As a new entity included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013, hybrid peripheral nerve sheath tumours are benign composite neoplasms that demonstrate features of more than one type of nerve sheath tumour, with a wide age distribution and a predilection for superficial location. Those involving deep sites are relatively rare. To the best of our knowledge, only one case of primary intraosseous hybrid peripheral nerve sheath tumours has been documented. In this article, we report another case of hybrid peripheral nerve sheath tumours occurring in bone with different clinical, radiological and pathological features from those in the previously reported cases.A 28-year-old female presented with a painful nodule in the right tibia. Radiological examination revealed an oval eccentric osteolytic lesion in the proximal tibia. Histologically, the circumscribed but unencapsulated lesion demonstrated biphasic cellular differentiation. Bland, small epithelioid cells arranged in clusters in the myxoid or collagenous stroma and inconspicuous spindle cells scattered in the hypercellular areas were suggested to originate from Schwann cells according to the detection of S100. Both the elongated spindle cells with thin, wavy nuclei and the spindle cells in fascicular or storiform pattern in hypercellular areas showed a positive immunoreaction for epithelial membrane antigen, indicating perineurial differentiation. Based on histological and immunochemical examinations, the patient was diagnosed with hybrid epithelioid schwannoma/perineurioma. The lesion was resected and has not recurred for 8 months since resection.The present case is the second primary intraosseous hybrid peripheral nerve sheath tumour to be reported. This is also the first reported intraosseous tumour composed of epithelioid schwannoma and perineurioma with hypercellularity, indicating diverse involvement sites and a wide range of histological features among hybrid peripheral nerve sheath tumours. Awareness of such diversity is critical for accurate diagnoses. The morphological overlap with other spindle and epithelioid cell neoplasms, especially pure peripheral nerve sheath tumours, requires that immunochemical and molecular examinations be used as objective tools to provide the necessary information for a differential diagnosis.The online version of this article (10.1186/s13000-019-0829-x) contains supplementary material, which is available to authorized users. Peripheral nerve sheath tumours encompass a spectrum of well-defined clinicopathological entities, ranging from benign tumours, such as schwannomas, neurofibromas and perineuriomas, to high-grade malignant neoplasms . SchwannOccasionally, neoplasms exhibit the co-existence of more than one conventional type of nerve sheath tumour and are difficult to fit into one specific diagnostic category. Such tumours were included in the 4th edition of the WHO classification of tumours of soft tissue and bone in 2013 as a new entity named hybrid peripheral nerve sheath tumours (HPNSTs). According to the histological components, a HPNST can be further classified into hybrid schwannoma/neurofibroma, schwannoma/perineurioma, neurofibroma/perineurioma and schwannoma/neurofibroma/perineurioma , the incThe tissue obtained via curettage was processed using routine histological methods: 10% formalin fixed, paraffin embedded and haematoxylin-eosin stained. Both immunohistochemical studies and fluorescence in situ hybridization (FISH) analysis were carried out on formalin-fixed paraffin-embedded (FFPE) tissue. Appropriate positive and negative controls were applied simultaneously. The primary antibodies used for the immunohistochemical studies are listed in Table A 28-year-old female presented with a palpable and painful nodule that had been present for five years in the right tibia. The pain was gradually aggravated in the last four months. On the day of presentation, the pain was so unbearable that it limited the movement of the right lower limb. Physical examination revealed a tenderness along the medial portion of the right proximal tibia. There was no external wound, bone friction or rubbing, swelling of the right popliteal lymph nodes or circulation disturbances. Clinical examination failed to show any features of neurofibromatosis, and her familial history was uneventful. Lesion resection and artificial bone graft were performed successively. The patient had a disease-free follow-up 8 months after the surgery.A conventional anterior-posterior radiograph showed an osteolytic lesion, 2.0*1.6*1.4 cm in size, located in the medial superior metaphysis of the right tibia. The sclerotic rim was observed, indicating its non-aggressiveness .Gross examination showed solid tissues with firm, white cut surfaces, measuring 2 cm in aggregate. Histologically, low-power magnification revealed a well-circumscribed and unencapsulated lesion with variable cellularity. The relatively hypocellular areas, which was the predominant part of the neoplasm, presented with biphasic cellular morphology. One morphology of these areas exhibited epithelioid cells with bland nuclei and eosinophilic cytoplasm arranging in small clusters within the myxoid or collagenous matrix Fig. a-c. AddiBased on the above findings, the present case was diagnosed as hybrid epithelioid schwannoma/perineurioma primarily occurring in the right tibia.Most benign peripheral nerve sheath tumours showing distinctive morphological and immunochemical features can be classified into a certain category. In 1998, Feany et al. [Hybrid schwannoma/perineurioma is the most common type of HPNSTs . Due to Compared with S100 and EMA, there were fewer researches on other proteins in HPNSTs that might be expressed in pure schwannoma or perineurioma. Apart from S100, SOX-10 has also been indicated to be a nonspecific, but highly reliable marker for schwannoma . A case HPNSTs have a predilection of superficial locations . Rare caApart from bland pathological characteristics, radiological features such as sclerotic rim indicated non-aggressiveness of the present lesion. The patient was still alive without recurrence 8 months after the surgery. To date, the vast majority of HPNSTs have demonstrated mild morphological features and benign biological behaviour, and local recurrence is extremely rare after standard surgical resection. In 2011, Rekhi et al. reported a case of malignant peripheral nerve sheath tumours (MPNSTs) arising in a hybrid schwannoma/perineurioma, occurring in the thigh of a young male. In some sections of the lesion, the hypercellular neoplasm exhibited diffuse moderate atypia, prominent mitoses, epithelioid malignant change, discrete tumour necrosis and apoptotic debris. p53 and MIB1 (a proliferation marker) immunostaining highlighted 40–50% and 30–40% of the tumour cell nuclei, respectively . HoweverThe main pathological differential diagnoses of the present case included epithelioid schwannoma, perineurioma, MPNSTs and synovial sarcoma (SS), low-grade fibromyxoid sarcoma(LGFMS) and solitary fibrous tumour(SFT). The non-overlapping S100 and EMA positive cell components were consistent with the hybrid features and suggest evidence against the diagnosis of pure epithelioid schwannoma or perineurioma.The tumour that perhaps most closely resembles the present case is MPNSTs. MPNSTs are malignant neoplasms showing nerve sheath cellular differentiation and may originate from pre-existing benign PNSTs, from peripheral nerves or within the context of NF1 . The morMonophasic SS is a malignant neoplasm with frequent local recurrence and metastasis. SS usually affects young adults and arises in deep soft tissues of the extremities and trunk. As the most common variant, monophasic SS is composed of uniform spindle cells with scanty cytoplasm arranging in vague fascicles or in dense cellular sheets. Variations in cellularity have been observed in some cases, creating a “marble” appearance . PrimaryLGFMS often affects young adults and arises in deep soft tissue of proximal extremities and trunk. Patients usually present as with a slow growing, painless mass with long clinical duration . AlthougSFT is a fibroblastic mesenchymal neoplasm composed of uniform spindle to ovoid fibroblastic cells with myxoid stromal change and varying cellularity . CompareIn summary, we described the first case of intraosseous hybrid epithelioid schwannoma/perineurioma. Awareness of the potential occurrence in unusual locations might enhance the recognition of these hybrid tumours. As for intraosseous neoplasm, radiological examination is a useful tool to identify biological nature. Due to the morphological overlap with a pure peripheral nerve sheath, immunohistochemistry is indispensable for definitive diagnosis. Long-term observation and large-scale studies would better reveal the biological behaviour of HPNSTs.Additional file 1:Figure S1. Collagen-IV was present around individual cells and small groups of cells in the hypocellular areas composed of epithelioid cells. Immunohistochemical staining, 400x magnification. (TIF 8519 kb)Additional file 2:Figure S2. The epithelioid cells(a), the elongated spindle cells(b) and the spindle cells composed of hypercellular areas(c) were positive for CD99. Immunohistochemical staining, 400x magnification. (TIF 8660 kb)Additional file 3:Figure S3. The tumour showed a normally fused SYT signal. A broken SYT signal was not detected by fluorescence in situ hybridization. . (TIF 5591 kb)


