Cm In Greatest Dimension Research Articles

Tumeur glomique intracardiaque : une tumeur péricytaire (périvasculaire) de localisation inhabituelle

Glomus tumor are rare mesenchymal neoplasm, belonging to the pericytic (perivascular) tumor family, witch recent molecular characterization has allowed highlight recurrent molecular abnormalities. In fact, glomus tumor involves frequent MIR143-NOTCH gene fusion whereas others pericytic tumor (myopericytoma and myofibroma) involve mutations of PDGFRB gene. Glomus tumor are usually developed in superficial localization. However visceral locations have been described. Cardiac location is exceptional with only one case reported in literature. Here, we report the case of cardiac glomus tumor (glomangiomyoma) developed in the left ventricle in a 34 year-old patient, diagnosed after chest pain. The length of tumor was 4cm in greatest dimension. Histologically, the tumor concerned both round glomus cells and smooth muscle cells with prominent branching thin-walled vessels. By immunohistochemistry, these two contingents exhibited diffuse expression of smooth muscle actin and heterogeneous expression of H-caldesmone whereas cytokeratins, melanocytic markers and chomogranine were negative. Next Generation molecular analysis using RNA sequencing highlighted the characteristic MIR143-NOTCH gene fusion witch supports the diagnosis of glomus tumor. In this observation, we recall histological and immunohistochemistry features of glomus tumor and we make a synthesis concerning the molecular data recently described in sporadic glomus tumor.

Reconsidering the American Joint Committee on Cancer Eighth Edition TNM Staging Manual Classifications for T2b and T3 NSCLC

IntroductionThe American Joint Committee on Cancer (AJCC) eighth edition TNM staging manual for NSCLC, derived from the International Association for the Study of Lung Cancer (IASLC) Staging Project, designates tumors with additional nodule(s) in the same lobe as T3. This study sought to externally validate the results of the IASLC, which showed a trend in improved survival for such tumors, but excluded treatment-based adjustment, by assessing whether these tumors have worse survival than T2b NSCLC. MethodsOverall survival of patients with T2b-T3, N0-3, M0 NSCLC (satisfying a single T descriptor of tumors >4 cm but ≤5 cm in greatest dimension [“T2b”], tumors >5 cm but ≤7 cm in greatest dimension [“T3-Size”], or tumors with additional nodule(s) in the same lobe [“T3-Add”]), according to the AJCC eighth edition, in the National Cancer Database (2010–2015), was evaluated using multivariable Cox proportional hazards modeling and propensity score matching. Results31,563 patients with T2b-T3, N0-3, M0 NSCLC met the study inclusion criteria. In multivariable-adjusted analysis, T3-Add tumors had improved overall survival compared with T3-Size tumors (Hazard Ratio = 0.86, 95% Confidence Interval: 0.82–0.89, p < 0.001) and similar survival compared with T2b tumors (Hazard Ratio = 1.04, 95% Confidence Interval: 0.97–1.12, p = 0.28). A propensity score-matched analysis of 2260 T3-Add and 2,260 T2b patients, well-balanced on 16 common prognostic covariates, including treatment type (surgery, chemotherapy, or radiation), revealed similar 5-year survival (53.4% versus 52.3%, p = 0.30). ConclusionsIn this national analysis, T3-Add tumors had better survival than other T3 tumors and similar survival to T2b tumors. These findings may be taken into consideration for the AJCC ninth edition staging classifications.

Free Tissue Transfer for Repair of Chronic Esophageal Perforations.

ObjectiveAnterior cervical discectomy and fusion have become a common intervention for cervical spine stabilization. However, complications can cause life-threatening morbidity. Among them, esophageal perforation is associated with severe morbidity, including dysphagia, malnutrition, and infection with the potential development of mediastinitis. Presentation is variable but often results in chronic morbidity. Herein we examine our experiences in the management of esophageal perforation with microvascular free tissue transfer.Study DesignRetrospective review from January 2013 to September 2020.SettingSingle academic tertiary care center.MethodsThis study comprised all patients (age, 41-73 years) undergoing free tissue transfer for the repair of chronic esophageal perforation secondary to anterior cervical discectomy and fusion at an academic tertiary care center. Four patients underwent repair via vastus lateralis myofascial onlay grafting for defects ≤2 cm in greatest dimension, while 1 patient underwent a fasciocutaneous radial forearm free flap repair of an 11 × 5–cm defect.ResultsDefect location ranged from hypopharynx to cervical esophagus. Mean operative time was 6.2 hours; the average length of stay for all patients was 6.6 days. Of 5 patients, 1 required additional hardware placement for spine stabilization. All patients underwent gastrostomy tube placement to bypass the surgical site during healing, and all eventually resumed an oral diet postoperatively. Recurrent fistula occurred in 1 of 5 patients. No flap failures were encountered in the study population.ConclusionVastus lateralis myofascial onlay grafting and fasciocutaneous radial forearm free flap are robust, relatively low-morbidity interventions with a high success rate for definitive repair of chronic esophageal perforation. Repair should be undertaken in concert with a spine surgeon for management of the cervical spine.

