Hepatic Langerhans Cell Histiocytosis (LCH) Mimicking Antimitochondrial Antibody (AMA)–Negative Primary Biliary Cholangitis (PBC), Presenting as a Harbinger of Multisystem LCH

2019 ◽  
Vol 152 (Supplement_1) ◽  
pp. S59-S59
Author(s):  
Hua Li ◽  
Peter Ells ◽  
Mustafa Erdem Arslan ◽  
Karl Robstad ◽  
Hwajeong Lee
Keyword(s):  
Liver Biopsy ◽  
Exceptional Case ◽  
Prior History ◽  
Primary Biliary Cholangitis ◽  
Elevated Alkaline Phosphatase ◽  
Indirect Bilirubin ◽  
Hepatic Involvement ◽  
Normal Limits ◽  
Eosinophilic Cytoplasm ◽  
The Right

Abstract Objectives LCH is a rare systemic disorder characterized by an infiltration of CD1a+/Langerin+ histiocytes. LCH commonly involves bone, skin, and lymph nodes in children. Hepatic involvement is rarely observed in multisystem LCH. We describe an exceptional case of hepatic LCH in an adult, mimicking AMA-negative PBC, preceding the diagnosis of multisystem LCH. Methods A 65-year-old man presented with intermittent pruritus, weakness, dyspnea, fever, and chills that have been progressive for 4 years. Physical examination was unremarkable. Laboratory work revealed elevated alkaline phosphatase (471 U/L; ref 40-120 U/L), GGT (271 U/L; ref 0-41 U/L), total bilirubin (2.0 mg/dL; ref 0.0-1.2 mg/dL), and direct bilirubin (1.1 mg/dL; ref 0.0-0.3 mg/dL). Indirect bilirubin, AST, and ALT were within normal limits. Liver biopsy was performed. Results Liver biopsy showed lobular and portal nonnecrotizing granulomas with one “florid duct lesion” with duct injury, suggestive of PBC. Tests for autoimmune diseases including AMA were negative. ERCP was negative for biliary obstruction. One month after the liver biopsy, he developed flaking, red, and burning rash on the right scalp, forehead, and epigastric skin. A skin biopsy at an outside institution revealed dermal and epidermal infiltration of CD1a-positive histiocytes with indented nuclei and pale eosinophilic cytoplasm, consistent with LCH. Subsequent reexamination of the liver biopsy showed that the histiocytes within the florid duct lesion were positive for CD1a and S-100, while the remaining granulomas were negative. The following day, a small focus of LCH was noted in his gastric biopsy performed for gastritis symptoms. Conclusion Hepatic LCH may mimic AMA-negative PBC histologically and clinically, and may present as a harbinger of multisystem LCH. While rendering the diagnosis would be challenging without prior history of LCH and with focal involvement, awareness of such presentation and communication with clinical colleagues may be helpful.

scholarly journals Gastrointestinal stromal tumor resulting in recurrent colic in a arabian horse gelding a report of case

2020 ◽  
Vol 4 (1) ◽  
pp. 048-050
Author(s):  
Briceño Abelardo Morales ◽  
Oliva Harmon
Keyword(s):  
Gastrointestinal Stromal Tumor ◽  
Body Condition Score ◽  
Vital Signs ◽  
Abdominal Ultrasound ◽  
Stromal Tumor ◽  
Normal Limits ◽  
Eosinophilic Cytoplasm ◽  
Arabian Horse ◽  
The Right ◽  
Adjacent Segments