Background: Duodenal masses are rare entities and symptomatic presentation generally is due to abdominal pain or the presence of gastrointestinal bleeding. A number of published case reports in the literature have detailed various neoplasms that have caused an intussusception isolated to the duodenum. This is a particularly unusual phenomenon due to the location and fixation of this portion of the proximal small bowel to the retroperitoneum. We present here a case of duodeno-duodenal intussusception secondary to a leiomyoma.Case: A 65-year-old Caucasian male presented with intermittent bloody stools and syncope over a 9-month period secondary to a duodenal leiomyoma causing intussusception, which was treated through a pancreaticoduodenectomy.Conclusion: Intussusception of the duodenum is an uncommon entity and the diagnosis of a leiomyoma should be considered in the setting of a potential mass in the small intestine. Etiologies for gastrointestinal (GI) bleeding commonly include bleeding ulcers, arterial venous malformations, diverticular disease, hemorrhoids, and anal fissures, whereas uncommon causes include Meckel's diverticulum and intussusception. Typically, conventional diagnostic modalities such as physical examination, endoscopy, and radiological imaging yield a cause, although occasionally surgical intervention is required to obtain a diagnosis. Here, we report the first case of a patient with GI bleeding secondary to duodeno-duodenal intussusception caused by a leiomyoma.A 60-year-old male presented to the Thomas Jefferson University Hospital in August 2015 with a syncopal episode, fatigue, abdominal pain, and dyspnea on exertion. A review of the patient's records revealed two previous hospitalizations to an outside institution over the prior 9 months for melena and near syncope. A computed tomography (CT) with enterography performed at the outside hospital in November 2014 revealed a 4 cm intraluminal mass-like density. An esophagogastroduodenoscopy (EGD) with endoscopic ultrasound was performed at that time, which revealed an extrinsic thickened fold of the second portion of the duodenum (D2), concerning for a duodenal duplication cyst. Fine-needle aspiration of the suspected mass revealed normal villous morphology. At the patient's second presentation to the outside hospital in July 2015, a second EGD evaluation revealed a bleeding ulcer in the stomach and a repeat CT showed that the duodenal mass had increased to 5 cm.At the time of transfer to our institution, the patient reported a recent episode of melena without gross blood. He was found to have a hemoglobin of 6.8 g/dL and received two units of packed red blood cells. A CT scan to evaluate the abdominal mass revealed focal dilation and thickening of the third portion of the duodenum (D3) with intussusception of D2 into D3 . There wDue to the concern for a malignant mass involving the ampullary complex, a pylorus-preserving pancreaticoduodenectomy was performed. The specimen was removed and gross pathology revealed a 7 × 6 × 3.5-cm duodenal submucosal leiomyoma . The mas1 Presenting symptoms are classically described with the triad of abdominal pain, a palpable abdominal mass, and bloody stools, but all three are clinically found in less than 10% of the population with intussusception.2 More commonly, patients present with nonspecific symptoms including intermittent abdominal pain, nausea, vomiting, and anemia.3 Given the wide range of diseases with this constellation of symptoms, physicians must have a strong clinical suspicion to make the diagnosis. Work-up may include hemoglobin levels, fecal occult blood tests, cross-sectional imaging (CT or MRI), and endoscopy . Definitive diagnosis may require operative intervention with surgical exploration of the abdomen.Intussusception is a condition that is more common in children and rarely seen in the adult population. The condition results from the full-thickness invagination of a proximal portion of the bowel into the distal portion.4 Intussusception of the proximal small bowel, especially with the intussusceptum and intussuscipiens isolated to the duodenum, is a rare condition with only 16 reported cases in the literature occurring in 19 total patients 18 or secondary to congenital malrotation of the bowel, where the duodenum is more mobile, allowing the intussuception to occur.4 The most common etiologies for such lead points include both benign and malignant tumors, lipomas, Brunner's gland hamartomatous polyps, or adenomas.5 Although leiomyomas have been known to cause intussusception in other parts of the intestine, our patient represents the first reported case to our knowledge of duodeno-duodenal intussusception secondary to leiomyoma.The most common location for intussusception is the ileocolic junction, followed by ileoileal and colocolic.patients .1,2,4–1719–21 The more frequently encountered malignant small bowel tumors are adenocarcinomas, lymphomas, and neuroendocrine tumors. Rarely seen are GI stromal tumors, leiomyosarcomas, and leiomyomas. Although leiomyomas are the most common benign lesions of the small intestine, they are a small proportion of total small intestine neoplasms. At one institution over a 115-year span, fewer than nine leiomyomas were diagnosed per year, whereas a second report examining 1091 smooth muscle tumors of the small intestine found that only 1% were truly leiomyomas.22Neoplasms of the small bowel as a whole are exceedingly rare, comprising only 1–5% of all GI neoplasms, with only 1–2% of these being malignant.22 Diagnosis may be difficult despite modern imaging modalities and endoscopy, as was demonstrated in this patient. He suffered from symptoms for nearly 10 months before the diagnosis was confirmed through surgical exploration. In the setting of intermittent GI bleed and abdominal pain with no easily identifiable source, it is important to consider the diagnosis of small bowel intussusception in an adult due to a neoplasm.Leiomyomas are found most often in the jejunum followed by the ileum and lastly the duodenum. They are characteristically well-defined solitary masses and may appear gray or white. Within the small intestine, they can be found intraluminal, extraluminal, or intramural.This case report is that of GI bleeding due to a rare duodeno-duodenal intussusception secondary to a small bowel leiomyoma. We emphasize the importance of a keen awareness for this diagnosis and thorough work-up. In this scenario, surgical intervention may prove to be both diagnostic and therapeutic as demonstrated here.


Pneumoperitoneum is often associated with surgical complications or intra-abdominal sepsis. While commonly deemed a surgical emergency, pneumoperitoneum in a minority of cases does not involve a viscus perforation or require urgent surgical management; these cases of “spontaneous pneumoperitoneum” can stem from a variety of etiologies. We report a case of a 72-year-old African American male with a history of metastatic pancreatic adenocarcinoma who presented with new-onset abdominal distention and an incidentally discovered massive pneumoperitoneum with no clear source of perforation on surveillance imaging. His exam was non-peritonitic, so no surgical intervention was recommended. He was treated with bowel rest, intravenous antibiotics, and hydration. He had a relatively benign clinical course with preserved gastrointestinal function and had complete resolution of his pneumoperitoneum on imaging two months after discharge. This case highlights the importance of considering non-surgical causes of pneumoperitoneum, as well as conservative management, when approaching patients with otherwise benign abdominal exams. Pneumoperitoneum is defined as the presence of free air within the peritoneal cavity. In the vast majority of cases (approximately 90%), this is a result of an intra-abdominal viscus perforation, often requiring intravenous antibiotics and acute surgical intervention . PatientHowever, there have been documented cases of pneumoperitoneum that do not involve viscus perforation ; this ha3/uL with neutrophil predominance (83%). A repeat CT scan showed patent biliary and duodenal stents without a definite source of perforation; however, three suspicious locations were noted by the radiologist containing small foci of free air adjacent to the bowel.A 72-year-old African American male presented to the emergency department from the outpatient oncology office with a week of mild abdominal pain, which was not life-threatening. His past medical history was notable for metastatic pancreatic adenocarcinoma, which had been diagnosed one year earlier, from endoscopic retrograde cholangiopancreatography (ERCP) brushings when he presented with obstructive jaundice and required biliary and duodenal stent placement at the time. He had undergone a routine CT scan of his abdomen and pelvis for disease progression two days prior to admission, which revealed a massive pneumoperitoneum Figures , 2. The Given the initial concern for perforated viscus, the patient was started on broad-spectrum antibiotics and antifungals. He was managed conservatively with intravenous hydration and strict bowel rest. He remained hemodynamically stable and non-septic throughout his hospitalization and continued to have flatus and intact gastrointestinal function. General surgery recommended no acute surgical intervention. In a few days, he was started on a clear liquid diet which he tolerated well. His abdominal distension gradually improved without any surgical intervention, and he was discharged home a week after admission. Two months later, he underwent a repeat CT scan that showed resolution of the pneumoperitoneum Figure .Despite its rare occurrence, spontaneous pneumoperitoneum can be attributed to a variety of causes. These can be roughly divided into four categories: intrathoracic, intra-abdominal, gynecologic, and idiopathic. Intrathoracic causes are among the more commonly reported cases and involve direct passage of air either through microscopic pleural and diaphragmatic defects or through retroperitoneal perivascular sheaths . IntermiWe report a case of a patient who presented with an incidentally found pneumoperitoneum on CT scan. Given his history of pancreatic cancer and stent placement, the concern for viscus perforation was high, as small bowel perforation from metastatic pancreatic cancer has been previously described . PerforaSpontaneous pneumoperitoneum is a rare but well-described phenomenon. A detailed history and physical examination with corroborative imaging studies are key to diagnosing this condition. The CT scan findings can appear worrisome; hence, it is necessary to monitor with serial abdominal exams and consult general surgery on presentation. Bowel rest, intravenous broad-spectrum antibiotics, electrolyte repletion, intravenous hydration, and serial abdominal exams are essential to managing this condition. Treatment is primarily non-surgical in the majority of cases, as long as the exam is non-peritonitic. Increased awareness about the various non-surgical causes may help to narrow the diagnosis and avoid unnecessary surgical interventions.


