Double aortic arch (DAA) is a congenital anomaly of the aortic arch. It is the most common type of complete vascular ring. When it occurs, the connected segment of the aortic arch and its branches encircle the trachea and esophagus, leading to symptoms related to these two structures.We present a case of a newborn baby who developed biphasic stridor immediately after a normal vaginal delivery. Endoscopic assessment of the trachea revealed a pulsatile narrowing at the level of the thoracic trachea, suggestive of an external compression. A contrast-enhanced computed tomography scan of the thorax with three-dimensional reconstruction confirmed the diagnosis of DAA with compression of the trachea and esophagus.Clinicians should strongly consider the possibility of a congenital vascular ring compression should an infant with a normal upper airway present with stridor. A precise diagnosis can be made by radiological examination. Double aortic arch (DAA) is the most common type of complete vascular ring that encircles the trachea and esophagus completely by the connected segment of the aortic arch and its branches, often rA 31-year-old woman gave birth to a full-term baby girl weighing 3.6 kg via spontaneous vaginal delivery at 40 weeks of gestation. The Apgar score was 9 after 1 minute; however, the child was noted to have a loud biphasic stridor with subcostal recessions, which did not improve with positioning. The respiratory rate was 60 breaths per minute and the pulse oximeter oxygen saturation was 100% under a head box with oxygen (5L/min). Liquor was clear. A physical examination of the cardiorespiratory system revealed normal heart sounds with transmitted sounds heard on auscultation of the lung fields. The case was referred to the otorhinolaryngology team on day 1 of life after being transferred from a district hospital. A bedside flexible nasolaryngoscopy examination revealed normal glottic and supraglottic structures. A direct laryngoscopy with telebronchoscopy carried out on day 2 of life revealed a pulsatile narrowing of the tracheal lumen at the level of the thoracic trachea, suggestive of an external compression. An uncuffed endotracheal tube with an internal diameter of 3.0 mm was used to stent the narrowed segment to maintain airway patency. A contrast-enhanced computed tomography (CECT) of the thorax with a three-dimensional (3D) reconstruction confirmed a diagnosis of DAA, which was tightly encasing the trachea and esophagus ,2.Echocardiography did not show any associated intracardiac anomaly. Feeding was maintained via a nasogastric tube from day 1 of life. Although corrective surgery was scheduled at a cardiac center in Kuala Lumpur, the infant developed acute massive hematemesis on day 37 of life and succumbed to it prior to the surgery.Vascular rings are rare congenital vascular disorders that inevitably encircle the trachea and esophagus. DAA is the most common form of complete vascular ring malformation , and is Respiratory symptoms at birth should raise the suspicion of vascular ring compression, especially in patients with normal fiberoptic findings of the larynx and also those exhibiting signs and symptoms of laryngomalacia. Patients may present with stridor, persistent cough, wheezing, and recurrent respiratory infection during infancy . GastroiIn this case, the child presented with biphasic stridor at birth. Direct laryngoscopy with telebronchoscopy under general anesthesia revealed a pulsatile narrowing of the tracheal lumen at the level of the thoracic trachea. A CECT with 3D reconstruction revealed the anomalous construction of the aortic arch. As in this case, CECT is one of the imaging techniques of choice for obtaining a diagnosis and when planning for surgical intervention. Early surgical intervention, by dividing the arches and freeing the trachea and esophagus from the surrounding tissue, is of the utmost importance to prevent late complications, such as tracheomalacia or aortoesophageal fistula ,5. The rAs the child developed sudden massive hematemesis, the possibility of an aortoesophageal fistula cannot be ruled out in this case.In conclusion, clinicians should strongly consider the possibility of a congenital vascular ring compression should an infant with a normal upper airway present with stridor. A precise diagnosis can be made by radiological examination.


Heterotopia is defined as the presence of mature, histologically normal, tissue in unusual anatomic sites. When this heterotopic tissue forms a mass, it is called a choristoma. This case series describes 3 cases of gastroesophageal junction (GEJ) salivary heterotopias. While heterotopias are usually incidental findings, choristomas can clinically and endoscopically mimic carcinomas and might lead to unnecessary procedures for the patients. Clinicians should therefore be aware of this entity. Literature search, however, failed to show any reports of salivary gland heterotopias in the GEJ. In fact, literature review revealed only 6 reported cases of salivary gland choristoma in the gastrointestinal tract, none at the GEJ. In this case series, we report 2 cases of salivary gland heterotopia and one case of salivary gland choristoma arising at the GE junction. To our knowledge, this is the first series of its kind in the literature. Heterotopia is defined as the presence of mature, histologically normal, tissue found in unusual anatomic sites. A mass formed by heterotopic tissue is called a choristoma. Salivary gland choristoma was recognized as early as 1967 by Youngs and Scofield in lower neck , periparDespite common occurrence in the head and neck, reports of salivary gland heterotopia or choristoma in the gastrointestinal tract are scarce. Salivary gland choristoma found in conjunction with gastric heterotopia was first described by Shindo et al. in 1972 . Our litA 44-year-old Caucasian male presented with an 8-month history of reflux and heart burn, which was relieved by Esomeprazole. Endoscopic evaluation showed a polypoid shaped mass measuring 1 cm in size at the gastroesophageal junction. Ultrasonic evaluation revealed a hypoechoic lesion that was confined to the deep mucosa and submucosa with no deeper layer involvement. The nodule was resected via the endoscopic mucosal resection technique (EMR). Grossly, the specimen was a 1 cm GEJ nodule. It was a single irregular fragment of tan-pink soft tissue that was bisected and entirely processed for microscopy. Microscopic evaluation showed squamous mucosa with oxyntic-type mucosa with moderate chronic inflammation, ectatic vessels, congestion in the mucosa, and a few lymphoid aggregates. In addition, there were prominent mucus glands with chronic inflammation consistent with heterotopic salivary glands, surrounded by prominent adipose tissue and congested vessels in the submucosa Figures .A 62-year-old Caucasian male with a history of hypothyroidism, Sjogren's syndrome, Raynaud syndrome, chronic GERD, and grade 3 esophagitis presented for an upper endoscopy for the evaluation of suspected Barrett's esophagus. Endoscopic evaluation and barium swallow showed a dilated esophagus. The z-line appeared slightly irregular and there were 2 small islands of salmon colored mucosal lesions immediately proximal to the GE junction.Grossly, the specimen consisted of one piece of tan-white soft tissue, measuring < 1cm. Microscopic evaluation showed fragments of squamous mucosa with focal mild acute erosive esophagitis and basal hyperplasia that was consistent with reflux. In addition, detached fragments of salivary gland type glandular tissue with chronic inflammation, consistent with heterotopic salivary gland tissue, were seen Figures  and 4.A 72-year-old Caucasian male with past medical history of Barrett's esophagus and high-grade dysplasia presented for EGD and biopsy in order to rule out invasive carcinoma. Endoscopic evaluation revealed a large hiatal hernia and an approximate 3 cm segment of Barrett's esophagus. There was a concerning focal area of ulceration which was removed via EMR.Grossly, the specimen was one piece of tan-white soft tissue, measuring 0.7 x 0.4 x 0.2 cm. Microscopic evaluation revealed focal high-grade dysplasia adjacent to squamous epithelium in the middle portion of the specimen. In the deeper aspect of the tissue, there were cavernous and ectatic venous channels as well as a few prominent lobules of minor salivary glands with cystification in the ducts Figures  and 6. TSalivary gland heterotopia in the GI tract is a rare entity. The heterotopic tissue can form a mass, choristoma, which can present as a mass on imaging or as a hypoechoic mucosal or submucosal lesion on EUS, mimicking the presentation of carcinoma. Other possible differential diagnosis includes gastrointestinal stromal tumor (GIST), schwannoma, leiomyoma, ectopic pancreas, cyst, lipoma, and carcinoid. This review will focus on the clinical features, endoscopic/sigmoidoscopic findings, histopathology, ancillary studies, pathogenesis, treatment, and prognosis of salivary gland heterotopias/choristoma based on the 6 cases published in the literature  with theSalivary gland choristoma in gastrointestinal tract occurs mainly in adults but has been identified in an age range from 5 to 61 years. It occurs more often in males than it does in females, with 7 cases (78%) occurring in males. The race of the patients was not reported in most cases. It can occur in any location in the GI tract with reports of occurrence in the rectal area, rectal diverticulum, jejunum, esophagus, and, now, gastroesophageal junction. The clinical presentation depends on the location. Rectal cases can present as a rectal protrusion/mass, rectal bleeding, anal pain, and sphincter spasm. Cases in the GE junction either present with chronic reflux or are asymptomatic. One case presented in the esophagus with symptoms of belching, regurgitation, and abdominal pain. The youngest reported case was a 5-year-old girl who presented with severe, intermittent abdominal pain and postprandial vomiting due to a salivary choristoma in the jejunum.The gross appearance is usually that of a single, tan-pink mucosal to subserosal nodule or pedunculated mass, ranging from 1 to 2 cm in size. Microscopically, mixed serous and mucinous glands with salivary ducts resembling submaxillary or sublingual salivary glands are seen.All the reported cases were treated with either sigmoidoscopic or endoscopic resection, except for the case of the 5-year-old Nigerian girl who underwent exploratory laparotomy and wedge resection due to the unavailability of endoscopic procedures. There were no reports of complications or recurrences. However, the follow-up intervals varied. There was only one report of malignant transformation in the GI. However, this phenomenon has been described in multiple cases of salivary gland heterotopias in the head and neck.Salivary gland choristoma in the GI tract can form a mass, which can present as a mass on imaging or as a hypoechoic mucosal or submucosal lesion on EUS, mimicking the presentation of a carcinoma. The authors do not suggest that it should be on top of the differential list, however, clinicians should be aware of its occurrence to prevent delay in diagnosis and inappropriate procedures and treatments for the patient.


