An 18-year-old Salernitano stallion was presented for enlargement of the left testicle with no other clinical signs. Sonoelastographic examination showed parenchymal changes with deformation of the normal testis, appearing with an irregular profile. Unilateral orchiectomy was performed. Considering gross, microscopic and immunohistochemical findings, a definitive diagnosis of diffuse seminoma was made. Three months later, follow-up assessment showed no evidence of recurrence, preserved reproductive abilities or fertility, but did show reduced testosterone levels.An 18-year-old Salernitano stallion developed a progressive enlargement of the left testicle over eight months. An ultrasound evaluation was performed, along with a hormonal profile. A histopathological evaluation of the testis was performed after unilateral orchiectomy. On B-mode ultrasound examination, testicular parenchyma was characterized by the loss of internal structure, with the presence of multiple coalescing, nodular, well-defined and heterogeneous lesions with capsule deformity, appearing with an irregular profile. On dissection, the testicular parenchyma bulged over the cut section, confirming the increase in size. Microscopically, the lesion consisted mainly of large, densely packed, polygonal-to-round-shaped neoplastic cells. Mitotic figures were plentiful and frequently atypical; further microscopic features included apoptosis and necrosis. At immunohistochemistry, the entire neoplasm showed strong and diffuse immunolabeling for vimentin, while CD117-specific immunoreactivity was only observed in scattered clusters of neoplastic cells. Based on the gross, microscopic and IHC findings, a diagnosis of diffuse seminoma was made. Three months later, a follow-up examination showed no evidence of recurrence and the preservation of reproductive abilities. The case presented shows an unusual ultrasonographic pattern for seminoma and the basis of the correlation between the characteristics of the sonoelastographic examination and histological diagnosis. Seminomas are reported to be the most common testicular neoplasm in the equine stallion ,2, as weThis type of germinal tumor affects horses of 11–22 years , but it Although the literature includes many cases of seminoma affecting cryptorchid horses ,10,11,12Seminomas can be single, multiple, unilateral, bilateral, or cystic ,11, but In contrast to other species, equine seminomas are frequently malignant, metastasizing to the abdominal organs, including the liver ,16 and kUltrasonography (US) is often used as a diagnostic tool in cases of testicular neoplasia, whose appearance ranges from circumscribed small nodules to large, complex masses with disruption of normal testicular anatomy; of note, different tumors cannot be distinguished based on US findings ,13,14,15Next to B-mode findings, it is also possible to obtain information regarding the structural tissue abnormalities in a non-invasive way through a US-based technique called sonoelastography. This technique, indeed, is able to detect different levels of tissue elasticity based on the underlying disease that causes tissues abnormalities.US elastography includes strain elastography (SE) and 2D shear-wave elastography (2D-SWE).With SE, tissue elasticity is measured under the strain generated by external compression using an ultrasound transducer or internal physiologic pulsation such as heart beats or respiration; the acquired tissue elasticity data are encoded as color-mapping  superimposed on the B-mode images and show the related elasticity of the anatomic lesion, with the possibility to compare the target tissue with a reference one through a semiquantitative analysis .Two-dimensional SWE induces a shear wave in multifocal zones and measures the shear waves (SWs) that propagate at several locations around the acoustic pulse through a detection pulse. Subsequently, SWs inside the field of view (FOV) are integrated, and the distribution of SWs is displayed through a color map showing areas of high stiffness as red and low stiffness as blue.The average SW is also quantified as Young’s modulus in kilopascals (kPa) and shear wave velocity measured in m/s by placing the ROI within the FOV .In human medicine, sonoelastography, a non-invasive ultrasound-based technique to assess tissue stiffness, is highly sensitive to detect the difference in stiffness in healthy versus diseased testicles and has shown some promise in veterinary medicine; in particular, different levels have been observed of stiffness in the case of inflammatory and neoplastic disorders, with the highest stiffness observed in cases of malignant lesions ,20. ThisIn this work, we present the clinical case of an unusual unilateral seminoma in a Salernitano stallion, with a specific focus of the B-mode features of this neoplasia followed by the elastographic findings, with the aim to introduce the application of this technique in cases of reproductive disorders in stallions in order to potentially reduce, in the future, the need for invasive investigations to obtain the final diagnosis.An 18-year-old Salernitano stallion, an endangered horse breed approved for reproduction, was referred to the Veterinary Hospital for a progressive enlargement of the left testicle, in the past eight months, with no other clinical signs.Reproductive anamnesis reported normal sexual behavior and fertility, confirmed by a spermiogram performed during the previous reproductive season.A general physical examination was performed, along with a focused examination of external genitalia, and basic hematological and serum biochemical tests, as a routine screening prior to any sedation or anesthesia. Trans rectal palpation and ultrasonographic examination  were also performed, along with trans abdominal ultrasound examination (5 mHz convex probe). A mild sedation with 0.05 mg/kg of acepromazine  was necessary to allow the procedures to be carried out safely.B-mode ultrasound examination was performed with a Logiq S8 sonographic system  and 3–5 mHz convex probe  on both testicles, in order to assess size, echogenicity, echotexture and borders; vascularization was assessed with color Doppler and B-Flow software with the same sonographic system.Sonoelastography was performed with an 8.5–10 mHz linear probe using SE and 2D SWE software in the same sonographic system ; in particular, since it was not possible to include the whole testicle in a single image field of view within the elastographic examination with the linear probe, each testicle was scanned including as much parenchyma as possible in the longitudinal plane and in the transverse plane, dividing the parenchyma into cranial, medial and caudal sections for the transverse plane.Both SE and 2D-SWE were conducted by adapting the existing scanning protocol used for humans ,26.SE was conducted as follows: each section was scanned in order to acquire a 4–5 s loop, from which several frames representing the positive or negative peaks of the strain graph with the color of elastography images unchanged were collected, with the color bar indicative of the image quality appearing as green.Qualitative evaluation of tissue stiffness was performed using a color scale adapted from the current human literature, in which normal parenchyma appears with a central parenchymal green color with blue edges surrounded by red bands. Testicular lesions, characterized by parenchymal abnormalities visible with B-mode US and resulting in a different elastogram appearance than the normal tissue, were classified following the classification explained in This scale is based on the basic elastography principle that the more rigid the tissue is, the higher the rate of malignancy is present, based on the positive correlation observed between stiff lesions and the rate of malignancy diagnosed with histopathology .Two-dimensional SWE was then conducted, acquiring a cineloop of 20 s on each section, and then 5 elastograms were selected for each part, and three regions of interest (ROIs) of 5 mm were placed on the elastograms without overlapping on each to collect measurements in m/s .Since specific elastography reference parameters are available for the equine species, the normality was assessed based on what was evidenced from the healthy testis.Unilateral orchiectomy was performed under general anesthesia and allowed the complete removal of the affected testicle according the procedure described as follows.The patient was prepared for a dorsally recumbent, open inguinal orchidectomy, under general anesthesia. Therefore, the patient was premedicated with medetomidine (7 μg/kg i.v.), and general anesthesia was obtained with an injection of ketamine (2.2 mg/kg i.v.) and diazepam (0.02 mg/kg i.v.) and maintained with isoflurane in oxygen-enriched air and CRI of medetomidine (3.5 μg/kg i.v.).The surgical approach was inguinal. Skin was incised conveniently to identify the vaginalis comunis, which was sharply opened to grasp and withdraw the testis. The ligamentum caudae epididimi was divided, and the funiculus spermaticus sealed and divided with the LigaSure TM system .Then, the testis was removed and the divided tissues surgically reconstructed lege artis.Since no signs of regional lymphadenopathy were detected either clinically or sonographically, lymphadenectomy was not performed. To determine whether the testicular neoplasm had altered the normal hormonal profile of the stallion, blood samples for the evaluation of testosterone and estradiol serum concentrations were collected before the surgery (T0) and repeated three (T1) and five days (T2) after the removal of the pathological testis. Hormone detection was performed by chemiluminescence.To determine the effects of the unilateral orchiectomy on stallion fertility, three months after surgery, during a follow up examination, a spermiogram was performed and compared with spermiograms obtained in the previous breeding season.Tissue samples, representative of several areas of the testicle, were promptly fixed in 10% neutral buffered formalin, embedded in paraffin and routinely processed for histopathological investigations (hematoxylin and eosin stain). Moreover, tissue sections were tested by immunohistochemistry (IHC) for vimentin , cytokeratins  and CD117 . To this aim, tissue sections were pre-treated with H2O2 (0.5% in methanol) for 20 min to quench endogenous peroxidase activity. Antigen retrieval was performed by heat treatment, at 96 °C for 45 min, in 0.01 M citrate buffer (pH 6). Primary antibodies were incubated overnight at 4 °C, and the immune reactions were visualized by means of a streptavidin peroxidase technique  using 3,3′ diaminobenzidine tetrahydrochloride as a chromogen. Suitable positive controls were included in each run, and negative controls were obtained by omitting the primary antibodies.p < 0.05.As far as it regards the data obtained from elastography, statistical analysis was performed on the data expressed in m/s with MATLAB R2015a software; in particular, the distribution of data was analyzed using the Shapiro–Wilk test, and the comparison between the two testicles was performed with Wilcoxon signed-rank test with statistical significance defined as an alpha level of Physical examination was normal for the species and breed .Clinical evaluation, trans rectal palpation and trans rectal and abdominal ultrasonography did not show any alteration in the explorable lymph nodes and organs; the size of the left enlarged testicle, detected with the caliper, was 15.8 cm CrCd × 8.7 cm DV × 11 cm LL ; the rigPalpation of the testis showed a considerable parenchyma inhomogeneity, losing its normal turgid resilient consistency. In fact, the testicle appeared to consist of harder areas and areas of decreased consistency, separated by harder septa, of a fibrous-like consistency. The evaluation did not cause pain, and the normal mobility of the testis with no scrotal adhesions was assessed.A complete physical examination showed no evidence of metastases.Hematological and serum biochemical tests showed no alterations.Comparison of spermiogram results is shown in The ultrasound examination showed that the right testicle appeared normal , while tRegarding SE results, since it was technically not possible to include the whole testicle in one single examination, the color-coded classification was performed considering the most prevalent pattern within the image.The right testicle was characterized using central parenchymal red-green color with blue edges and without red bands A,B, whilp < 0.0028) than the contralateral, with a median value for the left testicle of 3.66 m/s (1.8–6.64), and median values for the right testicle were 1.15 m/s (0.8–1.6)  A–D.Based on the ultrasonographic findings, a neoplasm was suspected.Surgery was performed without complications, and all the pathological testicular tissue was removed by surgical ligation on the spermatic cord that appeared normal, reducing the possibility of the dissemination of neoplastic cells.The hormonal profile showed a significant reduction in testosterone levels after surgical removal, showing values of about half, while estradiol assays showed only a slight reduction. Values are shown in At dissection, the parenchyma bulged markedly when the testis was incised, thus confirming its increase in size. Macroscopically, the testicular tissue was completely disrupted, with the normal appearance of the parenchyma being effaced .Microscopically, the lesion mainly consisted of