Clinicopathological Study of Subcutaneous Heminthicnodules : Unusual Sites and Diagnostic Dilemmas

Introduction: Superficial palpable swellings with helmintic infection as an underlying etiology is usually an accidental finding in the surgically excised specimens. Somatic nematodes and cestodes are the commonly implicated organisms, and the zoonotic nematodes show an emerging trend. The present study aims to reappraise the histopathological findings of helminthic etiology in superficial swellings which were clinically suspected to be of neoplastic/non neoplastic nature. Materials and methods: Thirty six cases of palpable superficial nodules with infective etiology over a period of five years were reviewed. 19/36 were of helminthic etiology were included in the present study. Pertinent demographic and clinical data were retrieved from the medical archives. Results: Amongst the 19 cases, 8 were males and 11 females. Chest wall (4/19), and eyelids (3/19) were the most common sites involved. The size ranged from 0.8-15 cm in greatest dimension. Presence of histiocytes (13/19), granulomas (11/19), eosinophils (10/19), and giant cells (9/19) were the most consistent histological findings. 14 cases had discernible parasite morphology with diagnosis of filarial worms (7/19), Dirofilaria (3/19), cysticercosis (4/19), and hydatid cyst (1/19). Four cases had dead and calcified parasites with no discernible morphology. Conclusion: Granulomatous inflammation and tissue eosinophilia are strong indicators of a parasitic etiology. Subcutaneous and intramuscular filariasis, cysticercosis and hydatid cyst are well documented etoiologies whereas Dirofilariasis is an emerging zoonotic infection with worldwide case reports. Imaging techniques and fine needle aspiration can point towards the diagnosis; however in the absence of characteristic features, histopathology can be relied upon to diagnose a helminthicetiology.

Large Ovarian Follicle Cyst: Benign Mimic of Cystic Adult Granulosa Cell Tumor.

While most ovarian follicle cysts are <8 cm in greatest dimension, much larger follicle cysts (up to 18.5 cm) have been reported. To our knowledge, the FOXL2 mutation status of such cases has not been documented in the literature. Here, we report the features of a 14 cm ovarian cyst with no FOXL2 mutation detected by targeted next-generation sequencing. While adult granulosa cell tumor was the chief entity in our differential diagnosis, the absence of convincing nuclear grooves, lack of architectural variability, presence of a theca layer, and absence of FOXL2 mutation were consistent with a diagnosis of ovarian follicle cyst.

Not a Huge but Two Tumors

A 73-year-old man presented to the thoracic surgery clinic with progressive difficulty breathing for about 3 months. He had a benign spindle cell tumor (about 3 centimeter in diameter) in right lung upper lobe, which had been resected by thoracotomy surgery 15 years earlier. The physical examination showed weakened right respiratory sounds. Computed tomography of the chest revealed a huge mass closed to the pulmonary vessels and bronchus, which measured more than 13 cm in greatest dimension (Panel A). Thoracotomy surgery was performed for the tumor resection again. Two separated tumors, instead of one huge tumor, originated from visceral pleura were found with well encapsulated (Panel B). And the histopathological analysis revealed solitary fibrous tumor. The immunohistochemical analysis was positive for Desmin-protein and CD34. Just followingup was initiated after surgery, and the patient was doing well after 3 months.