We illustrate the case of a brachial pseudoaneurysm in a 32-week preterm baby male who presented with a large pulsatile mass in the right antecubital fossa, with no clear aetiologic factor. The management of this type of lesion has been controversial and based mainly on case reports. In this case, after obtaining partial response with two thrombin injections, it spontaneously regressed during expectant management. This combination of therapeutic options may be an alternative for the management of complex lesions and, to the best of our knowledge, has not been previously reported. Endovascular management was not considered owing to the small size of the parent artery (less than 2 mm) and its critical role in supplying blood to the forearm.A 32-week preterm twin baby male was referred to us at 7 weeks of age (3 kg) with a large, readily palpable pulsatile mass in the upper portion of the right antecubital fossa, which was increasing since day 14 of life . He had Informed consent was obtained and the patient was anaesthetized. Two attempts at ultrasound-guided compression using a 12 Mhz linear probe with a large foot piece placed over the neck of the PSA for 10 min at each attempt were unsuccessful. Under sterile conditions and using sonographic guidance, 5 units of thrombin were injected with a 25-gauge needle into the sac of the PSA,  obtainin1 They can be secondary to different aetiologies, including iatrogenic causes.1 They can present as a pulsatile mass, and management by interventional radiology has replaced surgery as the first line of treatment.2PSAs are common vascular abnormalities secondary to a disruption in arterial wall continuity.3 It has been associated with trauma to the artery during venipuctures.4 Its management is controversial,5 and based on case reports, surgery is the most commonly used treatment option.4 The surgical approach requires resection of the lesion with vascular grafting, end-to-end anastomosis or repair of the donor artery,4 and is associated with a very good success rate, which has improved with the continuous advancements in microsurgical techniques.Brachial artery PSA is a rare condition in neonates.6 Pezzullo and Wallach5 successfully treated a brachial PSA in a 3-week-old patient with 300 units of thrombin.Ultrasound-guided thrombin injection for the treatment of superficial PSA had been widely used with a success rate that ranges from 91% to 100%.2In our case, surgery was not initially considered owing to the age and weight of the patient. Technically, it did not appear possible to use an endovascular approach owing to the size of the parent artery. Therefore, our option was initially to attempt ultrasound-guided thrombin injection. While performing the first injection, we noted quick thrombosis with significant hardening of the lesion. We believe that this hardening increased the pressure in the compartment of the arm, with secondary compression of the brachial artery and decreased flow in the distal arm. This necessitated heparinization and use of topical nitroglycerin. With the second injection, we only obtained a partial response, probably secondary to the wide and short neck and the associated high intralesional blood flow. Expectant management was undertaken, waiting for the patient to gain weight for a surgical repair. However, the lesion resolved without any further interventions. It is matter of speculation whether or not the occlusion may have been assisted by the partial intralesional thrombosis obtained with the thrombin injections. Expectant management has been mentioned as a therapeutic option; however, it is rarely recommended.In conclusion, this case illustrates the use of a combination of ultrasound-guided thrombin injection and expectant management in managing a technically challenging PSA from interventional radiology (neck characteristics and size of the parent artery) and surgical  perspectives.Brachial PSA in neonates is an uncommon condition, which has been associated with trauma to the artery during venipuncture.Its management can be challenging, especially if the PSA neck is wide.As treatment may risk the arterial supply to the arm, a multidisciplinary approach, on a case-to-case basis, is recommended.Informed consent was obtained from the parents of the patient for publication of this case report and unidentified pictures. This was recorded in the patient’s chart as per our hospital policy.


A spontaneous mesenteric hematoma is rare, and its etiology is unclear. We present a very rare case of spontaneous mesenteric hematoma with a fistula to the transverse colon.A 90-year-old male visited our hospital because of lower abdominal pain. The physical examination revealed tenderness in the lower abdomen. The laboratory data revealed anemia and low estimated glomerular filtration rate (eGFR). The abdominal contrast-enhanced computerized tomography (CT) indicated a mass with coexisting low- and high-density areas adjacent to the transverse colon. The mass was diagnosed as a mesenteric hematoma. We selected conservative therapy, because the patient’s condition was stable. After 15 days from discharge, he was admitted to the hospital again for the complaint of bloody stool. A fistula with blood flowing out at the splenic flexure was reported on colonoscopy. In the laparoscopic operative findings, the hematoma adhered to the stomach, pancreas and omentum. A hand-assisted laparoscopic transverse colectomy including hematoma in the mesentery was successful. The pathological reports revealed that a spontaneous mesenteric hematoma formed the fistula to the colon. The patient was discharged without any postoperative complication on day 10.Generally, laparotomy is often chosen for the treatment of mesenteric hematoma. The laparoscopic approach for mesenteric hematoma is useful under the specific condition that the preoperative patient’s condition is stable.This is the first case of a spontaneous mesenteric hematoma with a fistula to the colon resected safely by laparoscopic surgery. An angiography is also useful for the diagnosis of the origin of the bleeding point. A spontaneous mesenteric hematoma can be treated conservatively, if the hemorrhage can be controlled .A spontaneous mesenteric hematoma is a rare condition, and its detailed etiology is unknown . In this22). All the other data were within the normal range. The abdominal contrast-enhance CT indicated a mass with coexisting low- and high-density areas with a maximum diameter of 120 mm adjacent to the stomach and transverse colon  (hemoglobin level: 10.3 g/dL; eGFR: 35 mL/min/1.73mse colon a,b. An ese colon c,d. The se colon . We diagse colon a,b. Therse colon a,b. The se colon . Histopase colon a-c. Ther3The spontaneous mesenteric hematoma is a rare condition of unknown etiology, and it was first described in 1909 . The maiA mesenteric hematoma presents with non-specific symptoms, such as abdominal pain, vomiting, abdominal masses, diarrhea, and melena. Therefore, it is usually identified by an abdominal contrast-enhanced CT, ultrasound, or MRI . The diaThe treatment of a mesenteric hematoma depends on the clinical stability of the patients. The patients whose vital signs are unstable or those who do not respond to fluid resuscitation should be given an emergency operation. On the other hand, the patients in stable condition after the resuscitation can be treated with the non-operative methods . In thisWe could not find any previous reports on a spontaneous mesenteric hematoma resected by a laparoscopic surgery. This is the first report on the laparoscopic resection of a spontaneous transverse mesenteric hematoma with a fistula.4We experienced a rare case of mesenteric hematoma with a fistula to the transverse colon. A laparoscopic surgery is useful in the cases where the patients are clinically stable.None of the authors have any commercial or financial involvement in connection with this study that represents or appears to represent any conflicts of interest.This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.Ethical approval from our institution OJH-201854.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.All authors in this manuscript contributed to the interpretation of data, and drafting and writing of this manuscript. Tomoaki Bekki is first author of this paper. Takuya Yano is corresponding author of this paper. Tomoaki Bekki, Takuya Yano and Masahiro Nakahara conceived and designed the study and drafted the manuscript. Tomoaki Bekki first diagnosed. Tomoaki Bekki, Takuya Yano and Masahiro Nakahara were engaged in patient’s care in our hospital including surgery.Hiroyuki Egi contributed to study concept, and review of the final manuscript and submission of the paper. All the authors read and approved the final manuscript.The manuscript does not report the result of an experimental investigation or research on human subjects.Takuya Yano.Not commissioned, externally peer-reviewed.


A 76-year-old woman who experienced sinusitis for years was presented to the emergency department complaining of left orbital pain, eyelid swelling, and reduced visual acuity. She was nonfebrile, and she reported no recent trauma nor any violent Valsalva maneuver.Ophthalmological examination showed a proptosis and a limitation of eye movements, particularly in the up gaze Figure  as well Non-contrast computed tomography (NCCT) revealed a biconvex well-defined 41 × 31 × 17 mm collection with layering hyperdense blood products in the roof of the orbit, displacing superior oblique and rectus muscles, causing exophthalmia and deformity of the eye. No obvious bone erosion was visualized  and isointense to hyperintense signal on T1-weighted images  indicated an acute to subacute hematoma with hypointense signal on T2/FLAIR-weighted images . The visual field showed an altitudinal inferior defect with a macular sparing.Orbital MRI control performed one month postoperatively was unremarkable.Most cases of orbital subperiosteal hematoma (OSH) typically result from the rupture of blood vessels in the setting of trauma. Non-traumatic OSH is uncommon and typically associated with a sudden increase in cranial venous pressure, as in emesis, bleeding diathesis or chronic sinusitis . OSH ariBecause the possibility of orbital infection, surgical drainage is recommended. An anterior orbital approach is preferred. Early detections and treatment are necessary to prevent compressive optic neuropathy and to ensure the best prognosis. Antibiotic coverage is recommended.While computed tomography performs well to show sinusitis, possible bone changes, and the presence of blood collection or abscess, an MRI is superior for evaluating intraorbital structures, especially the optic nerve.


The surgical strategy for congenital perineal lipoma varies depending on the size, location, and accompanying congenital anomalies, with the optimum approach remaining to be determined. We herein report a case of congenital perianal lipoma that was first detected by prenatal ultrasound and review the literature.A female neonate was referred to us for the evaluation of a perianal mass. She had been considered to be male prenatally because fetal ultrasound showed a perineal mass similar to a scrotum and penis. A postnatal examination revealed an appropriate-for-age neonate with a soft round mass 1.5 cm in diameter just to the left of the anal verge. She passed urine and stool smoothly, and contrast enema confirmed no anorectal malformation. Magnetic resonance imaging showed that the lesion had a signal intensity consistent with fat located close to the anal sphincter, and no spinal anomaly  was identified. We excised the lesion  simply at 2 months old, taking care to avoid damaging the anal sphincter by using a muscle stimulator. She has been doing well with good bowel movement and satisfactory cosmetic results for a follow-up period of one and a half years.Our literature search revealed 49 cases of perineal lipoma reported in English in the last 25 years, and 74% of them—including ours—had other congenital anomalies, the breakdown of which was anorectal malformation in 40% of cases, labioscrotal fold or accessory scrotum in 28%, and urogenital malformation, congenital pulmonary airway malformation, and disorder of sex differentiation. The prenatal detection of the lesion, as in our case, was quite rare.A thorough physical examination after birth, magnetic resonance imaging and contrast enema to identify the nature of the perineal lipoma and accompanying anomalies are crucial for planning the surgical strategy. The lesion may be deeply interspersed between the sphincter muscle, especially when it accompanies anorectal anomaly. A muscle stimulator is useful for preserving and repairing the sphincter muscles during resection in order to ensure satisfactory bowel movement. Congenital perineal lipoma, including perianal lesions, has been reported in about 50 cases in English literatures –26. Its We herein report a case of congenital perianal lipoma that was first detected by prenatal ultrasound. The prenatal detection or diagnosis of such lesions is quite rare , 23, 25.A female neonate was referred for the evaluation of a perianal mass. She was delivered at 39 weeks and 1 day of gestation weighing 3097 g. The Apgar score was 9 at both 1 and 5 min after birth. She had been considered male prenatally because fetal ultrasound showed a perineal mass similar to male genitalia at a gestational age of 20 weeks and 4 days (Fig. A histologic examination of the specimen showed mature adipose tissue interspersed with collagenous bands, leading to a diagnosis of lipoma. She was discharged from the hospital without any complication and has been doing well with good bowel movement and satisfactory cosmetic results for a follow-up period of one and a half years.We conducted a literature search using PubMed with keywords of lipoma, perianal, perineal, and neonate, identifying 49 cases of this lesion reported in English since 1994 . The cliThe differential diagnoses of perineal lesions other than lipoma include lipoblastoma, sacrococcygeal teratoma, infantile hemangioma, hamartoma, choristoma, liposarcoma, enterogenous cyst, and ambiguous genitalia , 15. If Postnatally, a careful physical examination for its precise location, size, and accompanying anomalies is crucial to achieving a correct diagnosis. These lesions are typically lobulated, round, or pedunculated subcutaneous masses that are smooth, soft, mobile, and nontender . PostnatOn the other hand, most reported lesions accompanying anorectal anomalies have been resected at the time of anorectal reconstruction , 14. OneWe herein report a rare case with prenatally detected perianal lipoma that neonatologists and pediatricians should note. Surgical consultation should be sought, and thorough perinatal investigations of the lesion and associated anomalies are crucial for planning surgery. An electrical muscle stimulator is useful for preserving the anal sphincter during resection to achieve satisfactory bowel movement.