large, densely packed, polygonal-to-round shaped neoplastic cells. In some areas, such cells were organized as cords, small clusters or tubular-like structures, surrounded by loose connective tissue. Anisocytosis and anisokaryosis were always prominent. Most of neoplastic cells had a single nucleus, although bi- or multinucleate cells were often detected. Mitotic figures were plentiful and frequently atypical (1–3 mitoses/hpf). Lymphocytes were numerous, and frequently organized as large aggregates. Apoptosis and necrosis represented further microscopical features.At IHC, neoplastic cells proved to be negative for cytokeratins, but they showed a strong cytoplasmic immunoreactivity for vimentin. Clusters of cells were also positive for CD117, with a cytoplasmic and/or a membranous pattern of immunoreactivity.Considering gross, microscopic and IHC findings, a definitive diagnosis of diffuse seminoma was made .Three months after surgery, a follow-up evaluation showed no other changes in the right testicle. Hematology and serum-biochemical panels were repeated, with normal results. A complete spermiogram was performed to assess fertility, showing results comparable to those obtained prior to the onset of neoplasm. A comparison of spermiogram results is shown in Seminomas are the most common testicular neoplasms in horses , althougThe age of appearance of seminoma can range from 7  to 22 yeEquine seminoma can show a malignant course , but cliThis report describes an 18-year-old stallion, presenting a progressive enlargement of the left descended testis, with no other clinical signs. Such a nonspecific clinical presentation is clearly not suggestive of seminoma, despite the consulted literature reporting cases of horses affected by seminomas, in the absence of any clinical signs, except weight loss . AdditioThe clinical finding of an enlarged non-painful testis should be differentiated from other non-painful lesions, such as hydrocele and hematocele , or otheLittle information is available about the US features of equine seminoma, which has been described as a mass with variable echogenicity or diffusely hypoechoic with ill-defined regions of hyperechogenicity .In our clinical case, compared to what has been previously described, the affected parenchyma appeared markedly nodular, rather than characterized by ill-defined heterogeneous areas.For sonoelastography, both SE and 2D-SWE evidenced a difference in stiffness between the two testicles, with both qualitative and quantitative evaluations, since with SE, a different color distribution was evident comparing the diseased and the healthy one, with a similar trend quantitatively expressed by the measurements in m/s.The data described herein are consistent with what is currently observed in human medicine, where elastography is highly sensitive for use in non-invasively detecting focal lesions in the testicular parenchyma , especiaThe main difference and limitation of the elastography application (both SE and 2D-SWE) in the case described herein is the impossibility of including all testicular parenchyma in one examination.This might have partially influenced the final results, especially for the variable SE color pattern in the neoplastic tumor, since this technique is based on the manual compression exercised by the operator; the different scan planes, combined with having scanned different sections of the parenchyma might have caused a difference in the mechanical pressure distribution, which might explain the non-homogeneous color pattern in different testicle sections.This variability could also be due to the presence of necrosis next to the neoplastic tissue, thus causing a heterogeneous color pattern indicating different grades of stiffness within the same image.Particularly for SE, the choice to consider only the qualitative evaluation on the elastogram, instead of also including the semiquantitative analysis, was made because in human medicine, SE is typically performed by comparing the focal lesion with the background normal testicle at same depth , and in Furthermore, in this case, a structural difference between the two testicles was relatively obvious from the clinical and B-Mode examinations; introducing sonoelastography into routine examinations could be useful to identify lesions potentially able to impair the reproductive condition of the stallions before the appearance of clinical signs.Overall, pathological findings observed herein  match with those reported in seminomas in the horse and in other animal species.Regarding the hormonal profiles, results show a marked decrease in serum testosterone concentrations and a mild decrease in blood or serum estrogen in the days after surgery. Recent research by Valdez and colleagues  also shoThese findings are justified by the bibliography consulted, as it is well-known that hemicastration determines an increase in the serum Follicle Stimulating Hormone (FSH)  and LuteThe above-cited physical and hormonal modification, following the hemi-orchidectomy, determine the complete recovery of a normal fertility, as also demonstrated in this report comparing spermiograms pre- and post-surgery. The positive outcome after unilateral castrations is supported by consulted references ,32,33.B-mode ultrasonography is part of the clinical routine for evaluating the stallion’s fertility ; however


This is a case report of a 31-year-old woman with past medical history of hereditary angioedema (HAE) who developed acute right ventricular dysfunction. The patient presented to the emergency department with complaints of acute abdominal pain and swelling. Her electrocardiogram demonstrated sinus tachycardia and T wave inversion in leads V1-V3, otherwise without findings suggestive of ischemia. Troponin was elevated at 1.83 ng/mL. A transthoracic echocardiogram showed normal left ventricular function with ejection fraction of 65-70%, but the right ventricle (RV) was dilated and severely hypokinetic and there was moderate tricuspid regurgitation. Patient was managed symptomatically for her HAE exacerbation. Her abdominal swelling resolved, troponins continued to trend down, and she was discharged home after three days. A follow up echocardiogram done six months later demonstrated normal RV function.  The physiology and anatomy of the right ventricle (RV) is much different than its neighbor the left ventricle. Factors that influence the RV include preload, afterload, and left ventricular function ,2. IncreA 31-year-old female with history of hereditary angioedema (HAE) presented with acute abdominal pain with swelling and distension. The patient had a history of HAE and her exacerbations resulted in significant abdominal pain, swelling and, distension. On exam, she appeared in moderate distress. Vitals were significant for heart rate of 120. Abdominal exam revealed significant distension, hypoactive bowel sounds, tenderness to palpation, and guarding without any localization. Lungs were clear to auscultation. Electrocardiogram demonstrated sinus tachycardia with new T wave inversions in leads V1-V3, otherwise there were no acute findings suggestive of ischemia. Troponin obtained was elevated at 1.83 ng/mL. Chest X-ray showed no acute pathology. A transthoracic echocardiogram showed normal left ventricular function with ejection fraction of 65-70%, but the right ventricle was dilated and severely hypokinetic with moderate tricuspid regurgitation [Figure Baseline echocardiogram completed one month prior to admission was normal. She underwent ventilation perfusion scan which was low probability for PE. Cardiology was consulted, acute coronary syndrome with RV infarct was thought to be unlikely, and thus cardiac catheterization was not pursued. Patient was managed supportively with volume resuscitation and symptomatically for her HAE exacerbation. Her abdominal swelling resolved and troponins continued to trend down and she was discharged home after three days. A follow-up echocardiogram done six months later showed normal RV function Figure . This case demonstrates a rare etiology of RV dysfunction secondary to increased intra-abdominal pressure from acute flare up of HAE. Hereditary angioedema is an inborn autosomal dominant disease due to a deficiency of C1 inhibitor (C1INH) or a dysfunctional protein . C1INH iManifestations of intestinal angioedema include nausea, vomiting, diarrhea, abdominal pain, and abdominal distention from ascites . IncreasFactors that influence right ventricular function include preload, afterload, and left ventricular function ,2. The aIn this case, the patient’s HAE exacerbation led to acute abdominal swelling. This is an unusual case report of acute HAE causing abdominal distension leading to RV dysfunction. Maintaining a high index of suspicion of RV failure as a consequence of an abdominal HAE exacerbation may improve outcomes in such cases.


Intramuscular myxoma is a rare entity that may present as single or multiple lesions in patients with Mazabraud’s syndrome and is characterized by intramuscular myxomas with fibrous dysplasia. Though intramuscular myxomas occur in large muscle groups, they can very rarely occur in the chest wall. We present the case of a 41-year-old woman with an incidentally discovered intercostal mass on magnetic resonance cholangiopancreatography (MRCP). Repeat MRI demonstrated a lobulated, T2-hyperintense intercostal lesion and demonstrated adjacent fibrous dysplasia of the ribs, consistent with the patient’s history of Mazabraud's and McCune Albright syndromes. Histopathological exam following surgical resection confirmed a diagnosis of intramuscular myxoma without the presence of sarcomatous changes. Though small, slow-growing intramuscular myxomas may be observed with conservative management in the absence of significant symptoms, surgical resection is warranted to prevent complications such as osseous erosion or nerve impingement. Myxomas are rare, benign mesenchymal tumors that can occur in various anatomic locations either independently or as a constellation of pathologic syndromes. Though the most frequent site for myxomas is within the myocardium, extracardiac myxomas can occur most commonly in musculature, primarily in large muscle groups . Very raA 41-year-old woman with a history of Mazabraud’s and McCune Albright syndromes and known polyostotic fibrous dysplasia, multiple endocrinopathies , and multiple prior symptomatic myxomas, presented for the evaluation of an incidentally discovered right intercostal mass between the seventh and eighth ribs on MRCP performed for follow-up imaging of known pancreatic cysts and surveillance for intraductal papillary mucinous neoplasm. She notes that she has not had any recent pain associated with the right chest wall, and she has not noticed any enlarging, palpable mass. Relevant medical and surgical history includes multiple prior gluteal myxomas with surgical resection for worsening pain, resulting in symptomatic relief. Additionally, the patient reported ongoing evaluation for a slowly enlarging right neck myxoma causing right arm pain. Physical exam revealed a small, palpable right neck mass but did not reveal any palpable mass or tenderness along the region of interest at the chest wall.A review of the MRCP from one month prior demonstrated stability of her multiple cystic pancreatic lesions measuring up to 8 mm, without suspicious features, and revealed a significantly T2-hyperintense lobulated lesion measuring 3.1 x 2.7 x 2.5 cm in the posterior seventh to eighth intercostal space. Repeat MRI of the chest at the time of this visit confirmed this finding  antibiotics. Treatment with antibiotics  improved her clinical course with a resolution of her symptoms. Her chest tube output gradually declined, and all chest tubes were removed without complication.The patient was admitted for pain management and discharged on postoperative Day 1 following her resection with a right-sided Jackson Pratt (JP) drain terminating in her pleural cavity. Her postoperative course was complicated by right-sided empyema secondary to Intramuscular myxomas are a sub-type of soft tissue myxomas representing rare, benign tumors of mesenchymal origin with a predilection for middle-aged  and female patients, as seen in the current case . IntramuTypical imaging findings of intramuscular myxomas include homogenous, hypodense lesions with well-defined margins and soft-tissue attenuation on CT images . CorrelaHistological diagnosis of intramuscular myxoma requires a hypocellular, hypovascular lesion with an abundant myxoid component -9. CellsSmall, slow-growing intramuscular myxomas may be observed with conservative management in the absence of significant symptoms . HoweverSimilarly, surgical intervention is warranted for fibrous dysplasia in the context of worsening mass effect from growing lesions, or from rare malignant transformation to osteogenic sarcoma, as noted in case reports of patients with Mazabraud’s syndrome -13.Intramuscular myxomas are rare lesions associated with Mazabraud’s and McCune Alright syndromes. Though these lesions tend to occur in large muscle groups, they may rarely present in the chest wall. When associated with these syndromes, intramuscular myxomas tend to occur near regions of fibrous dysplasia. Small, slow-growing intramuscular myxomas may be treated with conservative management; however, surgical intervention is warranted to prevent complications such as adjacent osseous erosion or nerve impingement from tumor growth.


