with organized thrombus containing occasional neutrophil aggregates. The endothelial cells had cytoplasm with indistinct borders, elongated nuclei, scanty crust-like chromatin, and cellular pleomorphism ranging from discrete to moderate, without mitotic figures.Discussion: The histological findings characterized the morphological changes in the brain as cavernous hemangioma, and the growth and compression of this neoplasm were considered the cause of the clinical signs of this dog. The main complaint was seizures, although ataxia and lethargy were also noted. These clinical signs are often related to changes in the anterior brain and brainstem. The literature does not list computed tomography as a complementary diagnostic method in cases of cerebral cavernous hemangioma in dogs, but CT scanning was useful in confirming cerebral hemorrhage. The main differential diagnosis for cerebral cavernous hemangioma would be a hamartoma, but what differentiates them histologically is the presence of normal interstices between the blood vessels, since no intervening neural tissue occurs in the case of cerebral hemangioma. Therefore, even in the absence of immunohistochemistry to more confidently confirm a cavernous hemangioma, the clinical signs, CT scans and especially the pathological findings were consistent with a case of cerebral cavernous hemangioma, a benign neoplasm with a poor prognosis due to the severe neurological changes it causes and its difficult treatment.

Malignant melanoma of brown bullhead (Ameiurus nebulosus) in Lake Memphremagog, Vermont/Quebec

In 2012, brown bullhead (Ameiurus nebulosus) with large, raised, black growths were first reported from multiple areas within the Vermont portion of Lake Memphremagog. Subsequent surveys conducted from 2014 to 2017 at two sites within the lake indicated a prevalence of 30% in adult brown bullhead 200mm and above total length. These lesions ranged from slightly raised smooth black areas to large nodular areas on the body surface and fins and within the oral cavity. Microscopically, these lesions were determined to be malignant melanoma with invasion into surrounding hypodermis, skeletal muscle and bone as well as metastases to gill, ovary and intestine. Liver neoplasms were also observed in 8% of the bullhead collected from Lake Memphremagog in 2015. Neither skin nor liver neoplasms were noted in Ticklenaked Pond, a site used for comparison.

2519 A Rare Case of Mucosa-Associated Lymphoid Tissue (MALT) Lymphoma Involving Duodenum

INTRODUCTION: Primary small bowel lymphomas account for only 8-9% primary gastrointestinal malignancies and mostly represent extra nodal B- or T-cell origin. Immunoproliferative small bowel disease (IPSID) is a common small bowel extra nodal primary lymphoma. Associated Lymphoid Tissue B cell lymphoma (MALT lymphoma) is a common primary gastric lymphoma. However, it is very rare to find MALT lymphoma in small bowel especially in duodenum. We report a unique case of MALT lymphoma involving duodenal bulb. CASE DESCRIPTION/METHODS: A 58-Yr-Old African American female, with history of type II diabetes, Hypertension, Osteoarthritis, and chronic GERD with symptom control on once a day low dose omeprazole was referred for evaluation of epigastric discomfort, bloating, and nausea for 2 months. Abdominal examination normal. Labs showed normal CBC with differential, and CMP. An upper endoscopy showed mild patchy erythema in stomach and a localized, raised, and nodular area about 4-5 cm in the duodenal bulb. Biopsies from stomach showed Helicobacter pylori chronic active gastritis. Biopsies from duodenum showed homogenous population of lymphoid cells with moderate amounts of clear cytoplasm expanding the lamina propria. Molecular studies showed positive B-cell Gene Rearrangement with positive Framework 1, 2, and 3. Immunohistochemical staining was positive for CD20, bcl2, and kappa; and negative for CD3, CD5, CD10, bcl6, CD43, Cyclin D1 and lamda. The pathology findings confirmed Margina zone lymphoma of Mucosa Associated Lymphoid Tissue (MALT lymphoma). Colonoscopy was normal except few diverticula. A CT scan of abdomen, pelvis, and chest did not show any other masses except prominent lymph nodes at the gastrohepatic ligament and duodenum. She is treated with antibiotic therapy for H pylori gastritis, and referred to oncology for further management. DISCUSSION: MALT lymphomas of stomach usually occur in females and can be associated with Helicobacter pylori infection. They are usually low grade lymphomas and localized to gastrointestinal epithelium. Clinical features, imaging, or endoscopy findings are not specific. Immunohistochemical staining shows positive for B cell phenotype markers like CD20, Bcl-2, and negative for CD 23, CD 10, and CD 5. Usually, they present with non-specific symptoms like epigastric pain, nausea, vomiting, early satiety, though can present with obstruction when the tumor is too large. Treatment of associated H pylori infection may help though chemoradiotherapy may be needed rarely.