Background: A Grey 12-year-old Arabian endurance horse gelding was referred to the SHS Veterinary Center for anorexia, mild colic of 5 days duration, and melena of 1 day duration. The owner reported recurring colic, 12 episodes of mild colic in the previous year. Methods: On admission, vital signs were within normal limits and body condition score was estimated to be 3/9. Results: Packed cell volume (PCV) was 28% [reference range (RR): 31% to 47%] and plasma total protein was 58 g/L (RR: 60 to 80 g/L). Hematochezia was observed. Abdominal ultrasound examination detected no abnormalities. Over the next 12 h, the horse experienced hematochezia and several mild episodes of colic and death. A necropsy was performed. A mass arising from the right dorsal ascending colon near the base of the cecum and extending transmurally from the colonic mucosa into the mesocolon was a 8 cm × 5 cm × 8 cm firm, homogenous, tan mass. The portion of the mass that extended into the colonic lumen was pedunculated, with an ulcerated surface. The adjacent segments of colon were markedly reddened and edematous. Histologically, the mass was comprised of large interweaving sheets of small, spindle cells with ill-defined cell borders embedded in abundant myxomatous matrix. Tumor cells contained scant eosinophilic cytoplasm and oval to elongate nuclei with finely stippled chromatin and inconspicuous nucleoli. Mitotic figures were rare (1/10) high power fields. Tumor infiltrated between the muscularis interna and the muscularis externa at the myenteric plexi. Conclusion: Gross and histologic appearance, were consistent with a diagnosis of gastrointestinal stromal tumor.

scholarly journals Neonatal cardiogenic shock revealing obstructive cardiac Hibernoma: case report

2021 ◽  
Vol 16 (1) ◽  
Author(s):  
Rym Gribaa ◽  
Marwen Kacem ◽  
Sami Ouannes ◽  
Wiem Majdoub ◽  
Houssem Thabet ◽  
...  
Keyword(s):  
Cardiogenic Shock ◽  
Transthoracic Echocardiography ◽  
Right Atrium ◽  
Benign Tumors ◽  
Histopathological Analysis ◽  
Tricuspid Valve Repair ◽  
Flow Measurements ◽  
Surgical Specimen ◽  
Eosinophilic Cytoplasm ◽  
The Right

Abstract Background Cardiac Hibernomas are very rare benign tumors and usually remain asymptomatic. Neonatal cardiogenic shock due to cardiac tumors is extremely very rare. Until this date a few cases of cardiac hibernoma have been reported in the literature. Transthoracic echocardiography help in the differential diagnosis, but the definitive diagnosis is histological. The management strategy is not clearly codified. The Aim is to report and discuss the clinical features of a cardiac Hibernoma and review the relevant literature. Case presentation We describe a case of a 2-day-old Caucasian full-term male neonate admitted in neonate intensive care with cardiogenic shock, having fluid resuscitation and inotropic drugs. Ventilatory support was started immediately with the subsequent reestablishment of normal blood pressure. Then he was transferred to the echocardiography laboratory. Transthoracic echocardiography showed two echogenic masses in the right atrium and right ventricle. The masses were extended to the pulmonary trunk. Pulmonary artery flow measurements showed the presence of pulmonary and tricuspid obstruction. Surgery was rapidly considered since the baby was hemodynamically unstable. Intraoperative evaluation showed a mass embedded in the interventricular septum that occupy the right ventricular cavity and the right atrium. The tumor involved also the chordae of the tricuspid. Partial resection was done. Tricuspid valve repair was performed by construction of new chordae from the autologous pericardium. The specimen was sent for histopathological analysis. The baby died immediately after surgery. Histological examination of the surgical specimen revealed clear multivacuolated cells filled with lipid droplets and granular intense eosinophilic cytoplasm which confirms the diagnosis of Hibernoma. Conclusion Cardiac Hibernomas are rare benign tumors. The prognosis and treatment strategy is closely dependent on the location, initial clinical presentation and possible complications. The prognosis can be unfavorable if the tumor was obstructive and infiltrate the myocardium.

scholarly journals Cervical Sympathetic Trunk-Centered Inflammatory Myofibroblastic Tumor Complicated with Postoperative First Bite Syndrome

2021 ◽  
Author(s):  
Liu Yang ◽  
Wen Li
Keyword(s):  
Neck Region ◽  
Sympathetic Trunk ◽  
Cervical Sympathetic Trunk ◽  
First Line Therapy ◽  
Normal Limits ◽  
Serum Igg4 ◽  
Postoperative Serum ◽  
Cervical Sympathetic ◽  
The Right ◽  
First Bite Syndrome

AbstractInflammatory myofibroblastic tumors (IMTs) in the head and neck region are common, but those with sympathetic trunk involvement are extremely rare. Here we present a case of cervical sympathetic trunk-centered IMT which is also accompanied by ipsilateral carotid artery, internal jugular vein, and vagus nerve involvement. The patient initially complained of an episodic painful swelling on the right side of the neck and underwent surgery. Preoperative and postoperative serum IgG4 level during 3-year follow-up time is within normal limits. Immunohistochemical study of the tumor has also revealed negativity to IgG4. Postoperative first bite syndrome (FBS) was observed. Surgery seems to be first-line therapy in the patient with IgG4-negative IMT.