CCND1-IGH fusion and manifests with a spectrum of disease ranging from relatively indolent to aggressive. Here, we present a case of pleomorphic mantle cell lymphoma with three fusion signals that presented with lethal atraumatic splenic rupture. We discuss on the implications of variant CCND1 signal patterns as well as the epidemiology and pathophysiology of atraumatic splenic rupture.Mantle cell lymphoma is characterized by t with  It represents 3-10% of newly diagnosed cases of non-Hodgkin lymphoma in Western countries and predominantly affects men M:F 2:1) with a median age of 60 years.1:1 with aCCND1, the Cyclin D1 gene on chromosome 11, with IGH, the immunoglobulin heavy chain gene on chromosome 14. The remaining cases often demonstrate fusions with the Cyclin D2 or Cyclin D3 gene.3,5,3,7,Greater than 95% of cases of MCL are characterized by gene fusion of ,3,12,Although the diagnosis of MCL carries a poor prognosis with a median survival of 3-5 years, it can be further subclassified into several morphologic variants that have a range of prognoses.9,16,16,16,MCL presents with advanced stage disease (stage III-IV) in 85-87% of cases.16Herein, we present a case of pleomorphic MCL that presented with lethal atraumatic splenic rupture which has only rarely been reported in the literature.The patient is a 70-year-old man with a past medical history of hypertension, insulin-dependent diabetes mellitus, meningioma status post resection, and colon cancer status post resection who presented to an outside hospital with acute onset abdominal pain and was found to have a 10 cm spontaneous grade IV splenic laceration with active extravasation  splenomeDuring transfer to our tertiary care hospital, he had increased abdominal distension and became hypotensive. He was given two units of packed red blood cells but was hemodynamically unstable and became unresponsive upon arrival. A massive transfusion protocol was initiated with temporary regain of consciousness. An emergent IR coil embolization was performed and the patient was transferred to the surgical ICU. Over the next few hours, despite supportive measures, he became progressively hypotensive, acidotic, and hypercarbic. The patient expressed that he wished to discontinue treatment and die peacefully. His family was brought to the bedside and the patient was moved to comfort-measures only. He passed away shortly thereafter. An autopsy was not performed3  with 79% lymphocytes, 11% monocytes, 10% neutrophils, red blood cell 3.49 M mm3 , hemoglobin 10.0 g/dL , hematocrit 31.0% , MCV 89 fl , platelet 116,000/mm3 . Examination of the peripheral smear showed nucleated red blood cells and a population of atypical lymphocytic cells characterized by variable size, irregular nuclear contours, and high nuclear:cytoplasmic ratios  analysis which was positive for t, demonstrating a variant abnormal pattern with three CCND1-IGH fusion signals .-In a systematic review identifying 845 cases of atraumatic splenic rupture (ASR), Renzulli et al.19CCND1-IGH fusion gene appears to be associated with the more aggressive variants of MCL. In a case series of leukemic MCL, Rahman et al.CCND1-IGH fusion gene, massive splenomegaly, and an aggressive clinical course. Miao et al.CCND1-IGH fusion gene that initially presented as leukemic MCL (classic variant) and later transformed to the pleomorphic variant. As discussed by Miao et al.,CCND1-IGH fusion amplification alone has on the disease course is often masked in these cases by a complex karyotype, which is known to portend an aggressive clinical course. Finally, as reported by Ott et al.,CCND1-IGH fusion. Although analysis of the current case is limited by incomplete diagnostic data (karyotype was not performed), the pleomorphic morphology of the leukemic MCL fits into the same category as these previously reported cases. It is unclear whether the more aggressive course seen in pleomorphic MCL can be attributed specifically to the extra copies of CCND1-IGH or if this may be just a representation of a complex disease karyotype.The diagnosis of the pleomorphic variant of classic MCL in this case is supported by the finding of pleomorphic lymphoma cells in the patient’s peripheral blood, splenomegaly (20cm) and iliac lymphadenopathy (4.1cm) on imaging, immunophenotype and cytogenetic findings typical of MCL. Biopsies of the spleen and lymph node were not performed. Leukemic non-nodal MCL was ruled out given significant adenopathy. Though atypical t presentations may occur in up to 17% of MCL,-ASR in the setting of splenic involvement by an aggressive lymphoma raises the question of whether the neoplasm’s high proliferative rate had an impact on the risk of splenic rupture, i.e., whether the rate of splenic expansion outpaced the rate of capsular compensation such that the tensile forces were too great to maintain integrity of the capsule. This mechanism as well as two other possible mechanisms of ASR have been proposed: acute splenic compression by abdominal musculature during physiological activities, and progressive subcapsular hemorrhage in the setting of splenic thrombosis or infarction due to reticular endothelial hyperplasia.30-24,This case is not the first reported case of ASR in mantle cell lymphoma, nor is it the only pleomorphic/blastoid MCL that presented in the spleen.20Understanding the structural and physiologic consequences of hematopoietic neoplasms is important for informing screening and treatment decisions. This case of pleomorphic mantle cell lymphoma presented with splenomegaly and lethal atraumatic splenic rupture. While this is a rare presentation of MCL, this case highlights the importance of recognizing the risks of splenomegaly in aggressive lymphomas.


Pure erythroid leukemia (PEL) is an aggressive and exceedingly rare form of acute leukemia characterized as a neoplastic proliferation of immature cells committed to the erythroid lineage. It has a poor overall median survival of two to three months. To our knowledge, there are currently only a handful of reports of PEL arising from polycythemia vera. Most reported cases have been associated with radiation therapy or chemotherapeutic alkylating agents. Here we report a rare occurrence of polycythemia vera treated with phlebotomy and hydroxyurea that underwent leukemic transformation to pure erythroid leukemia.  Polycythemia vera (PV) is a chronic myeloproliferative neoplasm (MPN) caused by the overproliferation of red blood cells by the bone marrow. Current therapeutic options for PV  aim at reducing vascular and thrombotic complications. PV also carries the risk of leukemic transformation as a rare and late event in the disease . In our PEL is an aggressive and exceedingly rare form of acute leukemia. In 2016, World Health Organization (WHO) classified it as a subtype of acute myeloid leukemia and the only type of acute erythroid leukemia . CompareTo our knowledge, there are currently only a handful of reports of PEL arising from PV. Most reported cases have been associated with radiation therapy and chemotherapeutic alkylating agents -8. Here JAK2) V617F- positive polycythemia vera, managed for seven years with phlebotomy, aspirin, and hydroxyurea, presenting to the clinic with complaints of muscle aches, fatigue, loss of appetite, lightheadedness, and night sweats for two months. She was found to have developed petechiae on her upper extremities in addition to previous petechiae on her lower extremities. She was also noted to have a palpable spleen on physical exam. Complete blood count showed white blood cells (WBC) 7.9, Hgb 13.0, hematocrit (Hct) 41.8, platelets 63. She was advised to stop hydroxyurea and aspirin at the time.We present the case of a 67-year-old female with a history of janus kinase 2 (The following month (May 2020), the patient returned to the clinic with a complaint of continued fatigue. In addition, she had noticed her urine becoming darker and her legs becoming more swollen. Blood workup was notable for leukocytosis (WBC 17.4), anemia , and thrombocytopenia (18). Manual differential was notable for increased bands (22%), elevated lymphocytes (5.92), elevated monocytes (1.74), elevated metamyelocytes (0.52), elevated myelocytes (1.74), elevated promyelocytes (0.70). She was sent to the emergency department (ED) for transfusion. In the ED, she had an abdominal ultrasound performed, which showed hepatosplenomegaly with spleen measuring 26 cm in length and liver measuring 23.48 cm in craniocaudal diameter.The patient underwent a bone marrow biopsy due to concern for myelofibrosis. Bone marrow aspirate showed numerous blasts displaying a high N:C ratio, large nuclei, fine chromatin, prominent eosinophilic nucleoli, and deep blue cytoplasm, including some blasts showing vacuolated cytoplasm Figure . The bonUnfortunately, the patient was a poor candidate for aggressive chemotherapy and was started on non-intensive chemotherapy with azacytidine and venetoclax. She continued to spike fevers with worsening thrombocytopenia. After discussion with the patient and family regarding goals of care, the patient was transitioned to comfort-focused care and discharged on hospice. She passed away two weeks later.Polycythemia vera is a chronic myeloproliferative neoplasm (MPN) characterized not only by erythrocytosis but also by overproduction of the myeloid and megakaryocytic components in the bone marrow. The most prevalent complications in patients with PV are vascular and thromboembolic events, such as strokes, acute coronary syndromes, deep vein thromboses of the extremities, and pulmonary emboli. Given the high mortality associated with thrombotic events in patients with PV, the goal of current therapies is to reduce the risk of thrombosis. Initial treatment depends on each patient’s thrombotic risk. Patients are stratified into “high-risk” or “low-risk” categories according to their age and history of thrombosis . PatientCurrently, there are two recognized phases of PV. The first phase is an initial polycythemic phase characterized by an elevated hemoglobin level and increased red blood cells (RBC) mass. The later spent phase, also known as the post-polycythemia myelofibrosis phase, is characterized by ineffective hematopoiesis, extramedullary hematopoiesis, cytopenia, and bone marrow fibrosis. A rare event that can occur late in the progression of PV is the evolution to acute leukemia, also known as a myelodysplastic or blast phase. An international multicenter study estimates the cumulative incidences of leukemic transformation at 2.3% at 10 years after initial diagnosis of PV, 5.5% at 15 years, and 7.9% at 20 years . This trIn our case, the patient underwent a transformation to a rare subtype of AML known as PEL. PEL represents a neoplastic proliferation of immature cells committed to the erythroid lineage. 2016 WHO classification scheme classifies PEL as the only subtype of acute erythroid leukemia [PEL evolving from an MPN is exceedingly rare, with only a few cases reported to our knowledge. Between 1952 and 1962, five cases detailing the transformation of PV to PEL were documented in patients treated with radioactive phosphorus or alkylating agents . Kreft eThe mechanism underlying leukemic progression remains unclear and has been suggested to be multifactorial. Advanced age and leukocytosis are well-established risk factors in patients with PV for progression to myelofibrosis. The European Collaboration on Low-Dose Aspirin in Polycythemia (ECLAP) study revealed that the persistence of leukocytosis despite treatment with hydroxyurea may serve as a marker of disease aggressiveness, indicating a greater risk of disease evolution and decreased survival in patients with this risk factor . Other fOur case highlights one of only a few reported instances of leukemic transformation from PV to PEL, without the presence of radiation therapy or alkylating agents. However, there remains some confusion in pinpointing the pathogenesis of PEL in our patient. Some studies have accepted leukemic transformation as a late event in the natural progression of PV , 17. OurPerhaps there may also be a role for alternative therapies such as janus kinase (JAK)2 inhibitors as a second-line treatment option for PV. Currently, second-line treatments for patients with PV at high risk for vascular complications include busulfan and radioactive phosphorus. However, studies observed that these agents have been associated with an increased risk of progression of the disease to AML or myelodysplastic syndromes . In the Leukemic transformation to PEL represents a rare and fatal complication of PV that can occur even in the absence of treatment with known leukemogenic agents. As first-line therapy for PV, hydroxyurea use has been increasing over the last twenty years. However, the currently unmet therapeutic need and possible harm of patients with intolerance or resistance to hydroxyurea require that we investigate further into the mutagenic potential of hydroxyurea. This case also reveals another challenge, which will be the identification of patients who are at high risk of leukemic transformation. Going forward, it is anticipated that with advancements in genetic profiling, we will soon be able to identify the genetic mutations that lead to disease progression and transformation in MPNs, which will certainly be key to improving the classification, prognostication, and treatment of patients with PV.