Case series of vestibular schwannoma patients with no asymmetry in hearing

Objective(1) Describe the presenting symptoms and tumor characteristics of patients who are diagnosed with a vestibular schwannoma (VS) with normal hearing or symmetric hearing loss, and (2) report the prospective growth and treatment of each tumor. MethodsThis is a retrospective cohort study of acoustic neuroma patients who were found to have a VS and normal or symmetric hearing loss at a single tertiary care academic center from 1999 to 2012. Medical records were reviewed collecting the following information: patient demographics, symptoms, MRI characteristics, and treatment modality. Results15/195 (7.7%) patients met criteria for the study. Dizziness was the most common presenting symptom occurring in 9 subjects (60%), followed by headache in 6 (40%), vision changes in 5 (33%), tinnitus in 5 (33%), and unilateral hearing loss in 1 (7%). The average tumor size was 1.07 cm (range 0.3–2.2 cm). Eight (53%) patients presented with intracanalicular tumors. Growth was observed in 6 subjects (40%) with average growth rate in those who exhibited mean growth of 1.8 mm per year. Treatment consisted of surgical removal in 4 cases (27%), gamma knife therapy in 1 (7%), and observation or loss to follow-up in 10 (66%). ConclusionsThe incidence of VS with normal hearing was 7.7% Patients with VSs who do not exhibit unilateral hearing loss present most commonly with dizziness. Most of our patients had small, intracanalicular tumors with the largest tumor measuring 2.2 cm in greatest dimension. Of those who were managed conservatively with repeat imaging and observation, most showed tumor growth.

Müllerian Mucinous Cystadenomas of the Ovary: A Report of 25 Cases of an Unheralded Benign Ovarian Neoplasm Often Associated With Endometriosis and a Brief Consideration of Neoplasms Arising From the Latter.

A subset of ovarian mucinous tumors demonstrates müllerian-type epithelium, with such lesions variably designated "endocervical-like" and seromucinous since their popularization based on a report of borderline examples in 1989. While müllerian mucinous borderline tumors and carcinomas have been highlighted in the literature, there has been minimal attention given to benign müllerian mucinous tumors, particularly müllerian mucinous cystadenomas. Given the paucity of literature describing the features of müllerian mucinous cystadenomas/cystadenofibromas, diagnostic difficulties may arise when papillary features are present and in cases that show a subtle transition from endometriosis. We thus reviewed 25 cases of müllerian mucinous cystadenoma/cystadenofibroma to highlight the notable characteristics of this entity, including gross, cytologic, and architectural features that aid in the distinction from müllerian mucinous borderline tumors as well as, rarely, metastatic tumors. The patients ranged in age from 26 to 85 yr old. Bilateral ovarian involvement was frequent (40%). The ovaries ranged from 2.3 to 26 cm in greatest dimension. Most were multicystic (18 cases) and contained tenacious mucoid material (14 cases). All cases demonstrated predominantly columnar mucinous epithelium with abundant pale-pink cytoplasm. A minor component of ciliated and endometrioid epithelium was seen in 15 and 2 cases, respectively. Broad papillary formations were frequently encountered (9 cases) as was epithelial papillary tufting comprising <10% of the tumor (6 cases). Endometriosis was present in 9 cases, with a transition from endometriosis to mucinous epithelium noted in 8 cases. This series highlights the morphologic features of a relatively uncommon, benign, endometriosis-associated ovarian tumor that may be confused with a müllerian mucinous borderline tumor or bland metastatic mucinous tumors. It also provides an argument for the terminology "müllerian mucinous cystadenoma" or "cystadenofibroma" rather than "seromucinous cystadenoma" due to the frequent association with endometriosis as well as the dominant mucinous epithelium.

Head and Neck Sarcomas-clinicopathological Findings, Treatment Modalities and Its Outcome - A Retrospective Study.

Introduction:Sarcomas are malignant neoplasms constituting an important group of tumours due to their high morbidity and mortality. They can arise either from soft tissue or bone. Soft tissue sarcomas are common, but bone sarcomas are rare. The aims and objective of this study are to carry out the study of head-and-neck sarcomas between January 2006 and May 2020.Materials and Methods:Eighty-two patients of head-and-neck sarcoma were included. Study of demographic and clinicopathological characteristics including age, sex, gender, presenting symptoms, origin, primary anatomical location, size, tumour grade, staging, treatment modality, recurrence, metastasis, and patient status during the recent visit.Results:Fifty-six (68.3%) were males and 26 (31.7%) were females. The male/female ratio was 2.28:1. The most affected age group was 0–19 years. Tumour size of >5 cm in greatest dimension was seen in 67.1% cases and tumour size of <5 cm in 32.9%. Chemotherapy + radiotherapy (CT + RT) was given in 23.8% followed by surgery in 13.9%. Recurrence was seen in 19.5% and metastasis in 18.3%.Discussion:Rhabdomyosarcoma was the most predominant histological type. Maximum patients reported in late stage. The most commonly used treatment was the combination of CT and RT. Recurrence was seen in 19.5% and distant metastasis in 18.3% patients. Head-and-neck sarcomas are rare so epidemiological studies involving more cases must be carried out for better understanding and better outcome.