Rhabdomyosarcoma is a malignant tumor of the soft tissues which preferentially affects the pediatric population. Neonatal rhabdomyosarcoma is rare, and much of the published literature concerning this entity consists of isolated case reports and small case series. Recent work involving the classification of rhabdomyosarcoma has helped to delineate prognostic information based on gene rearrangements. Here, we present a case of congenital rhabdomyosarcoma seen in utero which manifested as a neck mass at birth and was found to harbor a favorable gene fusion. Sarcomas are malignant tumors of mesenchymal origin that are much more common in children than adults. Rhabdomyosarcoma is the skeletal muscle variant and is the most common subtype in children, accounting for approximately 3.5% of cancers in children ages 1 to 14 years . Other pAccording to the WHO classification of soft tissue tumors, there are four distinct subentities of rhabdomyosarcoma and they include embryonal, alveolar, pleomorphic, and spindle cell/sclerosing subtype. The majority of cases fall under the embryonal subtype which comprises botryoid and anaplastic variants. The embryonal subtype has the most favorable prognosis. The next most common subtype is alveolar, representing approximately twenty percent of cases. It tends to have a poorer prognosis because of its metastatic potential and is often diagnosed in the later stages. The pleomorphic subtype occurs almost exclusively in adults .Both CT and MRI can be helpful in diagnosing the tumor and evaluating the extent of disease. Tissue biopsy is required for definitive diagnosis and histologic classification. Treatment is individualized on a per patient basis and usually includes chemotherapy, surgery, and radiotherapy .A full-term female infant born to a healthy mother was found at delivery to have a soft tissue mass at the base of the left neck. Of note, the mass was diagnosed as a cystic hygroma in utero. An ultrasound at six days of life demonstrated a solid, ovoid, heterogeneously hypoechoic mass in the posterior left neck, adjacent to the paraspinal muscle measuring 3.2 × 1.4 × 2.1 cm . BecauseA follow-up ultrasound one month later showed stability of the mass in terms of size and imaging appearance. An MRI performed at six weeks old Figures  and 3 shRhabdomyosarcoma is a malignant neoplasm of mesenchymal origin which shows variable differentiation into striated skeletal muscle. Although relatively common in children, representing up to 7% of all pediatric malignancies, the diagnosis of congenital RMS in the neonatal period is rare. This case is unique in that the mass was present in utero as well as at the time of birth.Rhabdomyosarcoma has a variety of clinical presentations, the most common being a rapidly growing soft tissue mass. Depending on the location, the tumor may cause a range of clinical symptoms related to mass effect and obstruction. Tissue sampling is usually required to make a definitive diagnosis.Historically, RMS had been divided into three subtypes: embryonal, alveolar, and pleomorphic. The spindle cell variant was included within the embryonal category along with botryoid and anaplastic variants. Recent evidence, however, suggests that the spindle cell and sclerosing RMS may represent a discrete morphologic spectrum from the embryonal subtype because of similar clinical presentations and histologic features . SpecifiMuch work has been done on identifying gene rearrangements associated with the different subtypes of RMS. Alaggio et al. recently described a novel VGLL2 gene rearrangement which was found to be the most common genetic abnormality in congenital/infantile spindle cell RMS. VGLL2 is involved in regulating muscle-specific gene transcription and is exclusively expressed in skeletal muscle in the adult. Other genetic alterations include the NCOA2 gene fusion, which is common in congenital cases, and the MYOD1 gene mutation, which tends to occur in older children and adults. They found that cases which harbor gene fusions involving VGLL2 and NCOA2 had a favorable clinical outcome, whereas tumors associated with mutations in the MYOD1 gene showed a more aggressive course with a higher mortality despite multimodal therapy .Here, we describe a case of congenital rhabdomyosarcoma manifesting as a neck mass at birth. Because of its location on the posterior neck, our differential diagnosis included fibromatosis colli and solitary infantile myofibroma. Histologic sampling offered a definitive diagnosis and further characterized the tumor as the spindle cell variant of RMS. Furthermore, through FISH studies, we were able to identify the VGLL2 gene rearrangement which has been shown in recent studies to harbor a favorable clinical outcome.


Leiomyosarcomas (LMS) are rare malignant tumours of smooth muscle origin predominately affecting females in their sixth decade. Only 2% of LMS arise from blood vessels and most are in the inferior vena cava. We present the first reported case of LMS of the portal vein in a male patient. Multidetector CT showed a large mass in the main portal vein, which was initially misinterpreted as a pancreatic cancer. Careful examination of the multidetector CT images showed radiological features of an intraluminal mass, and a preoperative diagnosis of primary LMS of the main portal vein was made. The patient underwent curative surgery and made an uneventful recovery. Awareness of this entity and recognition of the salient CT features may facilitate radiologists in making the correct preoperative diagnosis. They predominantly affect females in the sixth decade of life and generally occur in the abdomen or the limbs. Only around 2% of LMS arise from the smooth muscle of the vessel wall, and the most common site is the inferior vena cava (IVC).6 In this article, we present the first reported case of LMS of the portal vein in a male patient with special emphasis on imaging findings seen on multidetector CT (MDCT) and positron emission tomography followed by a brief review of the existing literature.LMS of the portal vein is extremely rare with four previous reports in the English literature, all of which were in females.A 67-year-old male initially underwent contrast-enhanced multidetector CT of the abdomen in Sept 2014 at another hospital, which showed an incidental 4.2 × 3.9 cm mass in the upper abdomen. This was interpreted as a head of pancreas mass causing portal vein compression. The patient was asymptomatic; physical and laboratory examinations were all unremarkable.The patient was lost to follow-up and did not receive any further investigation or treatment. Although still asymptomatic, he re-presented 12 months later for a repeat MDCT, which showed that the mass had increased in size to 4.8 × 5.0 cm. Cavernous transformation of the portal vein was also observed. The patient underwent exploratory laparotomy, which showed a tumour at the upper border of the pancreas and duodenum compressing the portal vein. A 1-cm lesion in the subcapsular aspect of segment II of the liver was also seen. The presumed pancreatic tumour was deemed unresectable and multiple biopsies were taken. The liver lesion was resected and the histology of both samples showed spindle cell tumour. The patient also underwent a positron emission tomography-CT, which showed the lesion to be hypermetabolic with SUVmax of 7.3. No metastatic disease was identified.–1, alkaline phosphatase 54 U l–1, alanine aminotransferase 29 U l–1 and aspartate aminotransferase 23 U l–1). A repeat MDCT 2 months later showed that the lesion had a soft tissue density on non-contrast scans. The mass now measured 5.3 × 5.9 × 6.4 cm, extending from the superior mesenteric vein/splenic vein confluence to the porta hepatis. It exhibited heterogeneous enhancement with feeding vessels seen on the arterial phase, both within and around the mass. On the portal venous phase, numerous collaterals were seen surrounding the mass with a sharp interface between the mass and opacified portion of the main portal vein at the porta hepatis giving the appearance of a “beak”  was obtained from the patient(s) for publication of this case report, including accompanying images.