The World Health Organization (WHO) classifies odontomas as odontogenic tumors, consisting of odontogenic epithelium and ectomesenchyme. They result from developmental abnormalities and, therefore, do not constitute authentic tumors. It is a local malformation that has no growth autonomy. They are rarely symptomatic and are usually discovered accidentally during the realization of a radiographic examination. There are two variants of odontomas: complex and compound. Complex odontomas are made of a mass consisting of an anarchic assembly of mineralized tissue  and dental pulp; while compound odontomas are consisting of a set of small rudimentary teeth, assembling in clusters. They rarely show the features of both types together. The aim of this work is to report a rare presentation of an odontoma in a 24‐year‐old male patient, which present the characteristics of both complex and compound variants. Surgical excision of the lesion was performed. Anatomopathological examination confirmed the diagnosis. Clinical and radiological survey does not show any recurrence. Compound‐complex odontoma is an uncommon variant of odontoma; it is detected through routine radiographic examination. It is, therefore, advisable to conduct an accurate diagnosis in order to avoid subsequent complications. It is recommended to perform histopathological analyses so as to discard the possibility of association to some other type of odontogenic tumors. It would have a pedagogical and epidemiological interest as it shows a clear example of an uncommon variant of odontoma, which exhibits the characteristics of both composite and complex types.2A 24‐year‐old male patient presented to the Department of oral medicine oral surgery of the university dental clinic of Monastir, Tunisia with the chief complaint of pain in mandibular right wisdom teeth. Patient's medical and family histories were non‐contributory. The clinical examination showed, in addition to insufficient hygiene, a disorder of the mandibular anterior teeth, the right mandibular wisdom tooth was decayed. A routine panoramic radiograph showed a large radiopaque lesion in the right posterior mandible delimited by a radiolucent halo and extended from the first molar to the second molar region. The lesion was composed by two parts. The first one consisted of an assemblage of multiple odontoids and the second one composed of an anarchic radiopaque image. The cone‐beam computed tomography revealed a lesion involving the right body of mandible and shifted more on the buccal side. There was an expansion and thinning of the buccal cortical of the mandible Figure . Accordi3Odontomas represent an important entity among maxillary benign odontogenic tumors.A bibliographic research on PubMed platform was performed. The articles included were written in English language and published until 2021. Only five publications (five case reports and one retrospective study) report cases of compound‐complex odontomas.There are different clinical aspects of odontoma. It can be either intra‐osseous, extra‐osseous or erupted.Differential diagnosis must be performed with ameloblastic fibroma, ameloblastic fibro‐odontoma, and odontoameloblastoma.Odontomas can also manifest as part of syndromas, such as Gardner syndrome, basal cell nevus syndrome, familial colonic adenomatosis, Tangier disease, or Hermann syndrome.The treatment consists in a surgical removal of the lesion with curettage. Their enucleation is simple as they are capsulated tumors.The authors declare that they have no conflict of interest or sources of funding for this particular study.Khalifa Chaima has written the manuscript. Omami Mounir has participated in the design, the acquisition of data as well as the revision of the manuscript. The other authors discussed the results by revising critically for important intellectual content and have given final approval of the version to be published. All authors approved the final draft of the manuscript.Our institution does not require ethical approval for reporting individual cases or case series.Written informed consent was obtained from the patient for his anonymized information to be published in this article.


Medulloepithelioma is a rare tumor of the eye, arising from the posterior segment. This embryonic tumor is mostly seen in children and is very rare in adult population.This case report presents the case of a 39-year-old Indian male, who had gradual vision loss over 4 years in his left eye with new onset of pain. He was referred to our center in view of secondary cataract and intraocular mass. Vision in right eye was 20/ 20 while left eye had no light perception at presentation. Ocular examination of the left eye revealed shallow anterior chamber, florid iris neovascularization, raised intraocular pressure and cataractous lens. B-scan ultrasonography showed a heterogenous mass filling the entire globe. MRI scan confirmed the finding, showing a mass hyper-intense to vitreous. No invasion of optic nerve or sclera was observed. Left eye enucleation with PMMA implant placement was performed and histopathology confirmed the diagnosis of benign teratoid medulloepithelioma. At the time of submission of this report, the patient was still under follow-up and had no detectable metastases at 15 months follow-up. This report highlights a very rare case of embryonic tumor in adult male, which could be managed successfully with a high index of suspicion and timely intervention. Histopathology is characterized by presence of pseudostratified epithelium arranged as cords or ribbons along with loose connective tissue, appearing much like developing neurosensory retina and vitreous [2].Medulloepithelioma of the eye is an unusual and rare embryonic tumor, seen mostly in children and with very limited occurrence in adults. Origin of this tumor is noted most from the medullary epithelium of the ciliary body, but occurrence from the retina and optic nerve has also been seen . It has been seen mainly in children, with very few cases reported in adults. Medulloepithelioma in adults might represent delayed transformation of a pre-existent retinal anlage [4].Intraocular medulloepithelioma is a rare embryonic tumor, with a reported incidence of 1 per 4,50,000-10,00,000 population [5]. Sosinska-Mielcarek and colleagues have also reported a case on intraocular medulloepithelioma in a 44-year-old man in 2006, who was started on chemotherapy but died six months later [6]. Origin of medulloepithelioma in the eye is seen most commonly from the ciliary body, but tumors arising from the optic disc, iris, and retinal stalk have also been described [7]. Floyd and co-workers reported a case of intraocular medulloepithelioma in a 79-year-old patient in 1982, arising from ciliary body and was managed successfully with enucleation [4]. Initially, patients are asymptomatic and may later present with symptoms such as vision loss, leukocoria, pain, visible intraocular mass, or red eye. Vision loss is due to cataract formation, lens subluxation or neovascular glaucoma. The index case also manifested as neovascular glaucoma [8]. The presence of cataract, irregular anterior chamber and neovascular glaucoma raised the suspicion of mass lesion in the present case and radiological investigations containing both solid and cystic areas clinched the diagnosis.It is classically unilateral, showing no preference for either eye [9]. Since it is mainly seen in children, it should be differentiated from retinoblastoma, which has characteristic calcifications on B-scan and MRI, but not seen in medulloepithelioma. In adults, differentiation from more common tumors such as choroidal melanoma is required, which shows dome shaped/ mushroom shaped tumor on B-scan, choroidal excavation and subretinal fluid. A high index of suspicion is required to clinch the diagnosis of medulloepithelioma and offer timely management to these cases. Prognosis is generally good and the five-year survival rate is 90-95% if diagnosed early and there is no extraocular spread. However, if not treated in time, deaths have been reported in these cases [4].Primary misdiagnosis is reported in 39% of cases [4]. Smaller tumors may be treated with cryotherapy, radiotherapy or local resection or plaque brachytherapy [10].Enucleation is the standard treatment modality for advanced tumors and neovascular glaucoma. It should be performed with care and least manipulation to avoid spillage of tumor cells [Intraocular medulloepithelioma is very rare in adult population but this case report highlighted that it should be kept in mind as a diagnosis even in adults. A high index of suspicion and timely intervention in these cases is required for a successful long-term outcome.Conflict of Interest statementNil.Informed Consent and Human and Animal Rights statementAny information that could contribute to identify patients, including patients’ names, initials or hospital numbers have not been published in written descriptions or photographs. The manuscript was shown to the patient before submission to the journal and an explicit informed consent was obtained for purpose of publication. Authorization for the use of human subjectsEthical approval: The research related to human use complies with all the relevant national regulations, institutional policies, is in accordance with the tenets of the Helsinki Declaration, and has been approved by the review board of Government Medical College and Hospital, Chandigarh, India.AcknowledgementsNone.Sources of FundingNone.DisclosuresNone.Prior publicationNil.