1781 Esophageal Squamous Cell Carcinoma Masquerading as Neuropathic Pain

INTRODUCTION: The most common symptoms for esophageal carcinoma are dysphagia and odynophagia, though most are asymptomatic. We describe a case of upper esophageal squamous cell carcinoma masquerading as subphrenic neuropathic pain, not previously described in the literature. CASE DESCRIPTION/METHODS: A 67 year-old male with a past medical history of cholecystitis status post cholecystectomy, hepatic steatosis, acid reflux, hyperlipidemia, and 5-pack year tobacco history presented with 2 years of neuropathic subphrenic pain that radiated below the subcostal margins. The ingestion of solids and liquids triggered the pain, lasting for 1-2 seconds. The pain did not respond to viscous lidocaine or proton pump inhibitors. EGD two years prior revealed a nodular area of mucosa suspicious for esophageal squamous papilloma. His BMI was 29.6 and physical examination was otherwise unremarkable. He had normal peristalsis on esophageal manometry. EGD revealed a nodular mucosal patch in the upper third of the esophagus. Biopsies reproduced the patient’s sharp neuropathic pain. Pathology revealed ulcerated high-grade squamous dysplasia with a focus suspicious for stromal invasion. The stomach demonstrated segmental moderate mucosal changes characterized by atrophy, discoloration, and altered texture along the greater curvature of the gastric body with chronic inactive gastritis with extensive intestinal metaplasia on biopsy. H. pylori testing was negative.Endoscopic ultrasound demonstrated a confirmed a 4cm superficial lesion involving 1/3rd of the lumen. Endoscopic submucosal dissection was performed and pathology demonstrated a T1a squamous cell carcinoma with infiltration into the lamina propria and a focal tongue of tumor in the superficial muscularis mucosa. His pain resolved following resection. DISCUSSION: Esophageal squamous cell carcinoma masquerading as neuropathic pain is highly unusual. The mechanism behind this presentation remains unclear. The squamous papilloma visualized on prior EGD was a risk factor for the development of carcinoma. This case illustrates that squamous cell carcinoma can present with neuropathic pain, a symptom with swallowing that warrants further investigation.

Lymphoblastic Transformation of Follicular Lymphoma: A Case Report and Review of the Literature

Abstract Follicular lymphoma (FL) is one of the most common B-cell non-Hodgkin lymphomas in adults in the Western world. Prognosis is correlated with the clinical stage and histologic grade and, increasingly, the tumor genetic profile. A subset of patients with FL experiences histologic disease progression (ie, histologic transformation), of which the majority will demonstrate diffuse large B-cell lymphoma. We describe a patient initially diagnosed with low-grade FL with relapsed disease presenting as acute renal failure due to diffuse abdominal lymphadenopathy. Excisional biopsy of an inguinal lymph node at relapse showed high-grade areas with sheets of immature-appearing lymphoid cells adjacent to nodular areas characteristic of low-grade FL. Cells of both components were positive for BCL2 and CD19. The cells of the high-grade component were positive for CD99 and TdT and negative for CD20, whereas cells of the low-grade component were positive for CD20 and negative for CD99 and TdT. Fluorescence in situ hybridization studies demonstrated the IGH/BCL2 rearrangement in addition to an MYC rearrangement in both low- and high-grade components.