We report two rare cases of enchondroma protuberans originating from phalanxes.The patients visited doctors for a palpable mass in their phalanx without any pain or discomfort.Biopsy is the gold standard for the diagnosis of enchondroma protuberans. Radiographs usually provide important imaging information, while studied on the ultrasound manifestation of enchondroma protuberans are still limited. In our cases, significant information about ultrasound manifestation of enchondroma protuberans were presented. Sonographic examination of enchondroma protuberans revealed a hypoechoic mass located in and beyond the medullary cavity of bone through the interrupted bone cortex, and blood flow signals were usually not abundant.Patients were subsequently referred for surgical removal and the masses were confirmed by following pathological examination.After surgery, the patients recovered well with no relapse within 2 years.Enchondroma protuberans is a rare form of benign enchondroma. Enchondroma protuberans can present as an intramedullary hypoechoic mass extending to the surrounding soft tissue via the discontinuous cortex line on ultrasound. Ultrasound can provide important information for the diagnosis of enchondroma protuberans. Enchondroma protuberans is a rare type of enchondroma, defined as an exophytic enchondroma of bones because of its location; the phalanges or metacarpal bones are the most common location involved.,6 The main difference between enchondroma protuberans and the conventional enchondroma is the protruded mass. Therefore, the presence of an exophytic mass can clearly distinguish enchondroma protuberans from conventional enchondroma. The clinical symptoms of conventional enchondroma are subtler than those of enchondroma protuberans. Patients with conventional enchondromas can be asymptomatic, or can experience pain, and sometimes a pathological fracture, whereas enchondroma protuberans usually presents as a palpable mass in the phalanx. Radiographic imaging studies, such as X-ray, computed tomography (CT), and magnetic resonance imaging (MRI) on enchondroma protuberans are available, but those regarding ultrasound findings remain scarce. We retrospectively analyzed the clinical and sonographic features of 2 cases with exophytic masses that were confirmed pathologically as enchondromas in this article.Enchondromas are benign, cartilaginous tumors that are the most common (90%) benign bone tumors affecting the hand.2The 2 cases included were sonographically evaluated by using the iU22 scanner  equipped with a 12-5 MHz linear-array transducer. Both of the cases underwent mass resection and pathological examination, and recovered well after surgery with no relapse within 2 years. No extra intervention was involved in the 2 cases. Adverse and unanticipated events did not occur during the diagnosis and treatment progress. Complete clinical, ultrasonic, and pathological data were available. No family history or genetic history was found.2.1A 48-year-old woman noticed a palpable mass in the distal phalanx of her left ring finger with a medical history of enchondroma in the same location. Ultrasound examination revealed an exophytic hypoechoic mass that was connected to the medullary cavity through the interrupted bone cortex. No obvious blood signals or calcification were found in this area Fig. . It is d2.2A 25-year-old woman underwent ultrasound examination for a palpable mass in the middle phalanx of her left ring finger. A hypoechoic mass of 20 mm diameter was found. The boundary of the mass was unclear and punctuate or patchy hyperechoic calcification was present in the mass. The bone cortex was involved, with a broken continuity. In addition, a point-like blood signal was observed on switching to Doppler mode (Fig. 3 Unlike osteochondroma, the cartilage of the enchondroma protuberan was not a cap over normal trabecular bone, but extended outward from an intramedullary locus. A few small tumors or those with slow growth require close monitoring instead of surgery. Those with obvious symptoms, rapid growth, or pathologic fracture need timely surgery. The early detection of enchondroma protuberans is important.Enchondroma protuberans is usually caused by enchondroma expansion through the bone cortex leading to a single lesion in the phalanx. However, it has a much lower morbidity than conventional enchondroma. Enchondroma protuberans is a rare, benign chondromatous tumor that arises in the medullary canal, forming an exophytic mass in the surrounding soft tissue. For example, an enchondroma protuberans in a rib may present an eccentrically located, calcified mass expanding the bone on X-ray, and the junction of bone and cartilage is almost intact. A technetium-99 M radioisotope bone scan demonstrates slightly diffuse increased uptake. A specimen radiograph may demonstrate dense confluent calcifications in both the medullary and extraosseous portions of the tumor. The typical presentation of enchondroma protuberans in MRI is a well-defined intramedullary lesion with low signal intensity in T1-W image and high signal intensity in T2-W and short time inversion recovery sequences accompanied by cortical expansion and cortical defect.There are no exact diagnostic criteria for enchondroma protuberans, and biopsy provides the most convincing evidence for diagnosis. Radiographs can provide important imaging information. Radiologically, enchondroma protuberans is presented as a well-defined intramedullary osteolytic lesion and may be accompanied by fine matricidal calcification, cortical expansion and cortical defect, and round well-defined soft tissue expansion.Ultrasound shows great promise in musculoskeletal system imaging and can provide significant information in the diagnosis and detection of diseases. However, reports on ultrasound findings of enchondroma protuberans are limited. Therefore, sonographic appearances of enchondroma protuberans were analyzed and discussed in this article. which was in agreement with our findings. Moreover, blood supply of the tumors differed in the two cases. Generally, blood supply of benign bone tumors is always minimal and scattered, and enchondroma is no exception. In Doppler mode, blood signal could hardly be observed in case 1, while punctate signal was observed in case 2. In addition, biopsy of case 2 showed an active proliferation of a part of the tumor cells. This may indicate a relationship between blood supply and degree of tumor cell activity. A preliminary conclusion about ultrasound manifestation in enchondroma protuberans is achieved, that is, a hypoechoic mass appears in and beyond medullary cavity with an interrupted hyperechoic cortical line, while punctuate calcification and blood supply may exist. When the tumor cells proliferate actively, punctate and linear blood signal may be observed on ultrasound.The 2 cases with exophytic masses confirmed on pathological examination as enchondromas shared some similarities, for example, the lesions were solitary and closely related to the phalanges, which met the predilection sites of enchondroma protuberans. Sonographic appearance had certain characteristics Table . In both whereas enchondroma, including enchondroma protuberans, does not have a cartilaginous cap. Periosteal chondroma is a benign cartilaginous tumor of periosteal origin, and usually located beneath the periosteum and has no connections to the medullary cavity.,8,11 The absence of cartilaginous cap and underlying trabecular bone differentiates enchondroma from osteochondroma and delineation of contiguous intramedullary involvement can be a strong indicator to differentiate it from periosteal chondroma. Chondrosarcoma must be considered when distinguishing the properties of the lesion, especially in cases seen in costal bones, and more attention should be paid to low-grade chondrosarcoma. Most of the cartilage tumors present with characteristic features on imaging. However, it remains difficult to differentiate enchondroma and low-grade chondrosarcoma. Clinical symptoms, such as increasing size and onset of pain, may be of some help in diagnosis.Cartilage tumor is a mass closely related to bone with homogeneous echo and less blood supply, including osteochondroma, periosteal chondroma, and enchondroma. Osteochondroma is continuous with the bone, and typically has a cartilaginous cap outside the lesion and does not contain cartilaginous tissue in its base or in the medullary cavity,Despite the fact that ultrasound can provide important information for the diagnosis of enchondroma protuberans, it is still not as suitable as radiographs for the assessment of bone. In addition, radiographs cannot be replaced with ultrasound in the aspect of the diagnosis of enchondroma protuberans.4We report 2 patients of palpable masses on the phalanx, and we suspect that enchondroma protuberans can present as an intramedullary hypoechoic mass extending to the surrounding soft tissue via the discontinuous cortex line. Punctuate or patchy calcification and blood supply may occur in some cases. Appearance of blood signal may indicate tumor cell activity. Further study with a large sample size is needed to confirm it. Although ultrasound manifestation of enchondroma protuberans is atypical, awareness of enchondroma protuberans should be present when an exophytic mass originating in the medullary cavity is found on sonography.5This is a case report. And it is not necessary to obtain an ethics committee or institutional review board approval. Otherwise, written informed consents were signed by the 2 patients.Data curation: Xi Xiang.Funding acquisition: Li Qiu.Methodology: Xi Xiang, Li Qiu.Resources: Li Qiu.Software: Xi Xiang.Writing – original draft: Xi Xiang.Writing – review & editing: Shan Cheng, Yujia Yang.


As epidermoid cysts generally originate from hair follicle infundibulum, they appear as intradermal or subcutaneous tumors and are very rare in locations away from the skin. Here, we report a rare case of intertendinous epidermoid cyst of the forearm in a 68-year-old man that was treated surgically. Epidermoid cysts are common benign tumors presenting anywhere over the body , and areA 68-year-old right-handed Japanese man presented to our hospital complaining of a gradually growing swelling in his left forearm. He had noticed it for 3 years but did not remember any causal events. It was elastic, hard, and had poor mobility, without tenderness, and there were no findings suggesting its adhesion to the overlying skin. The patient showed neither motor impairment nor sensory disturbance of the hand and fingers. Magnetic resonance imaging (MRI) showed a unilocular and well-defined cystic mass (31 × 22 × 15 mm) in the forearm. It showed a slightly high intensity on both T1-weighted and T2-weighed images and was pushing out the extensor tendons . Based oExcisional biopsy was performed under general anesthesia with a tourniquet. The mass was detected under the deep fascia and was not connected to the skin. It was covered with a thick white capsule and its bottom was adjacent to the antebrachial interosseous membrane . As the Histopathological examination showed that the cyst wall was composed of stratified squamous epithelium with abundant keratin. The definite diagnosis was epidermoid cyst without malignancy .There have been no complications during a 6-month follow-up after surgery.Epidermoid cysts are common benign intradermal or subcutaneous tumors presenting anywhere over the body, including glabrous sites . The majIn the present case, however, the cyst was located under the deep fascia, and neither radiological examination nor intraoperative findings showed continuity between the cyst and the skin. Similar epidermoid cysts presenting deep in the body have been reported in the presacral region , spleen On the other hand, acquired epidermoid cysts have been reported to be derived from hair follicle infundibulum, trauma, surgery, spinal anesthetic injection, etc. . They arThe imaging features of epidermoid cysts vary according to the degree of cyst maturation, concentration, and amount of keratin . UltrasoIn the present case, the mass was detected under the deep fascia and was not attached to the skin. The patient had no history of trauma or surgery, and we found no evidence of such possible caused on physical examination. Based on these findings, a congenital origin was the most likely explanation for the presence of the epidermoid cyst under the deep fascia in this case.Epidermoid cysts can present at any location and any depth in the body. It is important not to exclude epidermoid cysts from diagnosis based only on a lack of continuity with the skin. Both US and MRI should be performed preoperatively.We encountered a rare case of epidermoid cyst presenting under the deep fascia. For masses in deep parts of the body, it is important not to exclude epidermoid cysts from the possible diagnoses.