A 42-year-old female with unremarkable medical history presented for a routine cervical screening upon which an endocervical polyp was identified and submitted entirely for histopathologic evaluation. Microscopic examination showed multiple well-circumscribed nodular fragments of polyp with superficial erosion and focal acute inflammation. Benign endocervical glands were seen within a fibrotic stroma with a prominent smooth muscle component. Intermixed mature adipose tissue and large thick-walled vessels were also identified. Stromal and epithelial atypia were absent; similarly, stromal cellularity, mitoses, and condensation were not identified. Additional deeper levels did not reveal other heterologous elements. The diagnosis of a benign choristomatous endocervical polyp was rendered. As anticipated, the patient recovered completely.A review of the English literature demonstrates rare, namely two other reports of hamartomatous tissue in an endocervical polyp. To the best of our knowledge, we report the third such case. Endocervical polyps (ECPs) are routine and common specimens in gynecologic pathology. Microscopically, these benign proliferations show a fibrovascular stalk and endocervical glands, and are on occasion accompanied by squamous metaplasia, chronic inflammation, or ulceration if irritated. Herein, we illustrate an unusual histopathologic presentation of an ECP in a 42-year-old female who presented for a routine well-woman visit.The patient has an otherwise unremarkable medical history. On physical examination and cervical cytology screenings, she was found to have no abnormalities. However, due to the presence of a high-risk human papilloma virus (HPV) on HPV co-testing, she underwent colposcopy during which a cervical polyp was visualized. Polypectomy was performed, and the specimen was sent to pathology.Grossly, a 2.5 x 1.9 x 1.0 cm nodular rubbery polyp was received, which was then trisected and submitted entirely for microscopic examination in three blocks. Four micron-thick sections of formalin-fixed paraffin-embedded tissue were prepared with routine hematoxylin and eosin staining protocol.Microscopic examination demonstrated fragments of nodular, well-circumscribed cervical tissue with smooth outer surface. The superficial endocervical epithelium was morphologically benign with focal erosion accompanied by a patchy superficial mixed inflammatory infiltrate Figure , 1B. TheNo stromal atypia or stromal mitoses were identified. Additional deeper sections were examined on each of the three blocks but other heterologous stromal elements were not identified. The diagnosis of benign ECP with hamartomatous elements was rendered.ECP are thought to represent a benign hyperplastic phenomenon of both the epithelium and underlying cervical stroma. ECP are an anticipated routine specimen in the daily selection of gynecologic pathology, and microscopically often show a loose, edematous stroma, large dilated or small thick-walled vasculature. A fair number of unremarkable endocervical glands are also normally identified. Depending on the extent of irritation, particularly in larger lesions protruding through the cervical os, a certain amount of acute and chronic inflammation, erosion, or even granulation tissue usually localized to the surface is anticipated.ECP with benign heterologous elements are extraordinarily rare. After a review of the English literature, two case reports, both describing ECP with mature adipose tissue and abnormal thick-walled vessels, were identified ,2. IlhanAs it is such an extraordinary event, the presence of heterotopia in the cervix is perplexing to most practicing pathologists. As non-pathological or mass-forming heterotopic tissue, fat and cartilage have previously been described at this site and despite encountering major challenges in accurately identifying adipocytes in the cervix, some studies claim fat can be seen in 15% of cone/loop electrosurgical excision procedures or hysterectomies . EncapsuThe origin of heterotopia is obscure, some speculating it to arise either from metaplasia of Wolffian duct remnants or from chondroid differentiation of the uterine stroma . AdditioThe identification of choristomatous ECP with the unusual and rare finding of adipocytes, cartilage, and abnormal large vessels within the stroma can be worrisome to the practicing pathologist. When obvious endocervical glands are not identified within the polyp, as illustrated by Pecorella, or mesonephric glands are noted, as per Ilhan ,2, the dWe report the case of a choristomatous ECP in a 42-year-old healthy female, which to the best of our knowledge, is the third such case in the English literature. The practicing pathologist and gynecologist should be aware that the presence of benign heterologous elements in an ECP albeit curious and quite rare is indeed possible. Comfort in a benign diagnosis can be found after complete histopathologic examination of the polyp, as is the protocol recommended for endometrial polyps, and most importantly after more common malignant mimickers such as carcinosarcoma and adenosarcoma are excluded.


Liposarcoma is a soft tissue sarcoma of adipocyte origin. Liposarcoma represents 20–30% of adult soft tissue tumors, which was most frequently seen in the retroperitoneal space in 45% and abdominal space in only 5% of cases, but the multicentric case is unknown. Herein, we describe a rare case of multicentric, large, intra-abdominal and retroperitoneal liposarcoma, one of which had caused infection and pressing the right ureter causing hydronephrosis, which was resected by two-stage surgery.The patient was a 46-year-old man who was referred for abdominal bloating and fatigue. Enhanced computed tomography showed a 23-cm intra-abdominal tumor and a 14.6-cm left retroperitoneal tumor. The intra-abdominal tumor which compressed the right ureter caused right unilateral hydronephrosis and deteriorated the renal function. The intra-abdominal tumor had also formed an intra-abdominal abscess. We performed emergent laparotomy and resected the intra-abdominal tumor. After the recovery of renal function, we resected the residual retroperitoneal tumor. Histopathological examination showed both tumors to be myxoid/round cell type liposarcoma. Considering clinical findings and their location, he was diagnosed with multicentric liposarcoma. He underwent adjuvant chemotherapy and has been alive without any recurrence for 9 months after the operation.We successfully resected large intra-abdominal and retroperitoneal multicentric myxoid/round cell liposarcomas. A two-stage surgery was a rational choice as it provides time to confirm the recovery of renal function. Liposarcoma represents 20–30% of adult soft tissue tumors, which is most frequently seen in the retroperitoneal space in 45% and in the abdominal space only in 5% of cases, but multicentric cases are unknown . It is oA 46-year-old man complained of heartburn, abdominal distention, and anorexia which persisted for over 2 weeks. He had no significant personal and family history. Body temperature on admission was 39.2 °C. Abdominal physical examination revealed large abdominal masses without tenderness or rebound. Blood examinations showed increased inflammation reaction . Renal dysfunction was also recognized Table . AbdominWe thought that the intra-abdominal tumor had formed abscess and caused bacterial infection. We decided to resect the right intra-abdominal tumor first to stabilize the general condition from a systemic inflammatory state. After the recovery of renal function, the left retroperitoneal tumor resection was scheduled.2) and ifosfamide (2 g/m2) for eight courses for 6 months. He has been closely monitored and recurrence-free for 9 months after the first surgery.In the first surgery, we inserted a ureteral stent to the right ureter and successfully performed resection of the right tumor without invasion to the right ureter. However, the tumor appeared to invade the small intestine, and combined resection was performed Fig. a. The opLiposarcoma is the most frequent soft-tissue sarcomas. It is usually seen in retroperitoneal 45%), extremities 24%), inguinal, gluteal, and popliteal regions, but it is rarely seen in the intra-abdominal space . Patient4%, ingui5%, extreLiposarcoma is usually a single tumor. When multiple tumors are seen, one of the main problems is to differentiate multicentric lesions from metastases of a single tumor. Multicentric liposarcoma accounts for only 1–2% of all liposarcomas in the USA and Europe . There aThe treatment for liposarcoma has not been established, but en bloc resection is recommended not only for treatment but for pathological diagnosis . Our casIn this case, multicentric tumors were composed of multiple compartments. Therefore, we thought that his prognosis would be poor and he should undergo chemotherapy in the adjuvant settings. The effectiveness of adjuvant chemotherapy remains unproven, and proper regimen has not been established.2) and ifosfamide (2 g/m2) for five cycles, and there has been no recurrence for 9 months. In our case of multicentric myxoid/round cell liposarcoma, en bloc surgical resection and adjuvant chemotherapy were effective.Tumor size (> 20 cm), histological subtypes, and tumor dissemination are considered important prognostic factors . In thisWe successfully resected large, intra-abdominal, and retroperitoneal multicentric myxoid/round cell liposarcomas. A two-stage surgery was a rational choice as it provides time to confirm the recovery of kidney function.