PB2238 AGGRESSIVE SYSTEMIC MASTOCYTOSIS: A DIAGNOSTIC CHALLENGE IN A PATIENT WITH MYOTONIC DYSTROPHY TYPE 2

Background:Systemic mastocytosis (SM) is a clonal neoplastic disease of mast cells (MCs) with heterogeneous clinical presentations, different organ infiltration, and is often overlooked. The spectrum ranges from indolent to aggressive forms. SM could also be associated with myeloid and lymphoid hematologic diseases.Aims:We present a case where SM was misdiagnosed as a progression of myotonic dystrophy.Methods:A 55‐year‐old caucasian woman with known familial myotonic dystrophy type 2 (DM2) proven by CCTG‐repeat expansion in the ZNF9 gene was referred with a diagnosis of unclassified myeloproliferative neoplasm with 10% bone marrow (BM) blasts and mutations in KRAS, ASXL1 and RUNX1. In the last two months, she complained of weight loss, muscle weakness, and fever. Because of progressive dysphagia, enteral nutrition by percutaneous endoscopic gastrostomy tube (PEG) was initiated. Clinically, a body mass index (BMI) of 13.6 kg/m2, normal skin, and hepatosplenomegaly were seen. Hilar lymphadenopathy and pulmonary infiltrates were detected by CT‐scan. Laboratory analysis showed a WBC count of 39.80x109/L with left shift, Hb of 4.4 g/dl, platelets of 78x109/L, hypoalbuminemia, and an elevated lactate dehydrogenase. Rapid neurological deterioration is typical in myotonic dystrophy type 1, which is associated with a worse prognosis and is unusual for DM2 (Schoser B, Timchenko L.; Current Genomics; 2010). Curiously, DM2 was genetically proven.Results:Assessment of serum tryptase is part of our routine diagnostic work‐up for hematologic diseases and was 46.9 μg/L (reference range <11 μg/l) in the patient. A repeat BM biopsy revealed a hypercellular marrow with delayed myelopoietic maturation and a normal blast count. There were peritrabecular conspicuous nodular areas with minimal reticular fibrosis. Therein, multifocal clustering of MCs (at least 15/cluster) co‐expressing CD2, CD25, CD117, and mast cell tryptase were detected. Cytogenetics were normal but the activating mutation KITD816 V was present.Thus, a diagnosis of SM could be made based on the major criterion (≥ 15 MC in clusters) and three minor criteria (the KIT mutation, CD25/CD2 co‐expression, serum tryptase > 20 μg/l). Due to the presence of C‐findings (transfusion dependent anemia, splenomegaly with thrombocytopenia, and malabsorption with weight loss), the criteria for aggressive SM (ASM) were fulfilled. A hilar lymph node biopsy revealed several small lymphocytes next to dense blastoid cell infiltrates of myeloid lineage without MCs consistent with extramedullary hematopoiesis.Together with the neurologist, we hypothesized that ASM and not the natural course of DM2 was causative for the rapid neurological deterioration. Treatment with the KIT‐tyrosine kinase inhibitor midostaurin was initiated. Within three months, the patient gained weight, regained muscle power, dysphagia disappeared, and the PEG could be removed. The blood count normalized, and hilar lymphadenopathy disappeared. Currently, allogeneic hematopoietic cell transplantation is planned because of the poor prognosis of ASM, particularly if KIT‐independent oncogenic driver mutations such as KRAS, ASXL1 and RUNX1 were found.Summary/Conclusion:In summary, awareness of physicians to SM as a possibility in the differential diagnosis of unclear clinical scenarios is the first step to make the diagnosis and offer adequate therapy. Serum tryptase is a simple tool that could draw the attention of physicians to SM.

Influence of nodular defect size on metal dielectric mixed gratings for ultra-short ultra-high intensity laser system

With the development of the high power laser facilities, laser resistance of pulse compression grating is getting increased attention. The metal dielectric mixed grating (MDMG) is widely studied, because it has wide diffraction bandwidth, low surface stress and potential high threshold. The effect of nodular defect size on damage of MDMG is studied by experiments and theoretical analysis in two typical cases. The initial damages appear in the district of nodular defect due to local electric field enhancement induced by nodular defect, which is different with the initial damage located in grating pillar of the perfect MDMG. Laser induced ionization theory is introduced to describe the damage process and give a quantitative analysis on the relationship of laser damage and nodular defect size. It is concluded that local electric field enhancement in nodular defect area would cause the electron density to reach the critical value and lead to the damage occurrence, and nodule damage critical diameters are 1400 nm and 1500 nm respectively in case 1 (seed under the grating pillar) and case 2 (seed under the grating groove) which will lead to nodule damage initiation.