scholarly journals An Exceptional Case of Intraparotid Plexiform Neurofibroma Originating from Autonomic Fibers of the Auriculotemporal Nerve

2017 ◽  
Vol 2017 ◽  
pp. 1-5 ◽  
Author(s):  
Sarantis Blioskas ◽  
Sotiris Sotiriou ◽  
Katerina Rizou ◽  
Triantafyllia Koletsa ◽  
Petros Karkos ◽  
...  
Keyword(s):  
Parotid Gland ◽  
Plexiform Neurofibroma ◽  
Surgical Excision ◽  
Neurofibromatosis Type ◽  
Exceptional Case ◽  
Benign Tumors ◽  
First Case ◽  
Superficial Lobe ◽  
The Right ◽  
Auriculotemporal Nerve

Plexiform neurofibromas are benign tumors that tend to occur in patients suffering from neurofibromatosis type 1 (NF-1). This report addresses a rare case where the tumor affected the parotid gland, deriving almost exclusively from the peripheral portion of the facial nerve. A 6-year-old male was referred to us complaining about a gradually enlarging swelling over the right parotid area. Imaging localized the lesion to the superficial lobe of the parotid gland, suggesting a neurofibroma. Cosmetic disfigurement and a functional deficit led us to perform complete surgical resection. Meticulous surgical dissection as well as auriculotemporal nerve origin made complete extirpation possible with almost zero morbidity and ensured alleviation of both aesthetic impairment and pain. This is the first case of an intraparotid PN in a pediatric NF-1 patient, which originated from branches of the auriculotemporal nerve and particularly from fibers of the autonomic nervous system. Radical surgical excision was decided according to established decision-making algorithms.

scholarly journals Recovery from Transient Global Amnesia Following Restoration of Hippocampal and Fronto–Cingulate Perfusion

2010 ◽  
Vol 22 (3-4) ◽  
pp. 131-139 ◽  
Author(s):  
Paolo Caffarra ◽  
Letizia Concari ◽  
Simona Gardini ◽  
Sabrina Spaggiari ◽  
Francesca Dieci ◽  
...  
Keyword(s):  
Acute Phase ◽  
Neuropsychological Testing ◽  
Transient Global Amnesia ◽  
Normal Limits ◽  
Global Amnesia ◽  
One Year ◽  
The Right ◽  
Rcbf Spect ◽  
Subject Comparison

A patient who suffered a transient global amnesia (TGA) attack underwent regional cerebral blood flow (rCBF) SPECT imaging and neuropsychological testing in the acute phase, after one month and after one year. Neuropsychological testing in the acute phase showed a pattern of anterograde and retrograde amnesia, whereas memory was within age normal limits at follow up. SPECT data were analysed with a within subject comparison and also compared with those of a group of healthy controls. Within subject comparison between the one month follow up and the acute phase detected increases in rCBF in the hippocampus bilaterally; further rCBF increases in the right hippocampus were detected after one year. Compared to controls, significant hypoperfusion was found in the right precentral, cingulate and medial frontal gyri in the acute phase; after one month significant hypoperfusion was detected in the right precentral and cingulate gyri and the left postcentral gyrus; after one year no significant hypoperfusion appeared. The restoration of memory was paralleled by rCBF increases in the hippocampus and fronto-limbic-parietal cortex; after one year neither significant rCBF differences nor cognitive deficits were detectable. In conclusion, these data indicate that TGA had no long lasting cognitive and neural alterations in this patient.