TP53 mutations. Most recent guidelines now recommend annual whole-body MRI for screening for cancer patients carrying germline TP53. With this report, we aim to share our experience with this rare situation in order to improve care about these specific cases.Li-Fraumeni syndrome is a rare inherited disease characterized by the early onset of multiple primary malignant tumors. Sarcomas account for more than 30% of all malignant tumors occurring at pediatric age. Furthermore, it was shown that the rates of second cancer were higher in childhood cancer survivors. We report the case of a patient with Li-Fraumeni syndrome who was referred to us with three synchronous skeletal tumors. This unique situation led to difficulties for the medical team regarding the diagnosis of malignancy and the surgical treatment to propose. The discovery of multiple lesions in the extension assessment underlines the usefulness of whole-body imaging for the follow-up of patients with germline  Li-Fraumeni syndrome (LFS) is a rare inherited disease characterized by the early onset of multiple primary malignant tumors. Li and Fraumeni described it in 1969 . New criTP53 in LFS families [TP53 was found in 77% of families with LFS [TP53 mutations contribute to a high risk of developing multiple cancers. In a report from the French group, more than 40% of 322 mutation carriers developed multiple tumors, either synchronous or metachronous [In 1990, Malkin et al. described the role of the germline mutation of the tumor suppressor gene families . Later, chronous , 9.We present the case of an LFS patient referred to us with three synchronous skeletal tumors. We will discuss the clinical presentation at diagnosis, the treatment, and the outcome at last follow-up.TP53 mutation c673-2A>G at the splice acceptor site of intron 6.A seventeen-year-old girl was referred for subacute foot pain. In her past medical history, she presented with rhabdomyosarcoma of the left thoracic wall at the age of 8 months treated by surgery and chemotherapy. In her family medical history, her mother developed breast cancer and a leiomyosarcoma diagnosed at the age of 45, two of her maternal aunts in her mother's side were diagnosed for breast cancer, and one of her first-degree cousins with a rhabdomyosarcoma. The genetic analysis found a Her recent history was marked by the occurrence of a continuous pain on the medial aspect of her right foot. Plain radiographs of the ankle and the foot revealed an ossification of the calcaneus . The preThe initial course of treatment of the high-grade osteosarcoma of the right calcaneus was chemotherapy, and then, the multidisciplinary team proposed a conservative surgery. A resection of the right calcaneus with a reconstruction by iliac graft along with a triple arthrodesis of the hindfoot was performed Figures . The hisThe two lesions identified on the PET scan were followed up. CT and MRI performed 7 months after the end of chemotherapy showed only a thickening of the femur tumor's cap while the aspect of the sacroiliac lesion stayed stable Figures . In addiAt the last follow-up of eight years, no recurrence had occurred, and the patient was pain-free with an unlimited walking capacity. She was submitted then to annual screening including whole-body MRI and was diagnosed with 3 new tumors .TP53 mutation (3.8%) or a rare exonic TP53 variant (5.7%) whereas none were identified in patients aged 30 years and older [Osteosarcomas are malignant tumors regularly reported among LFS patients . It has nd older . Few casnd older , 12. HowTP53 mutations. Because of the broad spectrum of cancer associated with germline TP53 mutations, cancer screening is challenging in this context. Since a few years, several reports have shown that whole-body MRI (WBMRI) allowing to detect cancers while still asymptomatic was efficient in the context of Li-Fraumeni syndrome. Indeed, this technique has been proven feasible, and several research protocols have demonstrated the efficacy of protocols including this procedure [The discovery of two additional lesions in the extension assessment underlines the need for whole-body imaging in patients with germline rocedure , 14. A mrocedure . A substrocedure .TP53 mutation presents a high risk to developing cancer—cumulative incidence of cancer of 50% by age 31 in females and by age 46 in males and nearly 100% in both sexes at age 70—and because the usual criteria used to differentiate benign and malignant tumors are based on a low level of certitude, every tumor should be suspected to be malignant until proven otherwise in a patient with a germline TP53 mutation [In the case of our patient, the two additional lesions presented several criteria of benignity: no pain, no evolution during the follow-up, no mass in the soft tissues, no cortical lysis, and a very low hyperfixation in both bone scintigraphy and PET scan. Only one pejorative aspect was noted: the axial localization in the pelvis and proximal femur . Howevermutation , 17, 18.For this patient, the rigorous follow-up allowed a curative treatment. Prognosis of osteosarcoma was dramatically improved by the introduction of chemotherapy and achieved 60-70% success compared to 10-20% with surgery alone. Bacci et al. studied the prognosis of synchronous multifocal osteosarcoma accounting for 3.6% of all patients with osteosarcoma treated in their institution (43/1154 patients). Only 16 patients were eligible for surgical treatment of all the tumors, 15 had complete remission, but only three remained free of disease after 5 years. This study showed the poor prognosis of this type of tumor. Furthermore, they suggested that they were bone-to-bone metastases of a single tumor because the response to chemotherapy was concordant in 13 of 16 cases . In contTP53 have been found in a minority of chondrosarcoma, and when present, it seems to be associated with a higher grade of tumor [TP53 mutation.Finally, it is also possible that LFS could have favored the occurrence of a malignant tumor on a preexistent benign tumor. Chondrosarcoma is the second most common bone tumor after osteosarcoma, representing almost 20% of all bone tumors [of tumor . Secondaof tumor . Hence, TP53 mutations. Because of the broad spectrum of cancer associated with germline TP53 mutations, cancer screening is challenging in this context. Since a few years, several reports have shown that whole-body MRI allowing to detect cancers while still asymptomatic was efficient in the context of Li-Fraumeni syndrome. With this report, we aim to share our experience of this rare situation in order to improve care about these specific cases.The discovery of multiple lesions in the extension assessment raises the problem of the surveillance of patients with germline


Internal jugular vein ectasia is a condition in which there is a dilatation of the internal jugular vein. A patient usually presents in the first decade with swelling in the neck, which aggravates in size while straining or coughing. This is a very rare condition and the chances of misdiagnosis are quite high. It is diagnosed by proper history taking, examination, and radiological study.We reported the case of a seven-year-old female presenting with right-sided swelling in the neck aggravating in size while straining or coughing. Regular follow-up was advised. Swelling regression was observed after one year of follow-up without any surgical treatment. This is a self-limiting condition and usually the treatment is not warranted. Regular follow-up is advised for the patient.  Jugular vein ectasia means the dilatation of the jugular veins. It is commonly observed in children or young adults . InternaDifferential diagnosis includes laryngocele, cystic hygroma, branchial cyst, and superior mediastinal mass . These cHowever, there is limited data mentioned in the literature regarding the regression of size without any intervention. Radiological studies are necessary to confirm the diagnosis and for records so that serial monitoring can be performed and documented. Chest X-ray can eliminate the diagnosis of laryngocele or mediastinal tumor as there will be the absence of air or wide mediastinum in the films, respectively. Diagnosis is confirmed by ultrasound combined with Doppler flow imaging or spiral computed tomography (CT) scan with contrast. Colour Doppler as a cheap technique is the diagnostic modality of choice, especially in developing countries. The treatment protocol is conservative in the absence of any complications or deformities .SurgicalA seven-year-old girl presented in our OPD with right-sided neck swelling for four years, which increased while coughing or straining .It was insidious in the onset and progressively increasing. There was no significant medical history, no history of trauma, pain, fever, cough, weight loss, difficulty in swallowing, breathing or any other site swelling. In examination, there was a nonpulsatile, nontender, nontransilluminant, nonpulsatile, soft, and cystic swelling that increased in size by coughing. Moreover, other intrathoracic pressure raising manoeuvres was situated anteromedially to the right sternocleidomastoid muscle with the size of 4×3 cm. Nasal endoscopy and Fiber-optic laryngoscopic examination of the child was normal.Chest X-ray was grossly normal. Colour Doppler ultrasound was performed and finally internal jugular phlebectasia was diagnosed. On ultrasound, preValsalva diameter of IJV was 8.4 mm, while postValsalva diameter was 24 mm . Parents were counseled and reassured and no surgical treatment was offered as the patient was asymptomatic. The regular follow-ups were also suggested to parents with the advice of review at the earliest time if there was any change in the size of swelling and development of any symptoms. After one year of follow-up, swelling was observed to be decreased  and a rePhlebectasia is a condition that means the dilatation of vein without tortuosity. When there is dilatation with tortuosity, it is referred to as “varix”. Phlebectasia of the jugular veins presents as a soft cystic swelling in the neck during straining. For the first time, internal jugular phlebectasia was described by Zukschwerdt . The IJVDiagnosis is usually made using colour Doppler ultrasound as it confirms vascular flow . ContrasColor Doppler ultrasound is the investigation modality of choice as it is cost-effective. It can be easily and effectively used for monitoring the swelling so that the size can be documented. Therefore, ultrasound is the best screening method. However, contrast-enhanced computed tomography gives the exact extent of the venous abnormality. It should be differentiated from laryngocele, superior mediastinal cysts or other nonpulsatile masses of the neck .Complications, such as thrombosis  and HornSurgery in such benign cases is usually reserved for complications. Surgical option includes the resection of dilated portion of vein or sheathing of the affected segment in a polytetrafluoroethylene tube graft (9). Jugular vein ligation is a too radical procedure for such a benign condition and definitely cannot be applied in cases with bilateral affliction . The IJV ectasia is a rare entity which presents in pediatric patients usually with the neck mass aggravating in size with maneuvers, such as coughing, straining or Valsalva. Imaging is essential for the diagnosis. Surveillance is the mainstay in most of the cases. It can regress spontaneously so it should be waited and watched. Surgery is suggested in the presence of complications or achieving cosmesis.