This problem is further complicated by the lack of clinical suspicion in patients without any hematological disorder.•Accurate diagnosis of isolated MS requires a multifactorial approach including histopathology, immunophenotyping, immunohistochemistry and cytogenic abnormalities.•A high level of suspicion is necessary, because early, accurate diagnosis is important to avoid delaying appropriate chemotherapy. A delay in the diagnosis may result in unwarranted fatality particularly so in pediatric patients.•Here, we report an unusual case of MS initially presenting as a tumor of the temporal bone and first manifestation of AML, in a pediatric patient suffering from Down’s syndrome. This case illustrates the challenges associated with offering a rapid diagnosis with an early initiation of treatment. Leukemia is the most common malignancy of childhood but myeloid sarcoma is a rare presentation of underlying leukemic disorder. Myeloid sarcoma (MS) is a rare tumor composed of proliferation of myeloid precursors at extramedullary sites.We report an unusual case of myeloid sarcoma involving the temporal bone in a young male child who presented with a large mass involving the left temporal region. This lesion was the initial presentation which led to further diagnosis of acute myeloid leukemia in our case. This case report brings awareness to the diverse extramedullary manifestations of isolated myeloid sarcoma, as well as the importance and difficulties that are associated with establishing a rapid diagnosis and initiating treatment.They can arise de novo or in association with hematological malignancies, most commonly acute myeloid leukemia (AML-M2). Clinically, it can masquerade as an abscess, cutaneous ulcer, or as a mass lesion. Morphologically, MS can mimic a variety of small round cell tumors including lymphomas, neuroblatoma and rhabdomyosarcoma. The occurrence of this tumor usually heralds AML or the onset of the blastic phase of chronic myeloid leukemia. Early recognition of this rare entity is important, because early aggressive chemotherapy and focal irradiation can cause regression of the tumor and thus improve patient longevity.The possibility of MS should be considered when dealing with unusual lymphoma like neoplasms that cannot be categorized as any of the Non-Hodgkin lymphoma subtypes and small blue round cell tumors. It is also known as granulocytic sarcoma, extramedullary myeloid neoplasm, or chloroma and is a collection of immature myeloid cells at extramedullary sites. First described by Burns in 1811, the term “Chloroma” was coined by King in 1853 to describe this tumor entity based on its green color which is due to the enzyme myeloperoxidase (MPO) present in the myeloid cells . It occu2A 2 year-old male child was brought to pediatric outpatient department with swelling in temporal region and history of convulsions one week back. For which he was treated by some private practioner and was reffered at our centre for further management. The patient had a history of recent trauma. Considering this, X-Ray was advised, which revealed a mass lesion in left temporal region. Magnetic resonance imaging (MRI) of the head revealed a well-defined 5 cm × 3 cm enhancing lesion with altered signal intensity in the left temporal region. The lesion showed intense contrast enhancement with underlying bone erosion and involving the left cerebellopontine angle . ImagingFine needle aspiration cytology (FNAC) of the mass was performed which showed small, uniform, blue, round cells which at places were forming rosettes . ConsideExtensive haematological workup, including bone marrow biopsy and immunophenotyping, cytogenetics and positron emission tomography (PET) scan were performed, all yielded negative results and the patient was discharged from hospital without additional treatment and the decision to begin induction chemotherapy with daunorubicin and cytarabine was made. After a month patient returned for his treatment. At that time peripheral blood examination revealed 90% atypical cells/blasts conforming to morphology of myeloid blasts . Child w3Leukemias are the commonest pediatric malignancies with 15%–20% of cases originating from myeloid origin . PatientOn CT scans, MS are generally homogeneously isoattenuating to slightly hyperattenuating. On post contrast studies they exhibit uniform homogenous enhancement.When the bone is affected, granulocytic sarcoma lesions appear lytic rather than sclerotic . MR imagMyeloid Sarcoma has to be differentiated from other morphologic mimickers which include Non-Hodgkin lymphoma (NHL) , poorly differentiated carcinoma, blastic plasmacytoid dendritic cell neoplasm (BPDCN), and small round blue cell tumors such as RMS and neuroblastoma. The differentials vary depending on the age of the patient and site of occurrence. IHC has an important role in this regard. CD68KP1 is the most frequently expressed marker in MS, followed by MPO, CD117, CD99, CD68/PGM1, lysozyme, CD34, terminal deoxynucleotidyl transferase (Tdt), CD56, CD61/linker of activated T lymphocyte/ factor VIIIrelated antigen, CD30, glycophorin A, and CD4. Expression of CD 117 has been associated with poorer outcomes. NHLs are positive for LCA and either Tor Bcell markers. MS may show weak LCA positivity but will be negative for B and Tcell markers. Carcinomas are generally cytokeratin positive. BPDCN shows cutaneous involvement and is positive with CD4, CD56, and CD123 [Owing to their rarity, there are currently no guidelines on whether to begin or delay treatment in patients without the involvement of the bone marrow. It has been documented that delayed or inadequately treated isolated MS will almost always progress to AML, with a median time of 5–12 months. There exist a variety of treatment options including chemotherapy, radiotherapy (RT), surgical excision, HSCT, or any combination of these treatments. In patients with isolated MS, treatment with AML-based induction regimens had complete remission rates comparable to those with AML without MS and they prolonged disease-free survival from 3.5 to 16 years. Induction chemotherapy with cytarabine and daunorubicine has been reported to induce complete remission in 65%–75% of patients. Radiotherapy could be used for consolidation along with systemic chemotherapy. A recent study suggested that RT may prolong failure-free survival but not overall survival in patients presenting with isolated MS. Surgery could be an option for tumors causing organ dysfunction and/or obstruction. However, systemic therapy should be considered in all patients as soon as the diagnosis is confirmed to prevent or prolong relapse and progression to acute leukemia . The mea4MS are extremely rare tumors with an aggressive clinical course. Morphologically, they can mimic small round cell tumors. This problem is further complicated by the lack of clinical suspicion in patients without any hematological disorder. Accurate diagnosis of isolated MS requires a multifactorial approach including histopathology, immunophenotyping, immunohistochemistry and cytogenic abnormalities. A high level of suspicion is necessary, because early, accurate diagnosis is important to avoid delaying appropriate chemotherapy. A delay in the diagnosis may result in unwarranted fatality particularly so in pediatric patients.We have no conflict of interest.Nil.Ours is a single case report, therefore, it was exempted by the ethics committee.Written informed consent was obtained from the patient’s parents for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-chief of this journal on request.Study concept: Dr. Nisha Marwah.Writing the paper: Dr. Namita Bhutani.Data interpretation: Dr. Rajeev Sen.Pictures: Dr. Archana Budhwar.Not applicable.Dr. Namita Bhutani.Not commissioned, externally peer-reviewed.


Ewing's sarcoma (ES) is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells. Primary ES commonly occurs in early childhood or adolescence. It may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is a case report of sinonasal ES in a 13-year-old female patient who presented with a 7-month history of right nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss. Clinical examination revealed a right nasal mass pushing the septum to the left side and extending to the nasopharynx. Endoscopic biopsy and histopathological analysis showed a small blue cell tumor suggestive of ES. The patient was treated with surgery, radiotherapy, and chemotherapy. After a follow-up of 5 years, the patient remains recurrence-free with excellent functional status and quality of life. Ewing's sarcoma (ES) is a highly malignant and rare tumor that subdivided into skeletal and extraskeletal entities , 2. The On March 2010, a 13-year-old female patient referred to the ENT clinic complaining of right sided nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss for 7-month duration. There was no history of trauma, anorexia, or weight loss.Clinical examination revealed a right sided nasal mass pushing the septum to the left side and extending to the nasopharynx. On throat examination, the soft palate was pushed down by the nasopharyngeal mass. Otoscopy showed dullness and retraction of tympanic membrane bilaterally. Cranial nerves examination was normal. No cervical lymph nodes were palpable. The results of hematological and biochemical investigations were within normal limits.On radiological evaluation, CT scan revealed an opacification of the right nasal cavity, maxillary, ethmoidal, sphenoid, and frontal sinuses with bone remodeling of the septum to the left side . SubsequES is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells , 5. It wES is a rare disease that accounts for only 4% to 6% of all primary bone tumors , 7, 8. FClinical manifestations of sinonasal ES include enlarging mass, nasal obstruction, rhinorrhea, and epistaxis , 9. ApprThe prognosis depends on the site of the primary tumor, the presence of distant metastasis at presentation, and the age of the patient , 6. ReseSuccessful treatment of ES includes a multidisciplinary approach with surgery followed by adjuvant radiotherapy and chemotherapy . The recEwing's sarcoma rarely affects the sinonasal tract. Diagnosis of the disease is challenging hence it requires histopathological examination, immunohistochemical studies, and cytogenetic studies. Treatment includes a multidisciplinary approach with surgery followed by chemotherapy and radiotherapy.