Pediatric Pancreatic Teratoma: A Rare Case Report

Abstract Introduction/Objective Teratomas are germ cell neoplasms arising from three germinal layers. They are classified as mature or immature. Mature cystic teratomas (MCTs), also known as dermoid cysts, are benign lesions. Common locations include the sacrococcygeal region, ovaries, testes, brain, mediastinumand retroperitoneum. MCT is extraordinarily rare in the pediatric pancreas; only 11 such cases have been reported in the English literature. Methods This eleven-month-old female presented for evaluation of two cystic masses in the body and tail of the pancreas. These masses were initially imaged as anechoic cysts in utero by ultrasound at 19 weeks gestational age and followed carefully over the interval. Her past medical history included a left upper lobectomy for congenital lobar emphysema. The pancreatic cysts were excised by laparoscopy. Results Grossly, the two tan-pink cysts measured 2.5 cm and 3.1 cm in greatest dimensions, had smooth inner linings and contained clear fluid. Microscopically, the cyst linings included low cuboidal, columnar, ciliated pseudostratified columnar respiratory or fundal gastric epithelia with underlying serous/mucous glands and smooth muscle. These findings were diagnosed as MCT. Typically, MCT of pancreas have nonspecific clinical presentations, serum markers and imaging; all depending on the tissue types present; so, the diagnosis is primarily made postoperatively by pathological evaluation. Previously reported cases presented as single cysts, ranged 4 months to 16 years in age and measured 8 to 18 cm in greatest dimension. Complete surgical excision and careful follow-upare the mainstay of therapy Conclusion Pediatric pancreatic MCTs are extraordinarily rare, so the preoperative diagnosis and treatment is difficult. This case of MCT is the first reported multicystic presentation, the first documented fetal imaging and the smallest size in the English literature. This report shows that MCT must be considered in the differential diagnoses of fetal abdominal cysts and in multicystic pediatric pancreatic masses.

Case Series: Seventeen Year Institutional Review of Placental Chorangiomas

Abstract Introduction/Objective Chorangioma is a rare tumor occurring in less than 0.5 - 1% of all pregnancies. Small chorangiomas (&amp;lt; 4cm) are mostly asymptomatic and incidental. Large chorangiomas (&amp;gt; 4 cm) may be associated with various fetal complications and complicated pregnancies. The aim of our study is to assess the ultrasound detection rate of these lesions. Methods A retrospective study of chorangioma cases seen at Vidant Medical Center between 2003 to 2019 was conducted. Size of the lesion, detection on prenatal imaging, gestational weeks at delivery, maternal age and pregnancy related fetal and maternal complications were analyzed. Results A total of 25761 placentas were examined from 2003 to 2019 in Vidant Medical Center. Out of these only thirty-nine cases (0.15%) of chorangioma were found. 41% of these women were above 30 years of age. 36 patients were non-Hispanic. 92.3 % (36 cases) of these lesions were less than 4cm and 7.6 %(3 cases) were above 4 cm, with only one recent case of 7.0 cm in greatest dimension. 92% of the total patients presented with complicated pregnancies. Preeclampsia, preterm delivery, gestational hypertension and intrauterine growth retardation were among the most common complications observed. 3 patients presented with oligohydramnios. Interestingly, none of the lesions were detected on prenatal ultrasound. Conclusion Large chorangiomas(&amp;gt;4 cm) are rare but are known to be associated with adverse fetal outcomes. Our seventeen year institutional review shows the largest chorangioma to be 7 cm in greatest dimension. Regular ultrasound monitoring is required to pick up these benign lesions early and prevent adverse outcomes. A retrospective review is needed to determine why the lesions in our study were not detected radiographically.

Metastatic Mucoepidermoid Carcinoma to the Pleura: An Unusual Presentation

Abstract Casestudy Mucoepidermoid carcinoma is the most common malignant tumor arising in salivary glands. Predictors of poor prognosis include high-grade tumors, positive surgical margins, lymph node metastasis, or distant metastasis. The most common sites for distant metastasis include skin, lung, liver, and bone. We herein present a case of salivary gland mucoepidermoid carcinoma with pleural involvement, which is an infrequent site for metastasis. A 64-year-old female presented with worsening dyspnea and cough, which showed no improvement with antibiotics. Imaging showed a right lung nodule measuring 1.2 cm along with bilateral pleural effusion and pleural based nodularities. The dominant nodules in right and left chest measured 5.8 cm and 4.8 cm in greatest dimension, respectively. The findings were suspicious for metastatic disease. Cytological examination of pleural fluid showed reactive mesothelial cells and inflammatory cells. Subsequent biopsy of the right pleural nodule showed sheets of tumor cells with abundant eosinophilic cytoplasm and few scattered clear cells. Mucocytes were absent. The tumor cells were positive for p40 and negative for TTF-1 by immunohistochemistry. A review of patient’s medical history was significant for high-grade salivary gland mucoepidermoid carcinoma, which was resected and radiated six years prior. A comparison of both lesions showed similar morphologic features. Mucicarmine stain was positive in resection specimen but negative in pleural biopsy, confirming the absence of mucocytes. Molecular studies on both specimens showed positivity for fusion of CRTC1 (19p13) and MAML2 (11q21) loci. The overall findings were consistent with metastatic mucoepidermoid carcinoma to pleura. Conclusion Pleura is an uncommon site for metastasis from primary salivary gland mucoepidermoid carcinoma but should be part of differential diagnosis. Extensive review of patient’s past medical history plays a crucial role in correctly classifying these lesions as primary or metastatic. Differential diagnoses should include primary squamous cell carcinoma, mesothelioma, and the rare primary mucoepidermoid carcinoma of pleura.