Granulomatous peritonitis represents a rare postoperative complication that should be considered as important differential diagnosis in all patients who present to the hospital with abdominal pain, abdominal tenderness, and fever after abdominal surgery. Clinical distinction from abdominal tuberculosis remains a diagnostic challenge and requires thorough histopathological and microbiological examination. Seven weeks before this presentation, an emergency laparotomy had been performed in the Ukraine because of spontaneous liver capsule rupture of a so far unknown liver tumor. During the diagnostic workup, a re‐laparotomy for evaluation of the liver tumor was performed. Intraoperatively, multiple abscesses and peritoneal purulent nodules were detected within the peritoneal cavity. Frozen section analysis of taken biopsies revealed epithelioid cell granulomas with central caseous necrosis. Due to the clinical and histological suspicion of tuberculous peritonitis, the operation was terminated prematurely. Follow‐up analysis excluded abdominal tuberculosis using microbiological, histopathological, and molecular biological methods. However, subsequent extensive histological analysis revealed multiple phagocytosed cellulose fibers and other foreign material within the granulomas. Based on these results and the clinical course of the patient, a postoperative granulomatosis peritonitis induced by exposure to foreign material of the initial operation in the Ukraine was diagnosed.Granulomatous peritonitis represents a rare postoperative complication that may occur after extensive abdominal operations and is often associated with surgery performed under emergency conditions. In such a situation, granulomatous peritonitis can be caused by foreign material contamination and can be triggered, eg, by surgical glove powder, foreign particles, or undigested particles from accidental intestinal perforation or cotton lint from disposable surgical drapes and laparotomy pads.Clinical symptoms of postoperative granulomatous peritonitis are heterogeneous and vary from abdominal tenderness, intestinal obstruction, fever, ascites to mild and nonspecific abdominal pain.Abdominal tuberculosis, which is the sixth most frequent form of extra‐pulmonary tuberculosis, represents the most important differential diagnosis of postoperative granulomatous peritonitis.The clinical, macroscopic, and microscopic characteristics of both entities are very similar. In addition, tuberculous peritonitis is equally difficult to diagnose primarily due to its insidious onset, due to its variability in symptoms, and due to a lack of easily applicable diagnostic biomarkers. However, pathophysiology as well as treatment differs fundamentally.Based on these facts, this case report intends to highlight the important differences of both diseases in order to help clinicians to determine the right diagnosis and initiate the correct treatment.2A 40‐year‐old woman presented to the hospital with abdominal pain in the right upper quadrant right, flank pain, and fever. She denied dysuria, polyuria, haematuria, nausea, or vomiting.Seven weeks earlier, an emergency laparotomy had been performed in the Ukraine because of a spontaneous liver capsule rupture of a liver tumor, which was unknown before. During the emergency operation, multifocal disseminated liver lesions were detected. Taken biopsies, considering the circumstances of reduced quality, were analyzed histologically, and a hepatocellular carcinoma (HCC) was initially suspected.After she had an uneventful postoperative course, she was discharged from the hospital in the Ukraine. For re‐evaluation of the histological samples and oncological treatment, she traveled to Germany.A few days after she had arrived, she appeared to our emergency department, presenting the above‐mentioned symptoms.3At physical examination, she was awake, alert, and interacting appropriately. The cardiac examination was without pathological findings, and lungs were clear with breath sounds bilaterally. Abdominal examination revealed right upper quadrant abdominal tenderness and right flank tenderness.The vital signs were as follows: systolic/diastolic blood pressure, 139/102 mm Hg: heart rate, 96 bpm; respiratory rate, 22 breaths/min; oxygen saturation (SpO2), 98% on room air; and body temperature, 38.2°C.Laboratory tests revealed increased C‐reactive protein (CRP) level of 46.9 mg/dL , a normocytic hypochromic anemia with a hemoglobin level of 9.8 g/dL , hepatocellular injury with slightly elevated aspartate‐aminotransferase (AST) level of 40 U/L , cholestasis with moderately increased alkaline phosphatase (AP) level of 165 U/L  and moderately elevated gamma‐glutamyl transferase (GGT) level of 101 U/L  as well as markedly increased levels of lactate dehydrogenase (LDH) of 751 U/L . Urine analysis revealed no features of urinary tract infection.Abdominal ultrasound revealed a large, solid, exophytic tumor with irregular margins, mixed echogenicity occupying the main part of the right liver lobe and displacing the right kidney.Abdominal computed tomography (CT) with contrast confirmed the ultrasound results and showed an inhomogeneous, large, nonenhanced hypodense lesion measuring 13.6 × 11.6 × 20 cm, occupying most of the right liver with exophytic components encroaching the upper right suprarenal region and displacing the right kidney inferiorly. Furthermore, multiple enlarged abnormal para‐aortic and para‐caval lymph nodes were detected  was also negative. Because all diagnostic tests for M. tuberculosis from intestinal tissue samples were negative, the diagnosis of abdominal tuberculosis was discarded.However, during the following diagnostic workup, no acid‐fast bacilli were detected with Ziehl‐Neelsen staining of the intestinal tissue samples and polymerase chain reaction (PCR) targeting Extensive histological analysis of the tissue samples revealed an unexpected result.The taken biopsies of the subcutaneous tissue and the peritoneum showed focal fibrosis areas with local hemorrhages. Furthermore, epithelioid granulomas with central necrotic regions and scattered hemorrhages were identified. The granulomas were surrounded by a diffuse inflammatory cell infiltration, consisting of lymphocytes, neutrophils, plasma cells, macrophages, and some multinucleate giant cells. Surprisingly, some of the giant cells contained phagocytosed foreign material, which was not birefringent in polarized light. Further amorphous foreign material was detected near the central necrotic areas, without association to the giant cells. Finally, the foreign material was identified as cellulose plant fibers Figures  and 4.Thus, the detected lesions closely resembled tuberculous lesions but were shown on microscopy to be granulomata containing cellulose fibers.Based on these results, a postoperative granulomatosis peritonitis induced by contamination with foreign material during the initial operation in the Ukraine was diagnosed.4After exclusion of abdominal tuberculosis, the patient recovered quickly without medical intervention and there were no postoperative complications. To definitely diagnose the liver tumor which was seen in initial CT scans and the emergency operation in the Ukraine, a CT‐guided needle biopsy of one of the suspect liver lesions in the right liver lobe was performed.Histological analysis of this biopsy revealed adrenal cortical carcinoma cells. Based on this, the diagnosis of an adrenal cortical carcinoma with diffuse infiltration of the liver was established and the patient was discharged for chemotherapy in a specialized center.5postoperative granulomatous peritonitis.Granulomatous peritonitis is commonly associated with infectious, malignant, or idiopathic inflammatory conditions, including sarcoidosis, granulomatosis with polyangiitis, lymphoproliferative disorders, and tuberculosis. In rare cases, granulomatous peritonitis can be caused by foreign material contamination during surgery.The true extent of postoperative granulomatous peritonitis is unknown.Clinical symptoms of postoperative granulomatous peritonitis are highly variable and heterogeneous. Patients with granulomatous peritonitis may either present with abdominal pain, abdominal tenderness, ascites, ileus, abdominal distension, and lymphadenopathy or with unspecific signs such as fever and progressive malaise.Potential complications of postoperative granulomatous peritonitis are peritoneal adhesions with consecutive intestinal obstructions or intestinal fistula formation.The pathophysiology of postoperative granulomatous peritonitis remains poorly understood. It is assumed that accidental intraperitoneal introduction of foreign material  induces a delayed cell‐mediated hypersensitive inflammatory response with the formation of foreign body granulomas in susceptible individuals.Because of the variable clinical presentation, the diagnosis of postoperative granulomatous peritonitis represents a challenge for clinicians. In order to find the right diagnosis in due time, all patients, presenting to the hospital with unclear abdominal pain, abdominal tenderness, and fever, should be asked for abdominal surgery performed during the past weeks or months. Laboratory findings are often nonspecific. The blood samples could reveal leukocytosis with left shift and acidosis. Imaging tests, including a plain abdominal film, computerized tomography (CT), or an ultrasound examination, can be performed to detect perforations of the intestinal tract, ascites, abscess formation, or peritoneal thickening. If ascites fluid is detectable, a diagnostic paracentesis should be performed.At explorative laparoscopy or laparotomy, the peritoneum and omentum are mostly studded with multiple small nodules.Microscopic examination of the granulomas may reveal a wall of epithelioid histiocytes, enclosing either caseous material, masses of disintegrating nuclei, or foreign material particles like cellulose fiber material.The most important differential diagnosis to (foreign body) postoperative granulomatous peritonitis represents tuberculous peritonitis and peritoneal carcinomatosis.Tuberculous peritonitis is frequently confused with postoperative granulomatous peritonitis as many clinical and histological features are almost identical.Tuberculous peritonitis is equally difficult to diagnose primarily due to its insidious onset and variability in symptoms, and, like postoperative granulomatous peritonitis, tuberculous peritonitis may present with abdominal pain, abdominal tenderness, and fever.M. tuberculosis or other mycobacteria.On microscopy, tuberculous peritonitis is also characterized by epithelioid cell granulomas with central caseous necrosis.Risk groups for both diseases differ clearly.Etiologically postoperative granulomatous peritonitis typically occur in patients with a medical history of surgery, whereas tuberculous peritonitis is common in those patients with an immunocompromised state, eg, due to end‐stage renal disease, old age, diabetes mellitus, or HIV infection.In our case, the detected lesions closely resembled tuberculosis lesions, but were shown on microscopy to be granulomas containing foreign material—cellulose particles.In the literature, a few cases of peritoneal granulomatous reaction to cellulose fibers are described.In our case, the origin of the cellulose fibers is not entirely clear. There are several possible sources of cellulose contamination in the emergency operation setting in the Ukraine: (a) cellulose fibers originating from disposable surgical fabrics like gowns, drapes, or laparotomy pads, which have been used during the emergency laparotomy and (b) cellulose fibers originating from digested chymus which was released from the intestinal lumen into the peritoneal cavity after injury of an intestinal loop.6In conclusion, postoperative granulomatous peritonitis represents a rare disease but should be considered as differential diagnosis in patients who present with abdominal pain, abdominal tenderness, and fever after abdominal surgery. Clinical distinction from abdominal tuberculosis, which represents the most important differential diagnosis, remains a diagnostic challenge. In most cases, the proper diagnosis can only be established by abdominal biopsy and thorough histopathological and microbiological examination of samples.None declared.PK: internist, wrote the manuscript, reviewed the literature, involved in the management of the patient. KP: pathologist, revised and reviewed the manuscript, created the histological images. HK and CB: surgeon, revised and reviewed the manuscript. NJ, SF, and IS: internist, involved in the management of the patient, reviewed the manuscript. JR: internist, revised and reviewed the manuscript, reviewed the literature, involved in patient management.