A 38-year-old Japanese housewife who was a heavy smoker was admitted to our hospital because of upper respiratory distress that developed half a year prior to admission. She had no past medical history. On admission, her vital signs were normal. Physical examination showed hoarseness, stridor, wheezing, and orthopnoea, but no swelling of the tonsils, thyroid, or lymph nodes. A computed tomography of the neck revealed an enlarged tumor .Laryngeal endoscopy revealed a giant vocal cord polyp . Direct What do we already know about this clinical entity?Vocal cord polyps are common lesions, and most are small; the common symptom is hoarseness.What is the major impact of the image(s)?Giant vocal cord polyps can mimic asthma and may cause critical airway obstruction leading to sudden death.How might this improve emergency medicine practice?Large vocal cord polyp can mimic asthma. Thus, physicians should consider the disease when patients present with a protracted upper respiratory distress history.Vocal cord polyps are common lesions, with a reported lifetime prevalence of 1.31% to 16.9% of the population.5Documented patient informed consent and/or Institutional Review Board approval has been obtained and filed for publication of this case report.


Central giant cell granuloma (CGCG) is classified by the World Health Organization as a benign bone lesion. It is found anteriorly in the mandible, with most of the cases crossing the midline. In total, 70% of CGCGs are encountered in young females. Fibro-osseous lesions are a group of pathologies that encompass neoplastic, dysplastic and reactive entities. Juvenile ossifying fibroma, which can be further categorized into juvenile trabecular ossifying fibroma (JTOF) and juvenile psammomatoid ossifying fibroma, represents an aggressive neoplastic example of these fibro-osseous lesions. JTOF occurs in children at almost equal ratios in both sexes, affecting the maxilla more than mandible. This study aims to report a peculiar case of a hybrid lesion comprising CGCG and JTOF in the mandible of a nine-year-old female patient. Clinical, radiographic and histopathological findings were assessed. Clinical examination revealed an intraoral swelling extending from the right impacted third molar area to the left first molar area. Computed tomography showed a well-defined multilocular radiolucency with diffuse flecks of radioopacities. Histopathologically, the lesion comprised fibrous connective tissue encompassing numerous multinucleated giant cells and other areas of cell-rich connective tissue stroma containing bands of osteoid matrix and anastomosing immature bone trabeculae intermixed with scattered clusters of multinucleated giant cells. We hereby report a case of a rare hybrid lesion comprising CGCG and JTOF. It occurs anteriorly in either jaw but is more commonly encountered in the mandible. The majority of cases manifest in children or young adults and are twice as frequent in females than males3. It attains a quite benign behavior, although rare aggressive lesions have been documented3.Central giant cell granuloma (CGCG) is believed to be an entity exclusive to jaw bones4. These lesions encompass varying entities including neoplastic, dysplastic and reactive pathologic processes1. Ossifying fibroma (OF) and its subtypes represent their neoplastic entity. Juvenile OF (JOF) is an aggressive subtype of ossifying fibromas that can be further classified into juvenile trabecular OF (JTOF) and juvenile psammomatoid OF5. JTOF occurs in children and adolescents with equal distribution of cases among both sexes. It affects the maxilla more than mandible, while other skeletal bones are rare to be affected with such lesion3.The term “benign fibro-osseous lesions” generically describes an aggregate of pathologies that are characterized by replacement of normal bone with a highly cellular connective tissue associated with newly formed bone trabeculae6. As for JOFs, despite their non-encapsulated nature, they appear well delineated, with early lesions found as unilocular radiolucencies. However, old lesions more commonly assume the multilocular configuration7. Depending on the degree of ossification within the lesion, JOF can present varying extents of radioopacities within its radiographic picture. We can then clarify that those precedent features often challenge the diagnosis and management of such lesions. We hereby report a case of a hybrid lesion that contained both of the previously mentioned entities.Radiographically, CGCGs usually present as well-demarcated radiolucencies that are multilocular, although unilocular lesions have been notedA nine-year-old female patient was reported to the Oral and Maxillofacial Surgery Department at the Faculty of Dentistry, Cairo University, complaining of a painless slowly growing swelling in the anterior area of the mandible of one year’s duration, causing buccal and lingual bone expansion, displacement and looseness of the incisors . Her med3 of the lesion through 6 weekly injections, upon which the lesion showed marked regression.The histopathological examination using routine H&E staining demonstrated heavily scattered multinucleated giant cells in a background of highly cellular fibrous stroma consisting of mononuclear stromal cells and extravasated red blood cells. Newly formed bone trabeculae and osteoid tissue were noted at the periphery of the lesion . AccordiSix months later, the swelling significantly increased in size, causing severe facial disfigurement. Intra-orally, it developed a brownish red discoloration and was non-tender but displaced the otherwise clinically sound related teeth. Computed tomography (CT) of the lesion showed it to be a well-defined multilocular radiolucency with diffuse flecks of radioopacities. It extended from the right impacted third molar area to the left first molar area causing perforations in the buccal and lingual cortical plates .The lesion underwent an excisional biopsy. On gross examination, it consisted of almost 30 pieces that ranged in size, with an average size of 2×2×1.5 cm. They were reddish in color, some were hard and others were soft in consistency . The speFollowing treatment, the patient was administered 50 mg diclofenac potassium tablets through oral administration, three times daily for one week. The patient responded well to both the surgery and post-operative treatment. The healing was uneventful and no worrying findings were observed through the 6-week follow up.9. It is a pathology that more commonly affects young females. Some authors like Chuonget al.10 and Ficarraet al.11 tend to classify it into aggressive and non-aggressive subcategories, due to varying clinical courses of reported cases12. Similarly, it can represent varying radiological pictures ranging from undulated unilocular to multilocular radiolucencies13.Central giant cell granuloma (CGCG) is a benign intra-bony lesion that was first introduced to the medical literature by Jaffé in 195314. It is an entity that affects individuals younger than 15 years of age with no sex predilection15. Radiographically, it is shown as a well-circumscribed unilocular or multilocular radiolucency that may exhibit ground-glass or even more discrete opacifications16.JOF is an aggressive, non-odontogenic, benign tumor of the bone17. Later, it was generally accepted that “hybrid” lesions represent those lesions that comprise significant portions of histopathological features of multiple, completely different entities20. We believe that this reported case represents a hybrid lesion. Previous cases of hybrid CGCGs associated with different fibro-osseous entities have been reported in the literature. Until now, to our knowledge ten cases of hybrid lesions have been documented. Out of them, five cases were of CGCG associated with ossifying fibroma, three cases of CGCG with fibrous dysplasia and one case was of CGCG with cemento-osseous dysplasia21. To our knowledge, excluding this case, only one analogous case has been reported22.The term “hybrid lesions” was first introduced by Waldron and El-Mofty during their work on cases of ameloblastomaet al.23 Moreover, in juvenile ossifying fibroma, multinucleated giant cells and areas of hemorrhage can be found scattered in its sometimes hypercellular fibrous matrix7, characteristics that can undoubtedly confuse investigators upon making a diagnosis and accordingly, choosing a treatment plan. An additional fact that may further complicate case management is the presence of hybrid lesions.As regards to the histopathological picture of the two lesions, In some instances, CGCG can show confined peripheral areas of reactive bone and osteoid formation in its highly vascular and cellular connective tissue stroma, as indicated by De Corsoet al.24, the authors state it may just be a coincidental finding or correlated by representing a “reactive process rather than a feature of a separate lesion”. While Farzanehet al.25 and Penfoldet al.26 assume that “osteoblasts may activate osteoclast-type giant cells through paracrine mechanisms”27. Accordingly, a debate has risen between two opinions, whether JOF had developed in a more central position first and then initiated an adjacent giant cell reaction or whether two lesions simply originated independently.The association of giant cells with fibro-osseous lesions could be explained on the basis of different postulations made by many authors. In the case report of ShettyConsidering this specified case, we were startled by the prominent divergence in the course of the lesion; i.e., its response to the selected treatment option. It raised multiple questions and so a decision was made to put the intrigue at ease by embarking on research to find answers. It was firstly suspected that there was a concealment of some histopathological features in the previous biopsy that could direct us to accurate deductions on such a lesion. This instinct was fortunately confirmed. By returning to the histopathological slides of the previous incisional biopsy, very minimal marginal areas of the lesion did indeed contain areas of fibrous stroma with few immature bony trabeculae, which led to the thought that they could in fact be indicative of juvenile ossifying fibroma, of the trabecular subtype, rather than what was previously thought to have been regions of an inflammatory bodily reaction in the form of ossification. Bearing that deduction in mind, along with the