High-Grade Uterine Sarcoma with BCOR Internal Tandem Duplication Presenting With Total Uterine Inversion

Abstract Casestudy: High-grade uterine sarcomas are rare mesenchymal tumors in which the underlying genetics of many have been recently elucidated. BCOR internal tandem duplications are present in a subset of high-grade uterine sarcomas. We report a diagnostically challenging case of a high-grade uterine sarcoma with a BCOR internal tandem duplication presenting with total uterine inversion. The case is that of a 24 year old G0P0 female with a four month history of intermittent diffuse abdominal pain and abnormal uterine bleeding. Pelvic examination revealed a mass protruding to the hymenal ring. The patient was suspected of having a prolapsed fibroid through the cervix, and a myomectomy was planned. During the procedure, total uterine inversion was noted as a result of a submucosal mass emanating from the uterine apex. The mass was removed off a broad base using sharp dissection, and the uterus was reverted in a subsequent procedure. Grossly, the mass was polypoid, rubbery, and measured 7 cm in greatest dimension. It was grey-white to red-brown, variegated, and fibrotic with focal hemorrhagic areas. Histologically, the neoplasm had variable cellularity with spindle cells and a myxoid background. There was diffuse mild to moderate cytologic atypia and areas of increased mitotic activity. Tongue-like growth was present at the interface of the tumor with the normal myometrium. The neoplastic cells showed strong immunoreactivity for cyclin D1, BCOR, and TRK. There was focal immunoreactivity for CD10, and ALK was negative. Next-generation sequencing was performed and demonstrated an insertion into the BCOR gene, consistent with a diagnosis of high- grade uterine sarcoma with BCOR internal tandem duplication. In conclusion, we report an interesting presentation of a high-grade uterine sarcoma with BCOR internal tandem duplication causing total uterine inversion. The morphologic features and immunohistochemical profile suggested the possibility of the entity, and next generation sequencing confirmed the diagnosis.

Mesenteric Cysts: A Retrospective Review. Its Not All That Simple

Abstract Introduction/Objective Mesenteric cysts are rare intra-abdominal lesions in adults. However, with the advanced imaging techniques and laparoscopic techniques, they are more often being identified and resected when clinically significant. There is a lack of detailed information in histopathology (except as case reports) since mesentery is generally neglected in our organ-based textbooks. The aim of our study is to highlight the importance of identifying and classifying mesenteric cystic lesions; they are not all that simple. Methods We performed a retrospective search on all mesenteric cysts submitted as excisions in our electronic database from 2013-2019. We classified them as per the de Perrot (PMID: 11053936) classification with modification. Results Our search showed: A. Lymphatic origin-11 (lymphangioma-10, Lymphangioma hamartomatous-1, associated with LAM-0), B. Mesothelial origin-68 (Benign mesothelial cysts-57, multilocular mesothelial cyst-11), C. Enteric origin- 3, D. Urogenital origin (Urachal cyst, mullerian inclusion cyst)-9, E. Mature cystic teratom-2, F. Pseudocyst-12, G. Epithelial cyst (not urogenital)- 11 (a/w LAMN-3, MCN-4, Mucinous cystadenoma-4), H. Associated with carcinoma-2. Case illustration: A 61-year-old male presented with worsening dysphagia, emesis and hiccups. A CT scan showed a 21.2 cm cystic mass with at least one septation (Fig 1). The cyst was resected. On gross pathological examination, the cyst measured 18 cm in greatest dimension with a thick, rough, tan-brown capsule. Microscopic examination showed a fibrous capsule, and cyst wall composed of numerous lymphatic vessels (CD31 positive) and prominent smooth muscle proliferation (Desmin positive). Scattered lymphoid aggregates were also present throughout the cyst wall. No definite epithelial lining was identified and was suspected to have been denuded. HMB-45 immunostain was negative, ruling out association with LAM. The final diagnosis of a Lymphangiomyoma, hamartomatous was rendered. Conclusion Though most of the mesenteric cysts are benign, some of them are significantly important such as Lymphangiomyoma (esp secondary to LAM), MCN, those associated with LAMN etc. and identifying and differentiating from their mimics has distinct clinical implications.