Signet ring squamous cell carcinoma – a case report

Squamous cell carcinoma (SCC) is the second most common type of skin cancer.1 A rare variant signet ring SCC cell was first described in 1980 by Cramer and Heggeness, who described these lesions macroscopically as an ulcerated nodular or papular area on sun damaged skin, typically occurring in the head and neck.1 There are several other histological subtypes of SCC including keratoacanthoma, acantholytic, spindle cell, verrucous, clear cell, papillary, pigmented, and desmoplastic SCC.2

Early diagnosis of subtype in early clinical stage lung adenocarcinoma by using an autoantibody panel and computed tomography

Objective: To explore the value of CT and autoantibody panel to diagnosis the subtype in early clinical stage lung cancer,especially lepidic predominant adenocarcinoma (LPA),and to provide the correct information for the clinical and the prognosis evaluation. Methods: A retrospective study was conducted of 60 patients (total 63 subsolid nodules,which included 39 PSN and 24 pGGN) who underwent surgical resection or needle biopsy for stage Ⅰa or Ⅰb lung adenocarcinoma at Affiliated Tumor Hospital of Zhengzhou University between June 2017 and April 2018,age from 28 to 82 years old,and the average age was (52±7) years. All patients underwent a pretreatment chest LDCT and the peripheral blood of all patients were used to detect the tumor related antibody (including p53, GAGE7, PGP9.5, CAGE, MAGEA1, SOX2, GBU4-5) through enzyme linked immunosorbent assay. All the patients were divided into LPA group (43 subsolid nodules, which included AIS 10 subsolid nodules, MIA 11 subsolid nodules, LPA 22 subsolid nodules) and invasive predominant adenocarcinoma (IPA) group (20 subsolid nodules). The information of CT scanning was measured and assessed in lung and mediastinal windows with double blind method. The mean computed tomography (m-CT) value and the solid component/tumor ratio in three-dimensional (3D) and two-dimensional (2D) planes were measured and analyzed using computer-aided diagnosis (CAD) system. Results: There were 20 partial solid nodules in IPA group, 19 partial solid nodules in LPA group and 24 ground-glass nodules (χ(2)=19.278,P=0.000). There were 4 circular nodules, 16 irregular nodules in the IPA group, 21 circular nodules, 5 oval nodules and 7 irregular nodules in the LPA group χ(2)=8.587,P=0.003). The incidence of burr, vascular aggregation and bronchial truncation in IPA group was higher than that in LPA group (40.0% vs 16.3%, 70.0% vs 18.6%, 30.0% vs 2.3%, χ(2)=4.234,15.860,10.580, P=0.040,0.000, 0.001). The incidence of clear tumor lung interface in patients in LPA group was significantly higher than that in patients in IPA group (97.7% vs 65.0%, χ(2)=13.146,P=0.00). Of all the quantitative analysis of nodules,the m-CT value, the solid component/tumor ratios in three-dimensional (3D) and two-dimensional (2D) planes in IPA group were higher than those of LPA group ((-180±156) vs (-410±213) HU, 0.44±0.32 vs 0.14±0.26, 0.54±0.26 vs 0.18±0.26, t=-4.208, -3.951、-5.166, P=0.000, 0.000, 0.000). Among the 60 patients with lung cancer, there were 33 cases with positive antibody in peripheral blood, with a positive rate of 55.0%. The positive rate of 7-AABs was 70.0% in IPA group and 44.2% in LPA group, which had no statistical difference (χ(2)=3.647, P=0.056), the positive expression of tumor-associated antibodies was independent of the patient's age, CT value and nodular solid components and lung nodular volume ratio and area ratio, P>0.05, only in correlation with pleural traction (χ(2)=3.866, P=0.049). Conclusion: Compared with IPA, the imaging features of LDCT about the mGGN and PGGN appearance, clear tumor-lung interface, low m-CT and the solid component/tumor ratio in two-dimensional or three-dimensional (3D) planes are benefit for the diagnosis of the LPA; the expression of tumor-associated antibody group is independent of the age of the patient and the number of nodular solid components, and is only related to pleural depression, which is not conducive to the identification of LPA and IPA.