Epstein–Barr virus-positive mucocutaneous ulcer (EBV-MCU) is a new category of mature B-cell neoplasms. Ulcers occur in the oropharyngeal mucosa, skin, and gastrointestinal tract. The onset of EBV-MCU is suggested to be related to the decreased immunity of the patient, the causes of which include the use of immunosuppressive agents and aging. EBV-MCU may regress spontaneously and it often has a benign course after the dose reduction or discontinuation of immunosuppressive agents or during follow-up. Here, we report the case of a patient who required surgical resection for the intestinal obstruction arising from EBV-MCU.A Japanese elderly male visited our hospital with chief complaints of a palpable mass and dull pain in the left upper quadrant, loss of appetite, and weight loss. Although abdominal computed tomography and total colonoscopy (TCS) revealed a tumor with circumferential ulcer in the transverse colon, histopathological analysis of a biopsy specimen of this lesion showed only nonspecific inflammation. Because the tumor spontaneously regressed during the time he underwent tests to obtain a second opinion from another hospital, TCS was reperformed on the patient. TCS revealed that the tumor decreased in size and the inflammatory changes in the surrounding mucosa tended to improve; however, tightening of the surrounding mucosa due to scarring was observed. Another histopathological analysis of a biopsy specimen showed widespread erosion of the mucosa and the formation of granulation tissue with marked infiltration of various inflammatory cells into the mucosal tissue of the large intestine. Moreover, some of the B-lymphocyte antigen CD20-positive B cells were also positive for EBV-encoded small RNA-1, suggesting the possibility of EBV-MCU. Later, the tumor developed into an intestinal obstruction; thus, the transverse colon was resected. Histopathological analysis of the resected specimen demonstrated scattered Hodgkin and Reed–Sternberg-like multinucleated large B cells in addition to EBER-1-positive cells. The patient was finally diagnosed as having EBV-MCU.This is the first report of a case of EBV-MCU that developed into an intestinal obstruction requiring surgical resection. It is necessary to consider the possibility of EBV-MCU when examining an ulcerative or tumorous lesion in the gastrointestinal tract. Epstein–Barr virus-positive mucocutaneous ulcer (EBV-MCU) is a new category of mature B-cell neoplasms and was formally adopted in the World Health Organization (WHO) classification of lymphoid neoplasms updated in 2016 . Ulcers An 81-year-old Japanese male visited our hospital with chief complaints of a palpable mass and dull pain in the left upper quadrant, loss of appetite, and weight loss of 5 kg within two months. He started noticing the mass in the left upper quadrant and the other symptoms in late July 2017. He was admitted to our hospital for detailed examinations and treatment in August 2017.He had high blood pressure and chronic kidney disease. He had a clear sensorium. His body temperature was 36.7 °C. He showed no yellowing of the bulbar conjunctiva, palpebral conjunctival pallor, or abnormal findings in the skin and intraoral mucosa. There was a palpable elastic, soft mass in the left upper quadrant. Although he felt a dull pain in that area, there was no obvious tenderness. There were no palpable superficial lymph nodes nor abnormalities in the extremities. His laboratory findings on admission are shown in Table Because the results of biochemical tests indicated renal damage, plain abdominal computed tomography (CT) was performed. An irregular tumor of approximately 130 mm × 120 mm × 80 mm in size was observed in the left upper quadrant, which corresponds to the transverse colon. No intestinal distention was observed on the oral side of the tumor. Moreover, the tumor was in close contact with the pancreatic body and tail, and the gastric corpus greater curvature, suggesting invasion into nearby organs. Also, mildly enlarged lymph nodes were observed at the periphery of the tumor  performed six days after his first visit revealed a tumor with a circumferential ulcer covered with a thick slough in the splenic flexure of the transverse colon. Moreover, the marked thickening and inflammatory changes of the surrounding mucosa made it difficult for the endoscope to pass through, although the lesion had not yet developed into an intestinal obstruction (Fig. The total ion Fig. a and b. ion Fig. c and d. ion Fig. a and theion Fig. b. Histopion Fig. e and f. ion Fig. d. The hiion Fig. e and f. In 2010, Dojcinov et al. reported on EBV-positive circumscribed ulcerative lesions occurring in the oropharyngeal mucosa, skin, and gastrointestinal tract of 26 patients with iatrogenic or aging-induced immunosuppression, and they identified these lesions as EBV-MCU [Because no diagnostic criteria have been established yet for EBV-MCU, it is diagnosed on the basis of clinical background factors and histopathological findings –13. It hIt is suggested that the onset of EBV-MCU is related to the decreased immunity of a patient, the causes of which include a history of organ transplantation, use of immunosuppressive agents, and aging –13. RobeIt has been reported that EBV-MCU most commonly occurs in the oropharyngeal mucosa at a rate of 40%, followed by the gastrointestinal tract and skin . As far It has been reported that most patients with EBV-MCU had a benign course after the dose reduction or discontinuation of immunosuppressive agents or during the follow-up , 10, 13.It is important to distinguish EBV-MCU from malignant lymphomas, especially Hodgkin lymphoma and DLBCL. In the present case, EBV-MCU was distinguished from a malignant lymphoma on the basis of histopathological and immunohistological findings of the specimen resected from the lesion site as well as flow cytometry and G-banding differential staining findings. Because the therapeutic strategy for EBV-MCU may largely differ from those for malignant lymphomas, it is necessary to know EBV-MCU as a new disease concept and carefully examine tumorous lesions in the gastrointestinal tract taking EBV-MCU into consideration.Although the long-term prognosis of gastrointestinal tract EBV-MCU still remains largely unclarified, it has been reported that most patients had a benign long-term course , 11, 12.EBV-MCU is a new disease concept  and is fWe experienced treating an elderly male patient with gastrointestinal tract EBV-MCU that developed into an intestinal obstruction and required surgical resection. The number of patients with EBV-MCU will increase with the aging of society and increasing use of immunosuppressive therapy. It is necessary to consider the possibility of EBV-MCU when examining an ulcerative or tumorous lesion in the gastrointestinal tract.


Hemorrhagic schwannoma in the medulla spinalis is a rare occurrence. It is a variant of the slow-growing nerve sheath tumor that usually has subtle clinical symptoms. Injury to the spinal schwannoma that was previously suspected by spinal manipulations may accelerate the progression of symptoms and cause an acute presentation of paraplegia. We report a case of a patient that was suspected of an intradural tumor with paraparesis that initially refused treatment. Spinal manipulation procedures were performed outside of the hospital setting with subsequent advancement of paraparesis. A surgical intervention was performed, which found that the tumor mass has grown along with hemorrhage within the schwannoma. The bleeding within the mass may have caused the acute paraplegia that is rarely reported. The patient had a fair improvement on her lower motor extremity function from 1‐2 to 3‐4 out of 5 at six‐month follow-up. Schwannoma tumor in the medulla spinalis is a relatively common tumor. Every year, worldwide, the number of reported cases in primary spinal neoplasms is around 1.3 to 10 for every 100,000 cases [Hemorrhagic type of schwannoma is very rare, with only a few cases reported previously. Acute neurological deficit may be caused by intratumoral bleeding which causes cord compression. This has been noted in cranial schwannomas previously, but in a spinal schwannoma, this instance is seldom found .We are presenting a report of a female with a case of hemorrhagic type of thoracolumbar schwannoma with sudden paraplegia, which was treated surgically after a period of therapy refusal and spinal manipulation by a traditional bone setter. A brief review of the literature is included to discuss current management options for these cases.A 38-year-old female patient came with a chief complaint of difficulty standing up from squatting position since 2 years ago. The patient also suffered heaviness and numbness from her hips that radiated to both of her knees and ankles. The symptoms worsened steadily in the past 4 months with both lower limbs getting weaker. Laboratory results came out normal, with no signs of infection or positive tumor markers. Radiological examination showed no apparent abnormalities as well. An MRI was obtained, and a tumor mass in the intradural region level of T10–T12 was found . At thatA surgical procedure was proposed for exploration and decompression to the patient. The operation started by opening the lamina on T10–T12 levels, followed with laminectomy and hemostatic procedure to stop the bleeding, until the dura was exposed . A denseAt the time of discharge, the patient did not regain the function on her lower extremities . After six months of follow-up, some improvement on her lower extremity function was noted. Motor strength was returned to 3-4 out of 5, and the patient was able to ambulate using a walker. No improvement of her bowel and bladder symptoms was noted.Hemorrhagic type of spinal schwannoma is a rare case and more commonly found in the cranial region of the spine . The tumThe hemorrhagic type of spinal schwannoma is a slow-growing benign lesion and occurs mostly as a solitary mass which may occur throughout the spinal canal –10. OnlyBleeding from hemorrhagic schwannomas is thought to arise from main theories, vascular and mechanical theory. Vascular theory postulates that spontaneous thrombosis occurred with distal tumor necrosis and caused secondary bleeding. The tumor maybe obliterated by endothelial proliferation with recanalization, whereas mechanical theory argued that movements of the spine induce forces on tumor vessels resulting in extravasation of blood .Diagnosis of hemorrhagic type of spinal schwannoma is made according to clinical history, physical examination, laboratory examination, radiological examination, and histopathological examination. Patients initially presented with local pain and signs of compression of neural structures with neurological deficits developing in late phase , 8. PatiEarly surgical removal is the treatment of choice, and complete removal can be achieved in almost all cases with good outcome in majority of cases , 5, 11. Hemorrhagic-type spinal schwannoma is uncommon, with only 13 cases ever being reported up to date. Prevalence among male and female patients is 7 : 6, with an average age of patients around 48.38 years old. From the 13 reported cases, hemorrhagic type of schwannoma was found in C6-C7 (30.77%), T9-T12 53.85%), L1-L2 (7.6%), and T12-L1 (7.6%). Schwannoma is usually intradural-extradural (58%), extradural (27%), dumbbell-shaped intradural-extradural (15%), and intradural (less than 1%). In our case, tumor was found intradural on the T10–T12 level, and the mass had grown to reach all the way to lumbar vertebrae L2 in just 4 months, making this case a case of hemorrhagic spinal schwannoma and also a long segmental hemorrhagic type of spinal schwannoma [3.85%, L1Hemorrhagic type of spinal schwannoma is also uncommon in hemorrhagic neoplasm cases. Neoplasms accompanied with hemorrhage are usually cavernomas, ependymomas, or hemangioblastomas. Hemorrhage in the spine is usually due to coagulopathy, malformations, or trauma. In our case, hemorrhagic type of spinal schwannoma as a rare case in hemorrhagic neoplasm may occur because of mechanical factor after manipulation by the bone setter .Clinical symptoms vary depending on the size and location of the tumor. Hemorrhagic type of schwannoma usually has an acute onset with clinical manifestation of segmental pain followed by radicular pain and motoric impairment. Some of the clinical symptoms ever recorded from the 13 reported cases are flaccid paraplegia, spastic paraplegia, urine retention, urine incontinence, progressive weakness, upper limb pain, cervical pain, cervical myelopathy, dysesthesia, back pain, severe paraparesis, nausea, and limb pain. It is very rare to have an acute and urgent onset. In our case, the first clinical manifestation was difficulty standing up from squatting position and heaviness and numbness in the hip that radiated to the knees and ankle. Progressive motoric impairment presented on both limbs within 4 months, accompanied with worsening of neurological statuses, impaired sensibility, as well as defecating and urinating problems.In our case, the spinal manipulation therapy exerted by the bone setter may be the cause of the rapid progression of the symptoms. Previous reports by Cohen and Uemura et al. described the case of acute paraplegia or paresis following a minor thoracic injury or physical exercise in patients that were later found to have intradural tumors, although further studies are needed to fully comprehend the mechanism , 11–16.Hdeib et al. in their paper cited a review of epidural hematomas following spinal manipulation therapies. In the review, some cases reported spinal epidural hematomas directly after manipulation. The literature however did not include the number of spinal intradural hematomas following the manipulation.The most important diagnostic examination in hemorrhagic type of schwannoma is MRI and histopathology . AnotherA surgical procedure was performed to our case on the T10–T12 level, and reddish vessel-rich tumor mass was found. The whole tumor mass was then evacuated and sent for histopathology examination. From microscopic findings, the following were observed: bleeding in the mass, Verocay body, cellular area (Antoni A), and loose myxoid area (Antoni B) . These fThe main procedure performed to the patients with hemorrhagic type of schwannoma or other spinal tumors is surgery (laminectomy), excision of the tumor as soon as possible , 14, 15.A spinal schwannoma is a rare tumor that can present itself with a period of acute progression of symptoms. Spinal manipulation may aggravate the symptoms of the tumor and elicit bleeding of the tumor. An immediate surgical intervention to evacuate the tumor mass may improve the prognosis for these patients.