Follicle Cysts of the Ovary: A Report of 30 Cases of a Common Benign Lesion Emphasizing its Unusual Clinical and Pathologic Aspects.

The common ovarian follicle cyst is typically straightforward from both clinical and pathologic perspectives, but may have a variety of unusual features from both aspects at various stages of life. Lack of familiarity with these may lead to diagnostic quandaries, the most common of which is distinguishing between a follicle cyst and cystic granulosa cell tumor of either adult or juvenile type. We reviewed 30 cases of follicle cysts, all sent in consultation, to highlight unusual aspects of a common lesion. Patients ranged from 3 d to 47 yr old. Clinical presentations included precocious puberty, pelvic pain, or an incidentally discovered pelvic mass, including those occurring in neonates and in 2 adults with pituitary adenomas, one of which was diagnosed 3 yr after presentation with the ovarian cyst. Size ranged from 0.5 cm (deflated) to 18.5 cm, with 7 exceeding 8 cm in greatest dimension. Twelve cases demonstrated small satellite cystic follicles in the wall of the dominant cyst. The granulosa cell layer varied in thickness and mitotic activity (which ranged from 1 to 36 per 10 HPF), but uniformly displayed round nuclei that lacked nuclear grooves. Luteinization of the granulosa cell layer, theca layer, or both was seen across all clinical scenarios, with unluteinized cysts being most common in precocious puberty patients. This series documents that although typically smaller, a subset of follicle cysts are the same size as cystic granulosa cell tumors and the 2 entities may be grossly indistinguishable. Helpful clues to the diagnosis of follicle cyst are the lack of nuclear grooves (vs. adult granulosa cell tumor) and lack of invagination of granulosa cells into the cyst wall (vs. both forms of granulosa cell tumor). Mitoses in the granulosa cells are of no aid in the differential with either form of granulosa cell tumor as follicle cysts may exhibit brisk mitotic activity. Our series highlights some of the unusual clinical aspects, one relatively well known-an association with isosexual precocity, but 2 not as widely known, those occurring in neonates and those due to a pituitary adenoma, the latter sometimes not being discovered until a few years after presentation with a follicle cyst.

MON-916 Pure Large Cell Neuroendocrine Carcinoma (LCNEC) of the Gall Bladder in a Patient with Down Syndrome - a Rare Case of an Aggressive Tumor of the Gall Bladder

Background: Pure large cell neuroendocrine carcinoma of the gall bladder (LCNEC-GB) is an extremely rare entity, with only 12 such cases reported in literature to date. None has been reported in a patient with genetic disorders. We describe the case of a patient with pure LCNEC-GB in the presence of Down syndrome and prior biliary atresia.Case:A 49-year-old female with Down Syndrome and history of neonatal surgery for congenital duodenal atresia presented with fever, vague abdominal discomfort and 6 month history of 15 pound weight loss. CT abdomen revealed a 5 X 4.2 cm exophytic, heterogeneously enhancing mass in the gall bladder fossa extending into segment 4B-5 of liver with mild intrahepatic biliary dilation, along with a 9 cm cystic lesion in continuity with the duodenum which was confirmed to a dilated duodenal anastomosis (from prior biliary surgery) on endoscopy. There was high suspicion for malignancy (gall bladder carcinoma versus intrahepatic cholangiocarcinoma) and subsequent metastatic workup including tumor markers, staging CT chest, MRI of the abdomen and diagnostic laparoscopy was negative.The patient underwent robotic converted to open en bloc resection of the gallbladder mass with segment 4B-5 liver resection and adherent loops of small bowel as well as resection of the dilated duodenal anastomosis followed by reconstruction with a gastrojejunostomy and Roux-en-Y/small bowel entero-enterostomy and closure of duodenotomy.Pathology demonstrated poorly differentiated “pure” large cell neuroendocrine carcinoma (G3), 6 cm in greatest dimension, with invasion of liver, duodenum and stomach, negative liver and gastric/small bowel margins. Lymphovascular invasion (LVI) was present with no perineural invasion (PNI), and 3/15 lymph nodes involved. Staging was determined to be pT4pN1.Patient was considered for adjuvant chemotherapy based on few case reports (platinum/etoposide) but unfortunately developed systemic complaints of fevers, fatigue, pain in bones and joints 2 months post operatively. Workup revealed metastatic disease which was confirmed on biopsy. Several small satellite liver lesions were also identified which was consistent with metastatic hepatic disease. Patient and family elected to proceed with hospice. Patient died within 4 months of surgery.Conclusion:Pure LCNEC-GB is an extremely rare and aggressive tumor with a poor prognosis as seen in our patient. This is the first reported case of pure LCNEC-GB in a patient with a genetic syndrome, although it is unknown if it had any causal relationship with the tumor. Prior biliary atresia/post -surgical inflammation may have also contributed to its pathogenesis by plausible development of metaplasia and expression of neuroendocrine cells which are normally absent in the gall bladder. Our case might help shed some light into pathogenesis and genetic basis if any of this rare entity.