Meningiomas are common intracranial neoplasms with benign features, and extracranial metastases are very rare. There have been no previous reports of solitary mediastinal lymph node metastasis from benign meningiomas without pulmonary lesions. Here, we present a case of an 82‐year‐old female who visited our department for mediastinal lymphadenopathy with a history of meningioma treated with total surgical resection six years prior. Endobronchial ultrasound‐guided transbronchial needle aspiration of the left lower paratracheal lymph node revealed a benign meningothelial meningioma. In patients with a history of meningioma, extracranial metastasis should be considered in the differential diagnosis of mediastinal lymphadenopathy. (a) Contrast‐enhanced computed tomography (CT) revealed a bulky lymphadenopathy in the left lower paratracheal area compressing the lower trachea and left main pulmonary artery. (b) On an axial section of the enhanced CT scan, a mediastinal mass compressing the left main bronchus was observed. (c), (d) 18F‐fluorodeoxyglucose‐positron emission tomography (FDG‐PET) showed high uptake of FDG with a maximal standardized uptake value of 9.2 by the tumor. Meningiomas are common intracranial neoplasms and tend to have a benign clinical course.Here, we report the first case of a benign meningothelial meningioma with solitary mediastinal lymph node metastasis in a patient without lung metastasis. Mediastinal lymph node metastasis was detected six years after surgical resection of the intracranial meningioma and diagnosed by endobronchial ultrasound‐guided transbronchial needle aspiration (EBUS‐TBNA).An 82‐year‐old woman was referred to our department with mediastinal lymphadenopathy, which was detected incidentally on workup for a rib fracture. The patient had a history of a meningioma and had been treated with total surgical resection at another hospital six years prior. Medical comorbidities included hypertension and diabetes mellitus. She had no family history of malignancy.Laboratory data were all within normal range. Contrast‐enhanced computed tomography (CT) revealed a bulky lymphadenopathy, 75 mm in diameter, located in the left lower paratracheal to the left hilar area. The mass was compressing the lower trachea, left main bronchus, and left main pulmonary artery Figure . 18F‐fluMost meningiomas behave in a benign course, but can sometimes display malignant behavior, such as metastasis. Distant metastases have been reported sporadically in case reports of atypical and anaplastic meningiomas, but have rarely been reported for benign meningiomas.The WHO classification of CNS tumors divides meningiomas into three major groups: grade I (benign), grade II , and grade III (anaplastic) Table .6 AccoExtracranial metastases of meningioma have been detected in the lungs (60%), abdomen and liver (34%), cervical lymph nodes (18%), long bones, pelvis, and skull (11%), pleura (9%), vertebrae (7%), and mediastinum (5%).In our case, the route of metastasis is of particular importance, and we developed several hypotheses to explain it. First, the tumor could have spread via the paravertebral venous plexus. Cases of solitary mediastinal lymph node metastasis in malignant extrathoracic tumors via the paravertebral venous plexus have previously been reported.No consensus on the optimal treatment for extracranial metastatic meningioma has been established to date. Although there are reports of curative metastatectomies for meningiomas, the long‐term prognosis in this rare patient population is not well defined.In conclusion, this is the first reported case of a solitary mediastinal metastasis from a benign meningioma several years after initial tumor resection. This case highlights the aggressive potential of meningiomas, which are typically considered benign tumors, and emphasizes the need to consider metastatic meningioma in the workup of mediastinal masses in patients with a history of meningioma.The authors declare that there are no conflicts of interest.


Calcifying odontogenic cyst (COC) is a rare cyst that affects mainly the anterior region of the jaws. Generally, it appears as a unilocular radiolucent lesion containing peripheral foci of calcification, but with radiographic variations depending on the type of presentation. Here, we report an atypical case of COC associated with odontoma, initially diagnosed as a tooth germ, in the posterior region of the mandible of a 10-year-old male patient. Interestingly, the radiographic aspect appeared as a unilocular radiolucent lesion without peripheral foci of calcification in the edentulous region, having its size increased after traction of the impacted tooth adjacent to that area. Thus, the case presented in this study is aimed at calling dentists' attention to its developmental changes and related pathologies. Calcifying odontogenic cyst (COC) is a rare developmental cyst that favorably affects the anterior region of the jaws , 2. It cTraditionally, COCs appeared as well-defined unilocular radiolucencies, exhibiting peripheral foci of calcification. Atypical radiographic presentations of COC are uncommon . Here, wA 10-year-old male patient was referred to orthodontic traction of an impacted right lower first molar (tooth 46). During clinical examination, no alteration was noted except the absence of tooth 46. No abnormalities were detected in previous medical history. A cone-beam computed tomography (CBCT) showed the presence of a radiolucent image measuring <1 cm located between teeth 46 and 48, which was initially interpreted as a dental germ . The patThereby, a provisional diagnostic hypothesis of odontogenic lesions was made, suggesting adenomatoid odontogenic tumor and COC. The chosen clinical conduct was the surgical enucleation of the lesion. A straight incision was made in the alveolar ridge with flap detachment to facilitate access to the lesion, and an aspiration puncture was performed revealing a liquid content . During 2 reinforce that one-third of patients are affected in childhood and adolescence. The case reported herein is in accordance with the literature.Although COC is a benign lesion and a pathological entity widely known, its occurrence is considered uncommon . GeneralCommonly, COC appears as a unilocular radiolucent area with well-defined limits and may exhibit small radiopaque deposits of various aspects , 7. In sIn the current case, the lesion initially appeared as a well-delimited radiolucent area in the edentulous mandibular bone near an unerupted molar, which did not resemble a COC. During the attempt to traction the impacted tooth, an increasing volume with cortical expansion and fenestration were observed surrounding the radiolucent area, raising the possibility of an odontogenic lesion. Curiously, the lesion resembled a tooth germ in the crypt stage. Due to the exceptionality of the case, a vast literature search was carried out, and to our knowledge, this is the first report of its kind related to COC. On the other hand, the unexpected histological result only increases the notoriety of this report; once, authors described that some cases of delayed eruption may be attributed to physiological disorders impacting interruptions on Nolla's stages , thus leRegarding that, the literature argues that the odontogenic tumors can share the same structures of a tooth germ, which is difficult to diagnose with immature structures of dental tissue, especially in children and adolescents . For thiThis comparative analysis is crucial and may avoid diagnostic mistakes to several oral lesions (odontogenic or nonodontogenic origin), especially when malignant lesions are underdiagnosed. In a retrospective study conducted by Bacci et al.,  the auth7 investigated the radiographic profile of 11 cases of COC and only one of them arose from the edentulous mandible. Arruda et al.2 debated that radiolucent COCs may undergo calcification and progress to a lesion of mixed radiographic appearance, thus causing swelling and cystic expansion. Also, some works suggest that the remaining odontogenic epithelial cords when stimulated may induce the formation of dental tissue in the adjacent connective tissue wall [Also, it is important to highlight that COC may present wide-ranging radiographic patterns, as discussed by different studies. One study by Lida et al.sue wall .The most remarkable feature of COC is the presence of ghost cells in an ameloblastoma-like epithelium , 14. In As a result, a CBCT evolution of a COC in the mandibular molar area was described, going from a hypodense profile to a mixed appearance, thus calling the attention of dentists that radiographic or tomographic images may lead to misinterpretations between odontogenic lesions and dental apparatus forming structures.


Calcifying aponeurotic fibromas (CAFs) are rare benign tumors that typically develop in the soft tissue of the extremities. We report a case of 64-year-old woman with a CAF in the soft tissue surrounding her left clavicle. A plain radiograph showed an asymmetrical increase in opacity of the left internal clavicular region. Computed tomography and magnetic resonance imaging confirmed the presence of a heterogeneous lesion of the periclavicular soft tissue, with peripheral calcifications, and remodeling of the adjacent clavicular bone. Following ultrasound-guided biopsy and surgical resection of the mass, the final histological diagnosis was made. To the best of our knowledge, this is the first case of a CAF described in the soft tissue adjacent to the clavicle. It is essential to use all the diagnostic methods available  to obtain the final diagnosis of this rare disease. In 1953, Keasbey first described a soft tissue lesion located in the hands and feet of four children; he classified the lesion as "juvenile aponeurotic fibroma." In 1964, Lichtenstein and Goldman termed it "fibromatosis cartilage analog." In 1973, Iwasaki and Enjoji termed the same lesion “calcifying aponeurotic fibroma” (CAF) after it was found that it was present not only in children but also in adolescents and adults ,7,11,15.A 64-year-old female patient was referred to our hospital with a history of long-term polyarthralgia and myalgia. She also presented with swelling of the left clavicular region that had been present for 3 years and had progressively increased in size and become more painful. On physical examination, there were no motor or sensory function abnormalities in the left upper limb. Routine laboratory tests showed no significant alterations. A standard chest radiograph revealed clear asymmetry and increased opacification at the left medial clavicular region A. Plain No-contrast computed tomography (CT) revealed the presence of a soft tissue lesion with chondroid calcifications located under the left pectoral muscles: between the first rib, its chondrocostal joint, and the clavicle . It was Because of these findings, the initial diagnostic consideration was synovial osteochondromatosis, although it was not possible to completely rule out a primary cartilaginous tumor of the soft tissue, such as chondroma, periosteal chondroma, or chondrosarcoma. Following an ultrasound B-Mode evaluation , an ultrMicroscopic examination revealed a multinodular, lobulated, architectural pattern with extensive areas of calcified cartilaginous tissue, adjacent non-calcified cartilage remnants, and an outer fibroblastic component without atypia, necrosis, or mitoses . Most ofCAFs are benign fibroblastic tumors located in the subcutaneous tissue Enzinger and Weiss suggested that CAFs develop biphasically, with initial and late phases. In the initial phase, seen more frequently in young patients, the tumor has an infiltrative and destructive growth and often lacks calcification; while in the late phase the tumor is more compact and nodular and exhibits a more prominent degree of calcification and cartilage formation ,8,13,18.MRI and CT are the imaging modalities of choice for CAFs The final diagnosis was reached after percutaneous and surgical biopsy, and subsequent cyto-histological analysis.In conclusion, we present a rare case of a CAF with the lesion located in the soft tissue around the clavicle, an unusual location for this lesion. A thorough clinical evaluation is vital for accurate diagnoses; advanced radiographic and imaging evaluation can aid in preoperative planning, and a definitive diagnosis is obtained by histopathology. Surgical treatment and excision of the tumor produce favorable results; however, recurrence rates are high due to its multinodular characteristics, which may result in seeding or hourglass remnant tumor nests. Although it is a well-known entity, CAFs can be a diagnostic challenge, particularly when they occur at unusual ages and in atypical locations, as in our case. The characteristic images of CAFs may mimic those of malignant lesions and this should be considered in the differential diagnosis when chondroid and sarcomatous soft tissue lesions are suspected.Publication approval was received from Netcare research operations committee. A copy of the approval is available for review by the Editor-in-Chief of this journal.No funding.Written informed consent was obtained from the patient for publication of this case report. A copy of the written consent is available for review by the editor-in-chief of this journal.Salvatore Marsico MD.None declared.