Dear Editor,A 41-year-old woman presented with an approximately one-year history of progressivefacial swelling and left-sided visual impairment. A computed tomography (CT) scan of theskull showed a sclerotic, expansile lesion on the lateral/upper wall of the left orbit,narrowing and extending to the optic canal. Magnetic resonance imaging (MRI) showed alesion with a hypointense signal in T1-weighted and T2-weighted sequences, withoutsignificant contrast uptake, accompanied by a slight contrast-enhanced thickening of thesubjacent dura mater, which was compressing the left optic nerve. A histopathologicalstudy confirmed the suspected diagnosis of intraosseous meningioma.(1-3). Meningioma is the most common primary intracranialtumor, representing approximately 14-20% of cases. The vast majority are intradurallesions, extradural lesions accounting for only 1-2%(4). Extradural meningiomasaffect the cranial vault in 68% of cases, such lesions being referred to as primaryintraosseous meningiomas (PIMs), which mainly affect the frontal and parietal bones, aswell as the region of the orbit(5-7). Other common locations for extradural involvement arethe subcutaneous tissue, paranasal sinuses, and parapharyngeal spaces, as well as, inrare cases, the lungs and adrenal glands. Unlike typical intradural meningiomas, which primarilyaffect females between the ages of 50 and 69 years and usually have a benign course,PIMs can affect either gender, have a peak incidence in the second decade of life, andare more likely to evolve to malignant degeneration(6).Recent studies in the radiology literature of Brazil have emphasized the importance ofimaging examinations in improving the diagnosis of central nervous systemdisorders(6). On MRI, they commonly hypointense in T1- andT2-weighted sequences, typically without significant contrast enhancement, as in thecase reported here(5). However, in rarer cases, if a PIM presents as anosteolytic lesion on CT, an MRI scan can show a hypointense signal in T1-weightedsequences and a hyperintense signal in T2-weighted sequences, as well as contrastenhancement. Although PIMs do not present the dural tail sign thatis often found in intradural meningiomas, there can be contrast uptake in the dura matersubjacent to the tumor due to venous stasis or to tumor invasion, as demonstrated in ourcase(7).There are inherent differences between CT and MRI, the former allowing betterdelineation of bone involvement, whereas the latter provides a better assessment of thesoft-tissue involvement and extradural extent of the lesion(6).On CT, most PIMs (65%) present as expansile, osteoblastic bone lesions, with or withoutcortical destruction(6).The differential diagnosis of osteoblastic PIM includes typical intradural meningiomawith reactive hyperostosis, in which the meningeal component of the lesion is the mostobvious. Other diagnoses that should be considered are metastases, plasmacytoma, fibrousdysplasia, osteoma, osteosarcoma, and Paget’s disease(6).In most cases of PIM, the treatment is total surgical resection, with subsequent cranialreconstruction. If the resection is partial, there should be radiological follow-up; ifthe disease has recurred or if the residual lesion has progressed, the next surgicalprocedure can be accompanied by adjuvant radiotherapyIn conclusion, although rare, PIMs should be considered in the differential diagnosis ofbone lesions, especially when the lesions are osteoblastic and located in the cranialvault.


Hepatocellular carcinoma (HCC) develops in the presence of chronic liver disease, and nonalcoholic fatty liver disease is becoming a frequent cause of HCC in developed regions. Spontaneous rupture of HCC (rHCC) is a potentially life‐threatening complication of a tumor. The patient's compliance with surveillance after liver resection is vital for the prevention of rHCC. After the first rHCC, he abandoned organized surveillance appointments, and the second rHCC occurred from a newly developed HCC. This time, surgical hemostasis and emergency liver resection were used.Patients with HCC present with a broad spectrum of disease that varies from early stages, often noticed at surveillance, to advanced lesions that can cause symptoms.Rupture of HCC can remain indolent without causing symptoms, but it can also lead to various degrees of abdominal pain and bleeding.This report follows the case of a 50‐year‐old patient who suffered from two episodes of life‐threatening bleeding from two rHCCs within a 6‐year span. He stopped attending organized appointments following radical treatment for the first rHCC and was again admitted to a hospital with bleeding from a newly developed and ruptured HCC.22 and a waist circumference of 120 cm, who was abstinent from alcohol and had arterial hypertension, diabetes mellitus type 2, and dyslipidemia. He was a full‐time employed construction worker and smoked more than 40 cigarettes per day. Later, tests for hepatitis C virus, hepatitis B virus, aflatoxin B1, autoimmune hepatitis, hereditary hemochromatosis, Wilson disease, primary biliary cirrhosis, and alpha‐1 antitrypsin deficiency were all negative.In 2010, a 50‐year‐old white male was admitted to the Emergency Unit of a tertiary referral center with an acute setting of abdominal pain and with an urge to vomit. His history revealed an obese patient with a body mass index (BMI) of 32 kg/mOn admission, the patient was pale and normotensive (125/70 mm Hg) and his heart rate was of 90 bpm. A clinical examination showed tenderness on abdominal palpation, which was dominant on the right side. Laboratory results revealed decreased levels of hemoglobin (87 g/L) and hematocrit (0.25). Liver function tests were within the normal range  or slightly impaired . Ultrasound sonography (US) showed free fluid in the abdominal cavity and a liver tumor in the right hemiliver. A computed tomography (CT) scan later revealed active bleeding from a solitary, vascularized HCC of 4.5 cm in diameter. The tumor was present in segment 6 and protruded from the liver surface  was performed Figure .3 The prThen, he had regular follow‐ups every three months.2 and had all the previously described comorbidities. On examination, he was pale, with a tense, distended abdomen, and a blood pressure of 88/66 mm Hg with a heart rate of 110 bpm. Therefore, aggressive fluid resuscitation was started promptly. Multiple blood tests revealed declining values of hemoglobin (81 g/L) and hematocrit (0.24) with increasing levels of serum creatinine (210 μmol/L) and elevated inflammatory markers, with C‐reactive protein (CRP) levels reaching 113 mg/L. A 58% prothrombin activity, bilirubin level of 27 μmol/L, and albumin level of 24 g/L showed impaired liver function.In 2016, following a 3‐year hiatus from his previous check‐up, the patient was again brought to the Emergency Unit with an acute setting of abdominal pain. He was still obese with a BMI of 31 kg/mThe abdominal US revealed a large mass in the left hemiliver and a collection of free intraabdominal fluid. CT confirmed active bleeding from a rHCC in the left lateral section alongside a massive hemoperitoneum Figure .To stop the bleeding, an urgent TAE was performed.Following successful hemostasis of the ruptured tumor, the patient was transferred to the intensive care unit (ICU). He received multiple units of blood components to correct his anemia and coagulopathy. Soon after, blood tests revealed worsening of acute renal failure (serum creatinine 477 μmol/L). The following day, renal dialysis was needed, and inflammatory markers (CRP 482 mg/L) were highly elevated. The patient was deteriorating despite intensive care support, and the multiple organ dysfunction syndrome (MODS) was worsening. Imaging was repeated, which exposed an expected extensive necrotic region on the side of a tumor. A definitive surgical procedure had been planned, aiming to remove the tumor and the surrounding necrotic tissue. Emergency liver resection was performed two days after surgical hemostasis with an anatomical left lateral sectionectomy.Histopathology of the tumor confirmed rHCC once again. This time, the tumor was more massive, with a diameter of 8 cm. Multiple tumor thrombi were present in the segmental branches of the portal vein. The trabecular growth pattern was again described, but this time, the histologic grade was defined as 3.After a successful surgical procedure, the patient showed straightforward signs of clinical improvement accompanied by encouraging results from the laboratory findings. He was discharged from the hospital twelve days after liver resection, with a new follow‐up date. According to the Barcelona clinic liver cancer classification, he was classified as stage C (pulmonary metastases at the time of the second rupture) and was therefore eligible for therapy with sorafenib.3Spontaneous rupture with severe bleeding is a potentially life‐threatening complication of HCC.First, the development of HCC is closely related to the presence of chronic liver disease.Another issue in this case is the risk factors of spontaneous rupture of HCC. In the literature, several risk factors for HCC rupture have been found, including underlying arterial hypertension, liver cirrhosis, tumor size more than 5 cm, tumor protrusion from the liver surface, vascular thrombosis, and extrahepatic invasion of HCC.A point of interest in this case pertains to the challenging treatment of an acute presentation of rHCC. The hemodynamic status of the patient is the main factor in clinical algorithms.Finally, recently reported overall early mortality rates associated with spontaneous rHCC have been from 0% to 63.8% Table .4, 5, 124This report revealed the complexity involved in the management of rHCC with severe intraperitoneal hemorrhage. TAE followed by an elective hepatic resection was an effective strategy at the time of the first rHCC. The second manifestation of rHCC, six years after the first, could have been prevented. However, the patient's compliance was not adequate since he did not manage to change his lifestyle, and he did not come to his surveillance appointments. Chronic liver disease progressed, and a new HCC developed in a different location. A two‐stage surgical strategy resulted in a complete recovery of the patient after the second rHCC. However, a systemic spread of the disease precluded long‐term survival at the time of the second rupture.The authors declare that they have no competing interests. The authors alone are responsible for the content and writing of this article.IP: prepared the review of the literature and wrote the manuscript. MJ: gathered the patient's data. SP: contributed to the review of the manuscript. AI: improved the manuscript and collected graphic material. IP and AI: should be considered joint first authors.A local Ethics Committee approved this case report.