1223 Cholelithiasis and Choledocholithiasis with Migration of Stones into Right Psoas Muscle Cavity

2021 ◽  
Vol 108 (Supplement_6) ◽  
Author(s):  
J Hunter
Keyword(s):  
Bile Duct ◽  
Common Bile Duct ◽  
Psoas Muscle ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Imaging Modalities ◽  
Prior History ◽  
Clinical Presentations ◽  
Multiple Imaging ◽  
The Right ◽  
Psoas Major

Abstract Aim To highlight the importance of appropriate imaging modalities for abnormal clinical presentations. Method Xyo woman presented with severe right upper-quadrant pain to the emergency department. Prior history of colicky abdominal pain for 1 year, right-sided nephrectomy and hypertension. She stated that the current epsiode of pain was the worst so far, becoming more persistent, prompting her to call an ambulance. Cholecystitis was suspected, so a Computed Tomography abdomen/pelvis was performed. A dilated, inflamed gallbladder was noted in contact with the right psoas major, with what appeared to be an abscess in the right psoas major. Results A Magnetic Resonance Cholangiopancreatography was performed, which demonstrated that the aforementioned abscess was in fact a large cavity within the psoas major, containing up to 15 gallstones. Cholelithiasis was also seen, with common bile duct dilatation and evidence of a common bile duct stone. An Endoscopic Retrograde Cholangiopancreatography was performed, and X recovered well post-procedure with analgesia and antibiotics. Conclusions Acute cholecystitis/intra-abdominal gallstones may result in abscess/cavity formations and abnormal biliary clinical presentations. It is important therefore to fully investigate abnormal hepatobiliary imaging with multiple imaging modalities to allow for accurate diagnosis and appropriate further management of presentations.

Extraovarian primary seromucinous cystadenoma masquerading as mesenteric cyst

2021 ◽  
Vol 14 (5) ◽  
pp. e240414
Author(s):  
Kishor R J ◽  
Bhuvaneshwari Harikrishnan ◽  
Naveen Alexander ◽  
Veena Bheeman
Keyword(s):  
Lower Abdominal Pain ◽  
Interesting Case ◽  
Mesenteric Cyst ◽  
Lumbar Region ◽  
Normal Limits ◽  
Mesenteric Border ◽  
Contrast Enhanced ◽  
Laparoscopic Excision ◽  
Abdominal Examination ◽  
The Right

We report an interesting case of extraovarian, primary seromucinous cystadenoma, which presented as a mesenteric cyst. A 31-year-old woman came with complaints of lower abdominal pain for 2 years, which is intermittent and aggravated during the menstrual cycle. On examination, her vitals were within normal limits; per abdominal examination revealed 7×7 cm mass in the right lumbar and right hypochondrium with well-defined margins. A Contrast-enhanced whole abdomen done showed a thin-walled of cyst of size 7×8×9 cm in the right lumbar region abutting and replacing the mesenteric border of ascending colon suggesting of mesenteric cyst. Patient underwent laparoscopic excision of mesenteric cyst. Histopathology revealed seromucinous cystadenoma without ovarian stroma.

Cytokeratin 7 and Copper Stains Facilitate Diagnosis of Small Duct Primary Sclerosing Cholangitis

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S125-S125
Author(s):  
A Wilhelm ◽  
H L Stevenson ◽  
K Kline
Keyword(s):  
Liver Biopsy ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Early Recognition ◽  
Clinical Suspicion ◽  
Cytokeratin 7 ◽  
Ductular Reaction ◽  
Elevated Alkaline Phosphatase ◽  
Pattern Of Injury ◽  
Small Duct

Abstract Introduction/Objective Classic primary sclerosing cholangitis (PSC) involves extrahepatic and/or intrahepatic biliary ducts with segmental biliary strictures and dilatations that often allow the diagnosis to be made via cholangiogram. Small duct PSC (sdPSC) is a rare subtype that presents similarly with a cholestatic pattern of injury, yet due to the small size of involved ducts, a cholangiogram is non-diagnostic and diagnosis is dependent on clinical suspicion and liver biopsy. The histopathological features of sdPSC are often subtle and may easily be overlooked. Diagnosis of this entity- though difficult- is important, as early recognition can facilitate the identification of associated disease processes and life-threatening complications. Methods/Case Report We encountered a 33-year-old female presenting with intermittent pruritis, episodes of jaundice, and persistently elevated alkaline phosphatase who was misdiagnosed with only fatty liver at an outside institution. Evaluation with MRCP showed no abnormalities within the biliary tract and a liver biopsy was performed to aid in the diagnosis. The H&E and trichrome findings of atrophic bile ducts and some peribiliary sclerosis were extremely subtle and may have been overlooked without clinical suspicion. Cytokeratin 7 (CK7) highlighted cholangiolar metaplasia in hepatocytes and the bile ductular reaction that occurs in cholestatic disease states. A Rhodamine copper stain showed periportal deposition suggestive of chronic biliary obstruction. Use of CK7 and copper stains supported the presence of chronic biliary injury and suboptimal bile flow, confirming the diagnosis of sdPSC. Results (if a Case Study enter NA) NA Conclusion Diagnosis of sdPSC has historically relied on H&E and trichrome stains. In this case, the findings on H&E and trichrome stains were non-diagnostic, while the use of CK7 and copper stains confirmed the diagnosis of sdPSC. We recommend using CK7 and copper stains to evaluate for sdPSC.