The ampulla of Vater is a unique, highly vascularized pouch. Its anatomic transition is halfway along the second part of the duodenum from the foregut to the midgut. According to the World Health Organization’s (WHO) latest nomenclature, carcinoid tumors are now called neuroendocrine tumors (NETs). Knowledge of NETs is important because of their rarity, reclassification, prognosis, and management. NETs involving the ampulla of Vater are extremely rare, constituting <0.05% of gastrointestinal NETs, and involving the pancreas are rare to our knowledge. There are only a few reports of ampullary NETs.We report two rare NET cases involving the ampulla and pancreas and review the relevant literature. A 71-year-old patient with neurofibromatosis and multiple comorbidities presented with chronic intermittent abdominal pain. Abdominal imaging studies showed a suspicious mass at the level of the ampulla. Endoscopic retrograde cholangiopancreatography and endoscopic ultrasound revealed bulging papillae. Histopathology of the biopsied mass revealed a low-grade NET. Given his multiple comorbidities, the patient was scheduled for endoscopic resection. Our second patient was an 83-year-old lady presenting with nausea and vomiting. Abdominal imaging studies revealed a lobular mass over the body of the pancreas. Histopathological examination of fine-needle aspiration of the mass confirmed a well-differentiated low-grade NET. Octreoscan and dotatate scans showed pancreatic, multiple hepatic and metastatic lesions in the left lung and left shoulder. The patient is currently stable after completing peptide receptor radioligand therapy at a tertiary oncology center. Because of the patients’ comorbidities and staging, their management has taken different approaches.More data and more research are needed for accurate assessment of prognosis; however, a review of the latest literature recommends Whipple resection with lymphadenectomy for all ampullary NETs provided patients can tolerate the procedure. Endoscopic resection or surgical ampullary resection should be performed on contraindicated patients. The majority of ampullary and pancreatic NETs would have metastasized by the time patients sought treatment. Because of their rarity and ill-defined and highly variable presentation, NET diagnosis is always delayed and sometimes incidental; therefore, we emphasize the importance of early diagnosis and management to reduce mortality and morbidity. The ampulla of Vater, also known as the hepatopancreatic duct, was named and first published in 1720 by the German anatomist Abraham Vater [Case 1The first patient was a 71-year-old man with a significant medical history of chronic respiratory failure (requiring oxygen at home), chronic obstructive pulmonary disease, essential hypertension, a remote history of prostate cancer, and neurofibromatosis. The patient was admitted for intermittent, vague, chronic abdominal pain over the past 1 year with a 20-pound weight loss in the last 6 months. He denied loss of appetite, melena, hematochezia, and altered bowel movements. He was a known smoker but denied alcohol and illicit drug abuse. Family history was significant for breast cancer in his mother and pancreatic cancer in his father. Vitals were stable and the patient was on 5 L of supplemental home oxygen. He was anicteric, and no lymph nodes were palpable. The abdomen was soft, non-tender, non-distended and no hepatosplenomegaly or other masses were palpable. The remaining examinations were benign. Preliminary laboratory results were essentially normal, including liver function tests. Computed tomography (CT) of the abdomen and pelvis with contrast Figure . ERCP F showed aCase 2The second patient was an 83-year-old woman with a significant medical history of essential hypertension and type 2 diabetes. The patient was admitted for intractable nausea and vomiting that had been ongoing for the preceding 4 days. The patient had been in otherwise good health before admission. She denied abdominal pain, altered bowel movements, loss of appetite, or loss of weight. She denied tobacco use and illicit drug abuse but was an occasional alcoholic. Family history was significant for; one sister had a history of breast cancer, her paternal aunt had pancreatic cancer, and another sister had a brain tumor. The patient’s vitals were stable. Her abdomen was soft and non-tender, with no hepatosplenomegaly, or other palpable masses. The remaining examinations were also benign. The laboratory results were only significant for serum alanine aminotransferase 85 U/L (reference range 16-63 U/L); aspartate aminotransferase 21 U/L (reference range 15-37 U/L); alkaline phosphatase 195 U/L (reference range 50-136 U/L); total bilirubin 0.9 mg/dL (reference range 0.2-1 mg/dL); and albumin 1.8 g/dL (reference range 3.4-5.0 mg/dL). Serum cancer antigen (CA-125) was 11 units/mL (reference range 0-35 units/mL) and cancer antigen-19 (CA-19) was 33 units/mL (reference range 0-37 units/mL). CT of the abdomen and pelvis without contrast Figure , consistNETs occur in the gastrointestinal (GI) system (73.7% of tumors), bronchopulmonary system 25.1%), and rarely in the ovaries and kidneys . Within .1%, and Ampullary NETs are exceedingly rare, with an incidence <0.05% of GI NETs, in comparison with duodenal NETs, which have an incidence of 2% . To our Pancreatic NETs (P-NETs) are rare, constituting only 0.55% of all pancreatic tumors observed among patients aged between 40 and 60 years ,13. The Metz et al. showed that P-NETs may occur sporadically or in association with genetic conditions such as multiple endocrine neoplasia-1 (80-100% of cases), Von-Hippel Lindau syndrome (20% of cases), and neurofibromatosis-1 (10% of cases) .The current nomenclature and grading systems of NETs were proposed by the European Neuroendocrine Tumor Society in 2006-2007 and were endorsed by the World Health Organization (WHO) between 2010 and 2019  is the most common presentation of non-functional NETs . AdditioDuodenal NETs are a differential diagnosis of tumors of the ampulla of Vater. These are categorized into five categories: duodenal gastrinoma (75%), duodenal somatostatinoma (15%), nonfunctional duodenal NETs , low differentiated neuroendocrine carcinoma, and gangliocytic paraganglioma. Ampullary NET carcinoid syndrome  is a rare presentation that occurs in only 2.8% of cases .The sensitivity of diagnosing primary tumors is only 33% because of their size in CT and MRI studies. ERCP/EUS is the best diagnostic modality; Picus et al. reported 100% sensitivity for EUS . For P-NNET markers, such as the isolation of neuron-specific enolase, synaptophysin, and chromogranin A on histopathologic examination, are important in establishing the diagnosis. Ki-67 is a cell proliferation marker and is also a marker of tumor aggressiveness . StudiesA review of the literature clearly recommends that Whipple’s resection with lymphadenectomy is the management of choice if the patient is suited to and can tolerate the surgery ,27. AnotFor patients with significant comorbidities, such as our first patient who had cardiopulmonary comorbidities (chronic respiratory failure secondary to chronic obstructive pulmonary disease), and tumor sizes <2 cm, either endoscopic resection or surgical ampullectomy is recommended and should be performed by a highly experienced surgeon because of the high associated mortality and recurrence rates ,29,30,31Two studies described that the characteristics of NETs decide their aggressiveness are its size >1 cm increases the risk for lymph node involvement, but not the tumor grade, which decides the aggressiveness ,33. The Prognosis is variable, and the exact prognosis of ampullary NETs is yet to be established because of the extreme rarity and the small number of reported cases. Hatzitheoklitos et al. reported an overall survival rate of 90% . Two stuAmpullary NETs, compared to duodenal NETs, have worse overall survival because they are more advanced at the time of presentation, but overall survival is the same for locally resected ampullary and duodenal NETs . The ove“Carcinoids” have been renamed “neuroendocrine tumors”, according to the latest WHO guidelines. Ampullary and pancreatic NETs are exceedingly rare, their presentation is highly variable, and their diagnosis is often delayed. More data and more research are needed for the accurate assessment of prognoses; however, for all ampullary NETs irrespective of size, Whipple resection has been recommended, provided that the patient can tolerate the procedure. Endoscopic resection or surgical ampullary resection is recommended for patients who cannot tolerate it. The majority of ampullary NETs and P-NETS have metastasized by the time patients present for treatment. Therefore, we emphasize early diagnosis and management to reduce mortality and improve survival rates.