Medulloblastoma is the most common type of aggressive pediatric primary brain malignancy. This case describes a 45-year-old Hispanic male with no significant past medical history who presented to the emergency department (ED) complaining of 15 days of 10/10 intractable headaches with one day of lightheadedness, confusion, and loss of balance. An urgent magnetic resonance imaging (MRI) of the brain revealed a 4.1 x 3.3 x 3.2 cm mass at the cerebellum, exerting a mass effect on the brainstem and mild tonsillar herniation. A pre-surgical physical exam revealed only a positive Babinski sign bilaterally with normal proprioception and cerebellar function. The intraoperative report concluded an undifferentiated neoplasm with a histological differential diagnosis of medulloblastoma, ependymoma, or other neuroepithelial neoplasms, suggesting a referral to a tertiary care center for further evaluation of the mass. Postsurgical complications included a severe vasogenic edema, causing obstructive hydrocephalus treated with frontal ventricular drainage, signs of meningitis treated with antibiotics, and hyponatremia. This case describes a rare occurrence of medulloblastoma in an adult patient, which required prompt diagnosis and urgent life-saving treatment. Medulloblastoma is the most common, aggressive, invasive, malignant embryonal tumor of the cerebellum with a preferential manifestation in children. In the United States, 2200 children are diagnosed with a brain tumor of which each medulloblastoma accounts for 22% of all childhood primary tumors . HoweverA 45-year-old male without a significant past medical history presented to the emergency department (ED) as a walk-in, complaining of 10/10 intractable headaches with lightheadedness, confusion, and loss of balance, starting one day before presentation to the ED. A computed tomography (CT) scan of the head done in the ED revealed a hyperdense lesion on the right at the level of the peri- pontine cistern and magnetic resonance (MRI) of the brain done for a further evaluation of the lesion revealed a 4.1 x3.3x 3.2 cm mass with mild tonsillar herniation and a mass effect on the brainstem . Consequently, he was admitted for further work-up. Since an adult brain neoplasm is more likely to be a metastasis rather than a primary malignancy, CT scans of the chest, abdomen, and pelvis were obtained. The scans identified a right thyroid nodule but no lung mass on CT of the chest, and the abdomen was free of masses except for a 2.3 cm renal cyst. He had a right suboccipital craniotomy on Day 2 of admission. The intraoperative report concluded an undifferentiated neoplasm with a histological differential diagnosis of medulloblastoma, ependymoma, or other neuroepithelial neoplasms. Additionally, the CD45 marker was positive, which raised the differential diagnosis of a lymphoproliferative disorder. The case was referred for consultation to a tertiary care center, which made the diagnosis of classic medulloblastoma, world health organization (WHO) grade IV. The tumor was composed of densely packed cells with round to oval highly hyperchromatic nuclei surrounded by scanty cytoplasm most consistent with classic medulloblastoma with non-desmoplastic nodules. He subsequently developed vasogenic edema that completely occluded his fourth ventricle, producing obstructive hydrocephalus and required the emergent insertion of a frontal ventricular drain into the right lateral ventricle. He later developed a fever, headache, and neck stiffness suggestive of meningitis. Lumbar puncture was deferred due to raised intracranial pressure, and the patient was started on empiric antibiotics. Leukocytosis resolved and fever subsided. Additionally, he developed hyponatremia with urine osmolality of 384, urine sodium of 24, and serum osmolality of 284 with a differential diagnosis of syndrome of inappropriate anti-diuretic hormone (SIADH), cerebral salt wasting, or reactive elevation of the anti-diuretic hormone secondary to surgery. He was started on sodium chloride tablets, demeclocycline, and fluid restriction. Post-surgery imaging showed no metastasis and radiation oncology recommended follow-up in a tertiary center.In adults above the age of 40, metastatic brain tumors account for more than half of all brain tumors. A statistical report of primary brain tumors states that medulloblastoma and other embryonal tumors are only common in age group zero-four years old with an incidence of 0.32 per 100,000 population and drop to 0.02 per 100,000 population in children age five–nine years. To date, there are only a few case reports published in the literature on medulloblastoma in the older age group .Most patients present with headaches and ataxia or other nonspecific symptoms of increased intracranial pressure . AdditioOur case illustrated the potential for developing an embryonal tumor late in adulthood. Adults with medulloblastoma have a worse prognosis compared with children and early diagnosis is paramount since disseminated or metastatic disease at the time of diagnosis is associated with a particularly poor prognosis . The delAdults with medulloblastoma have a worse prognosis compared with children and early diagnosis is paramount since disseminated or metastatic disease at the time of diagnosis is associated with a particularly poor prognosis. Though adult medulloblastomas are rare, there are distinct imaging biomarkers that are different from those occurring in children. Delayed complications of medulloblastoma can have a profound effect on quality of life and mortality and increased recognition is critical for the institution of appropriate and timely therapy.


Vincristine‐induced vocal cord palsy (VCP) is a rare but critical complication. Prompt recognition of VCP is imperative. Vincristine‐induced VCP is reversible, and a complete remission of a lymphoma is still feasible upon withdrawal of vincristine from the chemotherapeutic regimen early in the course of treatment. Vincristine‐induced vocal cord palsy (VCP) is a rare but critical complication. Prompt recognition of VCP is imperative. Vincristine‐induced VCP is reversible, and a complete remission of a lymphoma is still feasible upon withdrawal of vincristine from the chemotherapeutic regimen early in the course of treatment. We report a case of a young male who was diagnosed with human immunodeficiency (HIV)‐associated stage III Burkitt‐like lymphoma, who developed acute voice hoarseness and grade 3 peripheral neuropathy following the second dose of chemotherapy with dose‐escalated rituximab, etoposide, doxorubicin, vincristine, and cyclophosphamide. Fiberoptic laryngoscopy revealed a sluggish right vocal cord confirming unilateral vocal cord paralysis. Vincristine was discontinued from subsequent cycles of chemotherapy resulting in complete resolution of the vocal cord palsy and resolution in the peripheral neuropathy over eight weeks. He had complete response (CR) following completion of chemotherapy. This case highlights that instantaneous recognition of vincristine‐induced vocal cord palsy is paramount and requires an urgent otolaryngology evaluation. Furthermore, it can be reversed and achievement of a CR is still possible with withdrawal of vincristine from the chemotherapeutic regimen early in the course of treatment.Vincristine, a vinca alkaloid, is derived from the periwinkle plant (Vinca Rosacea) and has been used to treat numerous childhood and adult malignancies for several decades.We present the first reported case of an adult male with human immunodeficiency virus (HIV)‐associated high‐grade B‐cell lymphoma who developed acute vincristine‐induced vocal cord paralysis (VCP) and peripheral neuropathy during the early course of treatment with a vincristine‐containing chemotherapeutic regimen.23. An upper endoscopy for workup of the anemia revealed oozing ulcers in the stomach body and granular masses in the second part of the duodenum which were biopsied. Histology demonstrated a high‐grade B‐cell lymphoma, not otherwise specified (NOS) consistent with Burkitt‐like lymphoma in both sites, involving gastric and duodenal mucosa. Staging PET/CT demonstrated widespread metastatic disease, with gastric, duodenal, and small bowel wall thickening with intense FDG uptake, multiple peritoneal implants, hepatic lesions, moderate ascites, and bilateral thyroid intense FDG uptake, consistent with stage III disease. Bone marrow biopsy was negative for lymphoma.A 32 year‐old Caucasian male with recently diagnosed HIV was admitted with acute symptomatic microcytic anemia, fatigue, and abdominal pain. He was diagnosed with HIV four months prior to presentation and had been initiated on anti‐retroviral therapy (ART) as part of a trial. Absolute CD 4 count at diagnosis of HIV was 309 cells/mm2. There was no obstruction or anatomical abnormality noted on CT neck. An urgent otolaryngology referral was made, and a fiberoptic laryngoscopy examination showed a sluggish right vocal cord fold and an incomplete glottic closure with a gap confirming a diagnosis of unilateral VCP due to vincristine. The subsequent four cycles of chemotherapy were continued with omission of vincristine, and there were no further complications. The subjective voice hoarseness completely resolved, and the grade 3 peripheral neuropathy improved to grade 1 within 8 weeks of discontinuing vincristine. The abdominal pain resolved, fatigue improved, and hematocrit showed continued improvement. A re‐staging PET/CT following completion of six cycles of chemotherapy demonstrated a complete response (CR). He remains in CR over one year following completion of chemotherapy.Systemic chemotherapy with dose‐escalated R‐EPOCH  was initiated. Twenty‐four hours after completing the second cycle of chemotherapy, he developed acute profound voice hoarseness and bilateral grade 3 peripheral neuropathy in his fingers and toes. The cumulative dose of vincristine he had received was 3.2 mg/m3Vincristine is a critical component of chemotherapeutic regimens to treat various adult malignancies including HIV‐associated B‐cell lymphomas. Similar to other chemotherapeutic agents, it comes with its own potential toxicities including neurotoxicity. Various neurotoxic presentations may present as sensorimotor neuropathy, cranial neuropathy, autonomic neuropathy, and rarely as encephalopathy. Factors that make patients susceptible to more severe neurotoxicity include hepatic impairment, hypersensitivity to vincristine, hereditary neuropathy, concomitant use of drugs such as erythromycin, allopurinol, isoniazid, phenytoin, and interactions between ART and chemotherapy.The majority of cases of vincristine‐induced VCP have been reported in the pediatric population.Despite the unsettled mechanism of vincristine‐induced neuropathy, neuroprotective and neuroregenerative treatment efforts have been attempted. One study using animal models suggested a neuroprotective effect with erythropoietin (EPO).Vincristine‐induced VCP is fortunately reversible. One case study reported an older male with mantle cell lymphoma, who developed hoarseness due to bilateral VCP after the second cycle of chemotherapy with R‐CHOP. Vincristine was omitted from subsequent cycles of chemotherapy and he had full recovery of the VCP and had attained complete remission.Given the fact that our patient had underlying HIV, an HIV‐associated sensory neuropathy and vocal cord palsy was initially postulated. There is data to suggest an increased risk of autonomic neurotoxicity when vincristine‐containing chemotherapy is administered with proteasome inhibitor‐based ART. Vincristine is metabolized by the CYP 3A4 enzyme, and the use of CYP 3A4 inhibitors may result in increased levels leading to increased toxicity of peripheral and autonomic neuropathy. There is data evaluating the influence of ART on the pharmacokinetics of doxorubicin and cyclophosphamide4Clinicians must have a high index of suspicion of vincristine‐induced VCP in a patient who complains of acute voice hoarseness. An urgent otolaryngology consultation and prompt withdrawal of the offending agent are required. VCP can be a life‐threatening event but fortunately it is a reversible complication. Our patient remains in CR one year following completion of six cycles of chemotherapy, despite omission of vincristine with cycles 3‐6. To our knowledge, this is the first reported case of vincristine‐induced unilateral vocal cord paralysis in a patient being treated for HIV‐associated Burkitt‐like lymphoma with dose‐escalated R‐EPOCH, who had full recovery of the vocal cord paralysis within eight weeks after cessation of vincristine.The authors declare that there is no conflict of interest.LTR: provided contributions to the design of the case report, acquired and interpreted the data, and drafted the manuscript. PHB: drafted the manuscript and revised it critically for significant intellectual content. SSA: provided conception of the case report and provided final approval of the version to be published.