SUN-LB77 Incidental Anaplastic Thyroid Carcinoma: An Uncommon Entity

Background:Anaplastic thyroid cancer is an aggressive thyroid malignancy with a median survival of 3 to 9 months. It is rare and represents 2-5% of all thyroid tumors. Even more uncommonly in about 2%–6% of all ATC cases, it is identified as a small, incidental finding after surgical resection of a predominantly non-anaplastic tumor.Clinical Case:We report a case of 67 year old Caucasian male who presented with history of hoarseness of voice for one month. Fine needle aspiration biopsy of right dominant thyroid nodule revealed papillary thyroid cancer. Pre-operative imaging was negative for involvement of surrounding structures or distant metastasis. He underwent total thyroidectomy and final pathology revealed Anaplastic carcinoma arising in papillary carcinoma measuring 3.6cm in greatest dimension. Undifferentiated (Anaplastic) Carcinoma comprised approximately 5% of the tumor. Areas from anaplastic and papillary tumor were dissected separately. DNA separated from these two specimens were analyzed by PCR amplification and both were positive for BRAF mutation. External beam radiation and radioactive iodine therapy were administered after surgery. Given absence of invasion or metastasis adjuvant therapy was not initiated. His positron emission tomography, computed tomography imaging and whole-body scan has been negative for residual/ recurrent or metastatic disease. He remains disease free at 18 months after diagnosis.Discussion:Anaplastic thyroid cancer is a rare but highly aggressive tumor. In most cases it develops from a pre-existing well differentiated thyroid cancer. ATC incidence typically peaks at the 6-7th decade of life, predominantly in women. The median survival is between 3 to 9 months with less than 10% of patients alive 3 years after the time of diagnosis. Because of its aggressive behavior, the American Joint Committee on Cancer Staging Manual classifies all Anaplastic thyroid cancer Stage IV tumors. Surgery, chemotherapy and radiotherapy are the conventional therapeutic strategies performed in the attempt to improve survival.However, incidental anaplastic thyroid cancer is rare variant with very few reported cases. American Thyroid Association (ATA) Guidelines for Management of Patients with ATC do not include specific recommendations for this form of ATC. There is no consensus to define best treatment approach as to whether intrathyroidal incidentally detected ATC is best treated with surgery alone, surgery followed by radiotherapy, or surgery followed by chemotherapy plus radiation therapy.Conclusion:Based on review of our case as well as outcomes of similar reported cases, prognosis is favorable for incidental anaplastic thyroid cancer. Hopefully, with more data from similar cases to demonstrate difference in disease free survival we should be able to define the role of chemotherapy and adjuvant therapy for incidental ATC better. The question remains open, as to whether incidental anaplastic thyroid cancer should be considered as a separate entity from aggressive form of ATC.

Reconstruction of Large Post-Mastectomy Defect: a Plastic Surgeon’s Dilemma

Breast cancer is the most common cause of death due to malignancy among women worldwide. The goal of breast reconstruction post-mastectomy is to improve the quality of life whilst not increasing health risk. Reconstruction of a large-sized post-mastectomy defect with satisfactory outcome poses a challenge to the reconstructive surgeon. In advanced breast cancer, post-operative adjuvant chemotherapy and radiotherapy rates are also increasing. In these patients, plastic surgeons are presented with the substantial challenge to cover an extensive skin and soft tissue defect after mastectomy with autologous tissue. Such extensive defects are difficult to reconstruct with available flaps. The decision regarding the most appropriate reconstruction technique and whether to perform an immediate or delayed reconstruction should take into consideration the patient’s preference, risk factors and physical characteristics. We present three cases of post-radical mastectomy with large chest wall defects, > 200 sq. cm in greatest dimensions, one of which had an exposed lung, where immediate reconstruction with microvascular free antero-lateral thigh flap was done. Recipient vessels were internal mammary vessels and cephalic vein in all the cases. Post-operatively, patients did well with limited donor-site morbidity. Hence, the antero-lateral thigh (ALT) flap can be considered as a good option for cases with large defects.