Ectopic adrenocortical tissue is a lesion usually found incidentally during autopsy or inguinal surgery. Here, we demonstrate an extremely unusual case of intrahepatic adrenocortical adenoma which highly mimicks hepatocellular carcinoma (HCC) and brings challenges for clinicians and pathologists. The diagnostic pitfalls have been discussed in detail to provide clues for guiding differential diagnosis and future treatment.A 44-year-old man was admitted into our hospital for evaluation of a hepatic mass identified during routine examination. Enhanced CT revealed its margin displayed apparent enhancement in arterial phase, but hypointensity in portal and delayed phase. HCC was suspected and partial hepatectomy was performed. Microscopically, cells were arranged in solid sheets. Most of the tumor cells were large, polygonal, had prominent nucleoli and were rich in eosinophilic cytoplasm. Pleomorphic nucleus was frequently found. Focally, smaller cells were found with small nuclei and granular cytoplasm. Immunohistochemically, tumor cells were negative for Arg-1, glypican-3 (GPC3), hepatocyte specific antigen (HSA), and positive for synaptophysin (Syn), α-inhibin, and Melan A. The Ki-67 index was 1 %. The final diagnosis was ectopic adrenocortical oncocytic adenoma and the patient was uneventful after the surgery. Intrahepatic adrenocortical adenoma in the liver can hardly be diagnosed through radiology and little experience in pathology has been reported. In the present case, massive oncocytic changes and huge pleomorphism add greatly to the difficulties of making correct diagnosis. This lesion should be carefully kept in mind and a combination of markers is suggested for differentiating from HCC. Ectopic adrenocortical tissue is a condition where adrenocortical tissue appears in places other than the adrenal glands. The ectopic tissue can also form neoplasms such as adenoma or carcinoma . EmbryolClinical history.A 44-year-old man was admitted into our hospital for evaluation of a hepatic mass in April 2020. The mass was first discovered during regular examination in a local hospital five years ago. Ultrasound showed a 4.24 × 2.63 cm hypoechoic nodule in the right lobe of the liver. Abdominal computed tomography (CT) revealed a well-circumscribed oval mass with low density in segment 6 of the liver. Enhanced CT indicated that the mass was heterogenous with obvious enhancement in the margin and a hemangioma was suspected. The patient underwent regular examination in the following four years and the mass was growing slowly. In January 2020, enhanced CT revealed a much larger mass with the maximum diameter of 7.8 cm in the space between segment 6 of the liver and right adrenal gland Fig. . The marThe mass was located on the border of segment 6 and segment 7 of the liver and was close to the inferior right hepatic vein. Partial hepatectomy was performed.The specimen measured 8.5 × 8 × 4.5 cm. On the cut surface, a mass measuring 6 × 5.5 × 4 cm was found immediately beneath the liver capsule. The mass had a relatively clear margin with no obvious capsule. Its cut surface was solid and yellowish-grey with focal hemorrhage Fig. . No necrMicroscopically, the lesion was incompletely encapsulated Fig. a, b. CelImmunohistochemically, the tumor cells were negative for Arg-1, glypican-3 (GPC3), hepatocyte specific antigen (HSA), epithelial membrane antigen (EMA), CK7, CK19, desmin, HMB45, and chromogranin A (CgA). Synaptophysin (Syn), α-inhibin and Melan A were positive. The Ki-67 index was 1 % (Fig. Ectopic adrenocortical tissue or neoplasms were first described in 1740 and have been most commonly found along the pathway from gonads to adrenal glands , 5. HoweThe clinical features of cases that mimick HCC are summarized in Table Six of the eight previous cases contain detailed pathological description Table . Their pIn our case, the lesion was considered to arise primarily from the liver during the surgery. The macroscopic feature of the lesion shows a huge, single distinct nodule with heterogeneity and no obvious capsule, which could certainly happen in HCC. Microscopically, the lesion has a solid growth pattern with high cellularity. The tumor cells seem to show hepatocytic differentiation: Large, eosinophilic tumor cells with striking nucleus polymorphism and prominent nucleoli are characteristic in this case, together with scattered or focal small, clear cells. The lesion was firstly considered to be poorly differentiated hepatocellular carcinoma, with differential diagnosis including metastatic tumors and PEComa. There are several notable points: First, the frequent features in HCC, including combined growth patterns, necrosis, vascular invasion, high mitotic rate, intrahepatic metastasis, are not detected in our case. Second, only a minority of HCC cases develop in a normal or near-normal liver background, especially in elder patients , 18. ThiThe present case and review of the literature indicate that there are diagnostic pitfalls before and during pathological examination. Prior to the surgery, there is no method to differentiate an ectopic adrenocortical tissue from HCC, especially when the patient has history of excess alcohol or other risk factors of HCC. Pathologically, when the ectopic adrenocortical adenoma has substantial oncocytic changes, it is easily misdiagnosed as HCC. Several points should be of notification: First, an ectopic adrenocortical neoplasm should be kept in mind, even when the tumor arises from the liver. Second, the arrangement of cells and their characteristics should be carefully examined and factors suggesting malignancy such as necrosis and vascular invasion should be carefully examined. Third, the markers of HCC are not specific enough, and there has been one case in which ectopic adrenocortical adenoma partially expresses GPC3 Table  13, 21,,21, 22. The clinical manifestation of this patient and all the previous cases with follow-up information is relatively benign. No evidence of metastasis or recurrence is found, which is consistent with the biological behaviour of adrenocortical adenoma. Currently, our patient is visiting the outpatient regularly and is uneventful.In conclusion, intrahepatic adrenocortical oncocytic adenoma is an extremely rare lesion which can be easily misdiagnosed as HCC both before surgery and during pathological examination. Our report focuses on providing clues for surgeons and pathologists.


Acute mediastinitis is a rare disease that rapidly progresses with a high mortality rate. Its most common cause is direct injury of the mediastinum, including iatrogenic causes such as cardiac surgery or upper endoscopy. Enzymatic mediastinitis is a rare complication of a pancreatic fistula caused by the inflammatory digestion of the parietal peritoneum spreading to the mediastinum. Here, we present two cases of enzymatic mediastinitis caused by total gastrectomy with splenectomy. One of them was successfully treated and cured after early diagnosis and transabdominal drainage.Case 1 was that of a 60-year-old man (body mass index [BMI] 27) with a medical history of diabetes and hypertension who was diagnosed with advanced gastric cancer in the upper body of the stomach. A total gastrectomy with splenectomy was performed. The patient experienced acute respiratory failure 24 h after surgery. Pulmonary embolism was suspected, so a computed tomography (CT) scan was performed; however, no relevant causes were found. Although he was immediately intubated and treated with catecholamine, he died in the intensive care unit (ICU) 40 h after surgery. Post-mortem findings revealed retroperitonitis caused by a pancreatic fistula spreading towards the mediastinum, causing severe mediastinitis; a review of the CT scan revealed pneumomediastinum. We concluded that the cause of death was enzymatic mediastinitis due to post-gastrectomy pancreatic fistula. Case 2 involved a 61-year-old man (BMI 25) with a medical history of appendicitis who was diagnosed with advanced gastric cancer at the gastric angle between the lesser curvature and the pylorus, spreading to the upper body of the stomach. A total gastrectomy with splenectomy was also performed. The patient had a high fever 3 days after the surgery, and a CT scan revealed pneumomediastinum, indicating mediastinitis. As the inflammation was below the bronchial bifurcation, we chose a transabdominal approach for drainage. The patient was successfully treated and discharged.Acute mediastinitis caused by gastrectomy is rare. The acknowledgment of abdominal surgery as a cause of mediastinitis is important. In treating mediastinitis caused by abdominal surgery, transabdominal drainage may be a minimally invasive yet effective method if the inflammation is mainly located below the bifurcation of the trachea. Acute mediastinitis is a rare post-operative complication. Its incidence in cardiac surgery ranges from 1 to 2% with a 14% to 47% mortality rate –5. AbdomA 60-year-old Japanese man (body mass index [BMI] 27) with a medical history of diabetes and hypertension was diagnosed with advanced gastric cancer in the upper body of the stomach. Total gastrectomy with splenectomy and D2 lymph node dissection were performed as indicated by the Japanese gastric cancer treatment guidelines before 2018. The operation time of the patient was prolonged due to the thick visceral fat and firm adhesions observed on the lateral side of his spleen. The splenic artery was ligated and cut near the splenic hilum to preserve blood flow to the pancreas. The surgical time elapsed was 6 h and 45 min, and the total blood loss was approximately 1,499 mL. Two drains were placed on the right side of the esophagoenterostomy and below the left diaphragm. The final pathological finding was pT2N0M0, pStage IB . The post-operative course is shown in Fig. A 61-year-old man BMI 25) with a medical history of appendicitis was diagnosed with advanced gastric cancer at the gastric angle between the lesser curvature and the pylorus with a poorly differentiated component spreading to the upper body of the stomach. Total gastrectomy with splenectomy and D2 lymph node dissection were performed as indicated by the Japanese gastric cancer treatment guidelines before 2018. The splenic artery was ligated and cut near the splenic hilum to preserve blood flow to the pancreas. Two drains were placed, one below the esophagoenterostomy and another below the left diaphragm. The total surgical time elapsed was 5 h and 56 min, and the total blood loss was approximately 167 mL. The final pathological finding was pT4aN3aM0, Stage IIIB . Both drains turned dark red one day after surgery, and the drain amylase levels were measured at 2,904 U/L and 5,203 U/L below the esophagoenterostomy and below the left diaphragm, respectively. Although the drain discharge cleared and the drain amylase level decreased to 575 U/L, the patient developed a high fever 3 days after surgery. A CT scan revealed fluid around the retroperitoneum and near the pancreas. This continued to the mediastinum and was accompanied by pneumomediastinum below the branchial bifurcation (Fig.  with a mMediastinitis is mainly caused by direct infection of the mediastinum. This is called direct mediastinitis, and cardiovascular surgery is the most common cause whereas 90% of nonsurgical mediastinitis occurs from esophageal perforations . As the Mediastinitis caused by total gastrectomy is very uncommon, and among the few reports, all were caused by esophagojejunal anastomosis leakage –5. The dPseudomonas aeruginosa and Enterobacter cloacae, which are commonly seen in the intestines, are known to activate trypsinogen [Pancreatic fistula is a well-known complication of gastrectomy. Damage to the pancreatic parenchyma and blood flow disorder due to lymph node dissection of the upper edge of the pancreas are known to be the causes of this complication , 11, andpsinogen . A randopsinogen . AlthougSeveral reports have investigated the risk factors for post-operative mediastinitis in cardiothoracic surgery. Although the studies do not agree, obesity –19, prolMediastinitis manifests within a spectrum that ranges from sub-acute to fulminant and critically ill patients who require immediate intervention in order to prevent death . AlthougIn treating acute mediastinitis, aggressive early debridement and reconstructive procedures are recommended, but the type of approach remains controversial , 9 Tabl. In cardRegardless of the type of mediastinitis, the severity of its symptoms and inflammation will determine whether a surgical approach must be chosen, and the main location of the inflammation will determine how the surgery should be performed.In Case 2, as the disease was due to a transabdominal infection and the inflammation was observed below the bronchial bifurcation, we decided to perform transabdominal drainage. A post-operative CT scan revealed a remarkable reduction in the size of the abscess. Transabdominal drainage has the benefit of determining the infection site, and the drain can also be repositioned in a more effective location. Thus, we suggest transabdominal drainage might be a much simpler yet effective treatment for enzymatic mediastinitis if inflammation is observed in the lower mediastinum.We encountered two rare cases of enzymatic mediastinitis caused by total gastrectomy. In both cases, pancreatic leakage was the cause of this severe complication. Although rare, in cases of acute onset of high fever and severe respiratory disorder, the recognition of mediastinitis will enable early diagnosis and intervention. We suggest that transabdominal drainage is an effective approach for treating mediastinitis with inflammation observed below the bronchial bifurcation.