results of the recent biopsy, and correlating this to the clinico-radiographical information obtained, the case was identified as a hybrid lesion of both CGCG and JTOF.Owing to the small number of reported hybrid lesions, their biologic behavior cannot be predicted and hence their treatment is uncertain. As for the chosen treatment in our case, no other surgical intervention was made in an attempt by the surgeon to be as conservative as possible considering the young age of the patient. The surgeon also refrained to prescribe any pharmacological agents with the exception of analgesics three times daily for one week after surgery. The patient was given the appropriate self-care instructions following surgery in front of her guardian and the latter was asked to report the patient to the operating surgeon for follow up. The healing was uneventful for six weeks, but the patient was unfortunately lost for the planned three, six and twelve-month follow up.et al.22 To our knowledge, our case is the second to be documented as a hybrid lesion involving CGCG and JOF. Therefore, we believe that this report would significantly contribute to the understanding and clinical management of such a rare lesion.To conclude, in our search of the literature no cases of similar hybrid lesions were reported, except for one reported by GeethaAll data underlying the results are available as part of the article and no additional source data are required.Written informed consent for publication of clinical details and clinical images was obtained from the guardian of the patient. (JTOF) The authors present a hybrid case report of CGCG, with juvenile trabecular ossifying fibroma. The authors should mention the soft ware used to do the surgical planning , was it a CBCT? It is recommended that the authors provide a high power representative of the heavily cellular stroma and pseudoaneurysmal spaces, characteristic of the Juvenile trabecular ossifying fibroma (JTOF). Add reference to support the triamcinolone acetonide injection. In the discussion the authors need to reflect more on the differential diagnoses, that the bone deposition may be part of a reparative granuloma histologically, yet the rapid clinical expansion speaks against a reparative process. That JTOF can be associated with MNGCs, yet the incisional biopsy had enough evidence of a CGCG component to support a hybrid lesion.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above. Authors have described an uncommon case of  Introduction is relevant, adequate and properly written. a  case report is described in proper sequence with investigation and diagnosis with justification. Enough details are provided about the case.  Sufficient discussion is included depicting the importance of the findings and their relevance to future understanding of disease processes The overall case report is interesting and useful to readers.I confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard. In keywords: Fibrous dysplasia should be deleted.Please check the keywords to be according to MeSH system.In introduction: Last sentence of second paragraph about JTOF? In most references psammomatoid type of the JOF is more common in craniofacial skeleton than trabecular typeFigure 1: It would be better to add panoramic view or CBCT of the lesion.Figure 6C: It would be better to use higher magnifications for showing osteoblasts and cellular osteoid. The first sentence of the “Discussion” needs appropriate citation. The original citation needs to be applied to address Jaffé, 1953 original article.Is this case the first hybrid case of CGCG and JTOF? If not, please provide a literature review of the same cases in a table.It is recommended to summarize previously reported cases of hybrid CGCG in a separate table.major revision.The manuscript needsI confirm that I have read this submission and believe that I have an appropriate level of expertise to confirm that it is of an acceptable scientific standard, however I have significant reservations, as outlined above.


Despite the decline in mortality rates over the last 20 years, cancer remains one of the leading causes of death in children worldwide. Early recognition and treatment for acute oncological emergencies are vital in preventing mortality and poor outcomes, such as irreversible end-organ damage and a compromised quality of life.Imaging plays a pivotal and adjunctive role to clinical examination, and a high level of interpretative acumen by the radiologist can make the difference between life and death. In contrast to adults, the most accessible cross-sectional imaging tool in children typically involves ultrasound. The excellent soft tissue differentiation allows for careful delineation of malignant masses and along with colour Doppler imaging, thromboses and large haematomas can be easily identified. Neurological imaging, particularly in older children is an exception. Here, computed tomography (CT) is required for acute intracranial pathologies, with magnetic resonance imaging (MRI) providing more definitive results later.This review is divided into a ‘body systems’ format covering a range of pathologies including neurological complications , thoracic complications , intra-abdominal complications  and haematological-related emergencies . Within each subsection, we highlight pertinent clinical and imaging considerations.The overall objective of this pictorial review is to illustrate how primary childhood malignancies may present with life-threatening complications, and emphasise the need for imminent patient management. Paediatric cancers differ from adult malignancies in their type, prevalence and location and therefore knowledge of tumour behaviours is crucial in predicting acute complications.Conventional radiography can be helpful in suspected bowel perforation, bowel obstruction or airway obstruction from mediastinal masses; however, ultrasound and CT are frequently more informative.MR imaging, whilst excellent for characterising and staging a primary malignancy, has less of a role in the emergency scenario apart from in neurological emergencies, such as suspected spinal cord compression.Cancer remains one of the leading causes of death in children, after 1 year of age –4. NeverThis pictorial review serves to illustrate the many facets and scenarios seen during emergency presentations of paediatric cancers. We outline the abnormalities and best imaging modalities for each scenario by body systems and include cases seen at presenting diagnosis, prior to curative surgical resection and unrelated to chemotoxic agents. Acute presentations relating to complications of treatment and infective diseases are not included, given their substantial coverage elsewhere in the literature –10.Tumours of the central nervous system are the leading cause of cancer-related deaths in children , with thPresenting symptoms can be variable depending on age and commonly non-specific in nature. On the whole, intracranial malignancies have a subacute onset and commonly present with changes in mood, headache or intermittent seizures . In rareFocal neurology and stroke-like symptoms are more likely from tumour haemorrhage or venous infarction from cerebral venous sinus thrombosis (CVST) which although rare, has been associated with childhood intracranial malignancies . In one Imaging protocols for acute intracranial oncological pathologies are similar to those in children without suspected malignancies. Cranial ultrasound is a quick and accessible screening test in patients with patent fontanelles. Ventricular dilatation, a mass lesion or focally abnormal parenchymal echotexture in the context of stroke or haemorrhage, can be easily and quickly demonstrated. Colour Doppler can easily assess the superior sagittal sinus for thrombosis. Unenhanced computed tomography (CT) allows for rapid identification of intracranial shift, haemorrhage, oedema and hydrocephalus, and enhanced CT (in the form of CT venography) can be used to demonstrate deep sinus thrombosis or arterial compromise. Ultimately, neurosurgical consultation should be obtained, with magnetic resonance imaging (MRI) used for further lesion characterisation , particuAcute spinal cord compression occurs in 3–5% of children with cancer at diagnosis , usuallyPotential causative lesions commonly include neuroblastomas 3).Fig. In the above scenarios, plain radiographs of the spine are generally unhelpful. MRI imaging is the ideal modality and should include the entire neuraxis (the brain and whole spine), with sedation and analgesia occasionally required to allow for adequate patient positioning. Following the initial imaging of the brain, an additional dose of gadolinium contrast agent is not required—the dose provided for post-contrast brain imaging is normally sufficient for the spine given that both body areas are usually imaged within the same sitting, lasting less than 1hour in length .Causes of proptosis include benign and malignant pathologies. Orbital cellulitis is often clinically apparent with CT being used to confirm post-septal extension. MRI examination through the orbits is often definitive for benign aetiologies, with orbital haemangiomas and pseudotumour having characteristic MR appearances . The mosThe smaller diameter and compressible nature of the trachea in children places them at increased risk of airway obstruction with anterior mediastinal masses. Common aetiologies include lymphoma, leukaemia and germ cell tumours. These tumours may also cause vessel compression  resultinCommon symptoms of airway obstruction include cough, stridor and dyspnoea , with poPlain chest radiographs identify mediastinal masses in 97% of cases, if present  and aid Maintaining a calm presence, minimal child handling and obtaining a tissue diagnosis via the least invasive method possible is recommended . GeneralIn the antenatal period, obstetric sonography may identify a potentially fatal airway obstruction from a large neck mass, commonly a cervical teratoma  Fig. 9)9). AlthoAlthough malignancy is a rare cause, pleural effusion may be seen at diagnosis in 15% of children with non-Hodgkin lymphoma and is a marker for poor therapeutic response and relapse. Suggested causes include venous obstruction from bulky disease, leading to a decreased venous return to the heart and reduced lymphatic drainage of the thoracic duct if the left brachiocephalic vein is involved . PleuralCauses of pericardial