T2 Safety of Early Post-operative Range of Motion in Burn Patients with Newly Placed Hand Autografts

Abstract Introduction Patients who suffer hand burns are at a high risk for developing contractures, partly due to the presence of numerous cutaneous functional units, or contracture risk areas, located within the hand. Patients who undergo split-thickness skin grafting (STSG) are often immobilized post-operatively for graft protection. Restricting mobility following a STSG is thought to protect against subdermal edema and shear forces, despite limited evidence. Early range of motion (EROM) has been described previously. Recent practice at our burn center includes EROM following hand STSG to limit unnecessary immobilization. The purpose of this retrospective study was to determine if EROM is safe to perform after hand STSG and if there is any clinical benefit. Methods In an approved, retrospective, matched case-control study of adult patients who sustained hand burns, patients who received EROM were defined as cases; patients who did not receive EROM were considered controls and received the standard 3–5 days of post-operative immobilization in a resting hand splint. Adult patients admitted over a 3-year period were eligible for inclusion. Patients were evaluated for graft loss and range of motion. Results Seventy-two patients were included in this study; 37 EROM patients and 35 matched controls. EROM patients tended to have a larger area excised (170.4 ± 69.8cm2 vs. 132.9 ± 76.2cm2; p=0.034) and grafted (171 ± 70.8 cm2 vs. 132.9 ± 76.2 cm2; p=0.033). Most patients were male, with an average age of 39 years. Patients had an average of approximately 5% TBSA burns with 1.5% to the hands. On post-op day (POD) 1 and 2, patients received EROM for an average of 30 minutes (29.25 ± 14.9 vs. 31 ± 16.4 minutes). Six patients experienced minor graft loss. Three patients (8%) experienced graft loss not attributable to EROM. One patient (2.7%) experienced graft loss pre-EROM on POD2 and 3 patients (8%) experienced graft loss post-EROM on either POD1 or POD2. All graft loss was less than 1 cm in greatest dimension and no patient who experienced graft loss required additional surgery as they all closed by their first outpatient follow-up. Significantly more patients who received EROM achieved full digital flexion by the first outpatient visit (25/27=92.6% vs. 15/22=68.2%; p=0.028). Conclusions Performing EROM does not cause an increase in graft loss. All areas of graft loss from the EROM group healed without intervention. There appears to be a benefit to EROM since there was a significant improvement in the patients’ ability to make a full fist at initial outpatient follow up. Further prospective analysis is needed to examine the true clinical utility of EROM in the hand and other contracture-prone areas. Applicability of Research to Practice Clinical change in post-operative management after hand grafting.

A Rare Case of Rapidly Enlarging Myelolipoma in Sickle Cell Disease

Adrenal myelolipoma (AM) is a benign tumor composed of mature fat cells and hemopoietic elements. Most AMs are incidental findings on imaging and clinically asymptomatic. The purpose of this case report is to describe a rare case of AM and explore its clinical manifestations, imaging features, and treatment. In this study, we report a case of a rapidly growing right AM in a patient with uncontrolled hemoglobin sickle cell disease. A 38-year-old male presented to our institution's endocrine surgery clinic for evaluation of an enlarging right adrenal mass. This mass was incidentally found during an abdominal ultrasound performed for transaminitis and thrombocytopenia. Patient was asymptomatic without any abdominal discomfort, back pain, nausea, or vomiting. Patient was lost to follow up until 2018. Follow-up computed tomography scan in 2018 showed the right adrenal mass measuring 12.3 cm in greatest dimension with significant macroscopic fat. Given the imaging features, AM was the presumed diagnosis. However, with a medical history of uncontrolled sickle cell disease, extra-medullary hematopoiesis and rapidly growing liposarcoma could not be ruled out. Surgical excision was performed due to size and significant tumor growth. Diagnosis was confirmed with histopathology and revealed myelolipoma. Image characteristics can be helpful in diagnosis of AM; however, the appearance of this lesion on computed tomography can be similar to other adrenal gland pathologies such as liposarcoma and mass-forming extramedullary hematopoiesis. Percutaneous needle biopsy may be indicated if the diagnosis remains unclear.