Neurofibromatosis is a disease that causes various abnormalities such as neurofibroma, mainly in the skin and nerves. The common sites in the oral cavity are the palate, gingiva, tongue, buccal mucosa, and lips but, occurrence in the mandible is rare.A 26-year-old woman was referred to our clinic because of percussion pain. Radiographic findings showed a radiolucent area. The patient was clinically diagnosed with a radicular cyst by a previous doctor. Multiple café-au-lait spots were found disseminated on her body, and she had already been prenatally diagnosed with neurofibromatosis type 1 (NF1). We performed a biopsy and suggested a neurofibroma. Tumor extirpation was performed under general anesthesia. The histopathological diagnosis showed a neurofibroma.NF1 is a systemic nevus that causes abnormalities in melanocytes and Schwann cells, and various lesions appear, but intramandibular lesions are extremely rare. Diagnosis of NF1 and radicular cysts in the mandible is difficult due to their image resemblance. However, it should be kept in mind if the underlying disease is NF1. In our case, it was easy to detach and may have originated from small peripheral nerve endings in the mandible. It is an autosomal dominant inherited disorder whose main symptom is neurofibroma. It frequently occurs in the characteristic skin pigment spots, skin, and nerves [2Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request. This research work has been reported in line with the SCARE 2020 criteria [3A 26-year-old female patient was referred to our department by her primary care dentist because of percussion pain in the left lower first molar. Her face was symmetrical, and she had no paraesthesia in her left mental region. She had a bone-like hard swelling in the gingivobuccal fold from the left lower second premolar to the first molar a. Her leThe patient had multiple café-au-lait spots disseminated on her body. These spots were also observed on the facial skin e. Her chA biopsy was performed under local anesthesia during the first visit. Histological examination of biopsies revealed that most of them were inflammatory granulation tissue. In addition, spindle-shaped cells with wavy nuclei along with collagen fibers, some of which were positive for S-100 protein, suggesting a possibility of neurofibroma. We strongly suspected it to be a neurofibroma from her medical history.Under general anesthesia, we performed the extraction of the left lower first molar and tumor extirpation and apicoectomy of the left lower second premolar. There was no adhesion between the tumor and the surrounding bone. A part of the bone wall of the mandibular canal was missing, and a lower alveolar neurovascular bundle was observed. The patient’s prognosis was favorable with no signs of recurrence after the operation.The extracted specimen was 16 × 13 mm elastic-soft mass with heterogeneous granulation-like tissue with a mixture of milky white and red tissues a. Histop4Neurofibromatosis is a disease that causes various abnormalities such as neurofibroma, mainly in the skin and nerves. It is classified into neurofibromatosis type 1 and neurofibromatosis type 2 (NF2). NF1 has systemic café-au-lait spots and neurofibromas, and NF2 has bilateral acoustic neuroma. The causative genes of NF1 and NF2 are located on chromosome 17 17q11.2) and chromosome 22, respectively, and the causative genes are completely different ,4. NF1 w.2 and chNeurofibromas occurring in the mandible may be derived from the inferior alveolar nerve main duct or small peripheral nerve endings. Because the tumor does not have a capsule and is prone to recurrence, resection of the inferior alveolar nerve vascular bundle is indicated when the tumor is derived from the inferior alveolar nerve . HoweverNone.None.The study was approved by the Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences and Okayama University Hospital, Ethics Committee.Formal consent was obtained from the patient for the publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Yuki Kunisada: Main author, contributing in study concept/design, data collection, analysis, and writing the paper.Norie Yoshioka: Investigation, resources, writing - review & editing.Soichiro Ibaragi: Validation, supervision.Tatsuo Okui: Validation, supervision.Hitoshi Nagatsuka: Histopathology, data interpretation, and editing the paper.Akira Sasaki: Validation, supervision.Not applicable.Akira Sasaki professor.Not commissioned, externally peer-reviewed.


Ganglioneuromas are a benign tumor originating from neural crest cells. As one of the neuroblastic tumors, ganglioneuromas are most common in children, with a mean age at presentation of 7 years. Ganglioneuromas are typically singular in nature, but rarely can present with lymph node involvement and distant metastasis. We present a rare case of a retroperitoneal ganglioneuroma found in a human immunodeficiency virus positive adult, which was complicated by lymph node involvement. This case is notable not only in regard to the age of the patient, but also because of his human immunodeficiency virus positive status and the extension of tumor to lymph nodes.A 27-year-old Latino male with history of human immunodeficiency virus presented with a 6-month history of left upper quadrant and epigastric abdominal pain with associated nausea and vomiting. The patient had a computed tomography scan showing a retroperitoneal mass encasing the aorta, celiac axis, and superior mesenteric artery. Core needle biopsy revealed ganglioneuroma. Owing to obstructive symptoms, resection of the mass along with partial gastric resection, partial pancreatic resection, and splenectomy was performed by a multidisciplinary group of surgeons. Pathology results revealed metastatic spread of ganglioneuroma to surrounding lymph nodes.Ganglioneuromas are most common in children and very rarely occur in adults. However, it is still important to consider this entity in the differential for patients presenting with suspicious symptoms. While rare, it is essential to consider metastasis in this generally benign disease. Ganglioneuromas are benign tumors of neural crest cells. Along with ganglioneuroblastomas and neuroblastomas, they make up the neuroblastic tumors. Ganglioneuromas may form either spontaneously or through maturation of another, more immature, neuroblastic tumor . They prA 27-year-old Latino male with HIV presented to the emergency department complaining of a 6-month history of constant, non-radiating, left upper quadrant abdominal and epigastric pain, with associated nausea and vomiting. Besides HIV, the patient’s past medical and surgical history was unremarkable. The exact date of HIV diagnosis was not available in the patient’s medical record, but at the time of presentation, the patient was not on antiretroviral medication. Social and environmental history were remarkable for a 2-pack-year history of tobacco use and less than one alcoholic drink per week. His family history was only notable for diabetes and hypertension in several relatives. Physical examination revealed diffuse abdominal tenderness. Respiratory and cardiovascular examinations were within normal limits. There were no skin lesions. Neurologic examination revealed no focal neurological deficits. Vitals were notable for mild tachycardia to 105 beats per minute. Temperature was 37°C and blood pressure was 124/64 mmHg. The patient had no significant abnormal lab findings other than an elevated total protein of 9.4 g/dL (6.0–8.3 g/dL), elevated total bilirubin of 1.5 mg/dL (< 1.2 mg/dL), and high HIV viral load of greater than 100,000 copies/mL. Complete blood count, basic metabolic panel, and transaminases were normal at presentation.Contrast-enhanced computed tomography (CT) of the chest, abdomen, and pelvis Fig.  at initiDuring his stay, the patient was started on 1 elvitegravir-cobicistat-emtricitabine-tenofovir tablet daily for HIV, melatonin 5 mg tablet nightly as a sleep aid, acetaminophen 650 mg tablet every 4 hours for pain, and ondansetron 4 mg tablet every 8 hours for nausea as needed. His antiretroviral medication was continued at discharge.After multidisciplinary review, chemotherapy and radiation were not recommended. Because of extensive vascular encasement, the tumor was initially deemed to be unresectable; however, after deliberation, the decision was made to attempt resection as it was the only viable therapy. Three months after presentation, colorectal, vascular, and hepatobiliary surgery performed an exploratory laparoscopy and eventual resection of the mass, along with a partial gastric resection, partial pancreatic resection, right adrenalectomy, periportal lymphadenectomy, and splenectomy. The tumor was indistinguishable from the adrenal gland, which was its likely origin. Histology of the resected lymph nodes showed metastatic ganglioneuroma, which was determined to be clinically insignificant because of favorable histology Fig. . ImmediaIn the years following surgery, the patient experienced chronic diarrhea and frequent lower abdominal pain without a discernible cause despite extensive gastrointestinal workup.Here, we presented the case of a retroperitoneal ganglioneuroma with lymph node involvement in an adult patient with HIV. Ganglioneuromas generally affect young children and are typically singular in nature. However, our patient was already 27 years old at the time of presentation, approximately three times the typical mean age at presentation of 7 years , 4. In aMost ganglioneuromas are discovered incidentally and are typically located in the posterior mediastinum, retroperitoneum, or adrenal glands , 7. WhenThe patient was HIV positive, which may have influenced his presentation. HIV patients are well known to be at increased risk of cancer due to oncogenic viral infection such as Kaposi’s sarcoma, non-Hodgkin lymphoma, and cervical cancer , 9. AddiWhile not specific, imaging provides a valuable tool in the workup of patients with ganglioneuromas. Imaging descriptions of ganglioneuromas are largely based on studies that include mostly children. On contrast-enhanced CT, ganglioneuromas typically appear as a homogeneous low attenuation mass with progressive but incomplete enhancement , 11. CalDiagnosis of ganglioneuroma requires analysis of tissue samples by excisional biopsy, core needle biopsy, or fine-needle aspiration . Of the Our patient was found to have metastatic ganglioneuroma in adjacent lymph nodes. This is a rare finding, as ganglioneuromas do not typically present with nodal spread beyond the primary mass. The most widely proposed cause for the presence of multiple tumors is independent development. In this theory, a malignant neuroblastic tumor (either neuroblastoma or ganglioneuroblastoma) is present in the patient first and metastasizes to distant locations. Once spread, the multifocal tumor may mature into a ganglioneuroma , 6. ThisWhile ganglioneuromas are benign, they may cause functional problems because of involvement of adjacent structures, necessitating surgical intervention to remove the mass. However, in cases like our patient’s, with extensive involvement of surrounding structures, complete resection may be contraindicated as damage to vital structures (neurovasculature) may cause significant postoperative morbidity such as bleeding, ischemia, or neurological dysfunction . In suchGanglioneuromas have excellent prognosis and are unlikely to cause tumor-related death . Rates oRetroperitoneal ganglioneuromas occur most often in children but may rarely occur in adults. While generally limited to a solitary tumor, ganglioneuroma may occasionally have metastatic spread to local lymph nodes, as demonstrated by this case. The patient in this case was HIV positive; however, it is unclear if this is a contributing factor to this disease. Increased awareness of this rare presentation will improve patient care.