scholarly journals Giant adrenal hemangioma: Unusual cause of huge abdominal mass

2015 ◽  
Vol 9 (11-12) ◽  
pp. 834 ◽  
Author(s):  
Mohamed Tarchouli ◽  
Adil Boudhas ◽  
Moulay Brahim Ratbi ◽  
Mohamed Essarghini ◽  
Noureddine Njoumi ◽  
...  
Keyword(s):  
Adrenal Gland ◽  
Abdominal Mass ◽  
Abdominal Distension ◽  
Microcytic Anemia ◽  
Hypochromic Microcytic Anemia ◽  
Normal Limits ◽  
Laboratory Examinations ◽  
Right Lobe ◽  
The Right

Adrenal hemangioma is an extremely rare benign and non-functioning neoplasm of the adrenal gland. We report a case of a 71-year-old woman admitted for intermittent abdominal pain and abdominal distension associated with vomiting and chronic constipation for 5 years. Physical examination revealed a large abdominal mass. Both computed tomography scan and magnetic resonance imaging suggested hemangioma in the right lobe of the liver. Laboratory examinations and tumour markers were within normal limits, except for hypochromic microcytic anemia. The mass was removed intact by conventional surgery and histopathology revealed a cavernous hemangioma of the adrenal gland with no signs of malignancy. Surgical resection was curative, with no recurrence at the 2-year follow-up.

scholarly journals Malignant Glomus Tumour: A Case Report and Review of the Literature

Sarcoma ◽  
2003 ◽  
Vol 7 (2) ◽  
pp. 87-91 ◽  
Author(s):  
Annarosaria De Chiara ◽  
Gaetano Apice ◽  
Stefano Mori ◽  
Giustino Silvestro ◽  
Simona N. Losito ◽  
...  
Keyword(s):  
Clinical Course ◽  
Smooth Muscle Actin ◽  
Tumour Cells ◽  
Small Cells ◽  
Glomus Tumour ◽  
Recurrent Tumour ◽  
Lymphnode Metastasis ◽  
Histological Criteria ◽  
Eosinophilic Cytoplasm ◽  
The Right

Purpose:Glomus tumours are characteristically benign solitary tumours. At our knowledge, about 23 reports are present in literature regarding the malignant counterpart, but only a minority developed metastases. We describe a locally aggressive glomus tumour with lymphnode metastasis.Patient:The patient was a 40 year-old man presenting a 1.5-cm lesion on the right wrist incompletely excised and a recurrent tumour, 4 × 2 cm in size, removed after 9 months, for which he received radiotherapy. After 2 years he developed an axillary lymphnode metastasis.Results:Histologically, both tumours (primary and metastasis) were similar. There were sheets and nests of uniform small cells with scant eosinophilic cytoplasm and round to polygonal nuclei; there was some degree of pleomorphism and the mitotic index was high (up to 18 m/10 HPF). The tumour cells were positive for vimentin and smooth muscle actin, but negative for desmin, NSE, Factor VIII, chromogranin, cytokeratin. Remarkably, in the primary, the cells strongly expressed p53 (70%) and MIB-1 (35%).Discussions:In many reported malignant cases, the histology of the tumour cells suggested that they were malignant, yet the clinical course has been benign. Carefully reviewing the literature, it seems that actually we have enough histological criteria to identify the cases with biological adverse outcome. Those unfortunate cases behave as high grade sarcomas and therefore may deserve an aggressive therapeutic treatment.

Sign in / Sign up

Export Citation Format

Share Document