effusion in paediatric oncology patients are myriad ranging from general medical causes of cardiac failure, side effects of chemotherapy, radiotherapy or haematopoietic stem-cell transplantation  to maligMass effect on the diaphragm from an intra-abdominal mass may cause splinting of the diaphragms, restricted self-ventilation and poor gaseous exchange. Causes can include gross hepatomegaly from primary hepatoblastoma or metastases in stage MS neuroblastomas which may require urgent intubation and ventilatory support Fig. . AscitesAbdominal compartment syndrome is a potentially life-threatening complication carrying a mortality rate of up to 60% , 53. TheThe diagnosis is clinical; however, by identifying venous compression, particularly IVC obstruction on either an abdominal ultrasound or portal venous phase, post-contrast CT study, the radiologist can help raise awareness . SurgicaBowel obstruction is frequently caused by intussusception. Whilst relatively common in healthy children with median age at presentation of 8 months , occurreBowel perforation may occur secondary to bowel obstruction, intestinal tumour infiltration  or) or61]) Biliary rhabdomyosarcoma (RMS) is a rare primary malignancy, but the most likely primary tumour to cause biliary tract obstruction, and can be accompanied by abdominal distension and hepatomegaly , 63. OccThe diagnosis of biliary RMS remains challenging and first-line imaging is primarily focussed on ultrasound identification of biliary dilatation, with location of a soft tissue mass, frequently within the common bile duct . FurtherPelvic masses such as rhabdomyosarcomas, neuroblastomas, sacrococcygeal teratomas and germ cell ovarian tumours in girls may present with distal ureteric obstruction and subsequent upper urinary tract dilatation Fig. . Mass afWhilst a rare complication (with respect to adults), the incidence of thromboembolism (both deep venous thromboembolism and pulmonary emboli) in children with cancer is still higher than that seen within their healthy counterparts. One British study estimated the thrombosis risk at 1.52 per 1000 person-years for children with cancer (95% CI = 0.57–4.06) versus 0.06 per 1000 person-years (95% CI = 0.02–0.15) in children without cancer [hazard ratio of 28.3 (95% CI = 7.0–114.5)] . OverallDoppler ultrasound imaging of the extremities, including venous compression techniques should be adopted to identify suspected limb thromboembolism, whilst CT pulmonary angiography (CTPA) is suitable for suspected pulmonary emboli. Contrast-enhanced CT head for venous sinus thromboses have been discussed earlier in this article. At present, there are no specific guidelines for thromboprophylaxis treatment in children with cancer. It is therefore even more important for radiologists to be astute to the presence of potential thromboembolism in children.Disseminated intravascular coagulopathy (DIC) is characterised by excessive activation of blood coagulation and consumption of clotting factors. It is found in children with disseminated metastases or secondary to AML , where iWhilst a pre-contrast CT of the affected body part may be routinely acquired in adult radiology, this is generally avoided in paediatric imaging with only the arterial and portal venous phase imaging being sufficient .End-organ damage or haemorrhagic stroke secondary to refractory hypertension may be the presenting feature of some paediatric malignancies. These can be biochemical in nature (such as from excessive catecholamine production in neuroblastomas or phaeochromocytomas) or from mass effect on the aorta or renal arteries Fig. .Fig. 21Whilst vascular compression from a mass may be investigated with ultrasound examination and spectral Doppler analysis, definitive characterisation with cross-sectional imaging and angiography should be performed for surgical planning and/or endovascular intervention.Familiarity with life-threatening oncological emergencies is essential in contributing to the continued low rates of mortality and morbidity in children. Given that most children suffering from an acute complication may not initially present to a specialist centre, it is the responsibility for all general radiologists to have a basic awareness of such cases. In this article, we have provided an overview of the various ways in which oncological complications manifest and the main imaging considerations. Ultimately a multidisciplinary approach with good team communication and prompt action are all required to ensure the best imaging and treatment strategies going forward.


A 7-month-old girl was referred to the head and neck department with a history of gradual painless swelling over the left side of her face for 4 months. Physical examination revealed asymmetric appearance of the face, with a 120 × 70 mm non-tender, non-mobile, and non-pulsatile prominent soft tissue mass with a smooth contour in the left parotid and periauricular spaces. Neurological examination was unremarkable.On ultrasound examination, a solid cystic mass in the parotid space was recognized, but precise determination of the depth, margin, and extension of the mass was not possible, and complementary diagnostic studies were necessary.MRI of the skull base and face  in axialVascular or lymphatic malformation, teratoma, encephalocele or meningocele, neoplastic lesion (such as rhabdomyosarcoma), and adenoid cystic carcinoma were considered in the differential diagnosis . The patExtracranial glial choristoma is a rare non-teratomatous tissue that has been reported in two common sites, both distant from the neuraxis. Infratemporal glial heterotopia, also recognized as glial choristoma, is a congenital non-neoplastic migration of neuroglial tissue to an aberrant location. Glial choristoma is usually seen at birth or within the first few years of life, but can be present in any age group .The most common site for these lesions is around the nasal cavity, but they may be seen in locations as varied as the scalp, head, neck, and lung . Glial cCT and MRI are two important imaging modalities that should be used in the study of such masses to ascertain the anatomical site and extension of the lesion, and to rule out any communication with neural tissue. Surgical resection is the main treatment method for glial choristoma, but the lesion must be identified and distinguished from an encephalocele or other intra- and extranasal masses before surgery is planned.Histopathologically, glial choristomas consist of neuroglial fibers and astrocytes associated with vascularized and fibrous connective tissue. The presence of plentiful neurons increases the probability of an encephalocele . A few gConservative surgical resection is sufficient for neuroglial choristomas, and the few reported cases of recurrence were related to inadequate resection . In the Our case was of interest not only because of the rarity of choristoma, but also due to its rare location in the infratemporal fossa, without intracranial communication and with normal brain tissue.The authors declare that they have no competing interests.


Leiomyomas represent about 3.8% of all benign soft tissue tumors. Vulvar localization is very rare. We present a case of a vulvar leiomyoma and discuss diagnostic and therapeutic features of this disease. A 30-year-old female patient with no medical history, had a 5cm mass located in the left large lip causing a discomfort at the perineum especially in sitting and walking. She underwent a complete surgical excision of the mass. The pathological examination confirmed the diagnosis of a leiomyoma. There was no recurrence after 24-months' follow-up. The vulvar leiomyoma is a rare benign tumor. The diagnosis is made only postoperatively after resection of the mass. The treatment is essentially based on total excision of the mass with a good prognosis. Leiomyomas represent about 3.8% of all benign soft tissue tumors . They caA 30-year-old female unmarried patient, with no medical history, having had menars at the age of 13 years, with regular cycles, had consulted for a vulvar mass which appeared recently since 3 months and increasing gradually in volume and causing a discomfort at the perineum especially in sitting and walking. On vulvar examination, there was a firm multilobulate pedicled mass measuring 5cm located at the posterior surface of the left large lip . The supet al found a single fibroid after examining 144 vulvar tumors [et al. reported 25 cases of fibroids of the vulva. Most cases presented a painless mass. But sometimes there were symptoms of pain, itching, erythema [Leiomyomas are frequent benign monoclonal tumors developed from smooth muscle cells . Their er tumors . Preopererythema . SurgicaThe vulvar leiomyoma is a rare benign tumor. The diagnosis is often made only postoperatively after resection of the mass. Several hypotheses have been put forward to explain their origin, but the exact etiology remains unidentified. The treatment is essentially based on total excision of the mass.The authors declare no competing interests.


Escherichia coli infections in giant neonatal scalp hematoma.Neonatal scalp mass is common in clinical practice. After birth canal compression and traction force, a cephalohematoma is usually found. However, cephalohematoma with abscess is extremely rare and dangerous. So far, there have been no reported cases of multidrug-resistant We present a 9-day-old with a scalp abscess and a large scalp defect that remained after surgical drainage.Physical examination showed a giant mass suggestive in the parietal region. B-mode ultrasound indicated the scalp mass was liquid. The early diagnosis was massive scalp hematoma. During conservative treatment, purulent fluid flowed from the mass region through a rupture in the scalp. MR examination showed the scalp had burst and no abnormalities were found in the medial side of the skull and skull.The surgeon opened up the mass and removed necrotic tissue. The scalp was severely damaged; the aseptic auxiliary materials that we made in-house were used to gradually reduce the defect.The scalp was healed by anti-infection treatment and frequent changing of the dressings. The patient was successfully treated without two-stage surgery. There were no complications.A scalp hematoma is a potential site of infection. Anti-infection treatment and surgery are necessary to correct infected scalp hematoma. This work offers a new way of treating other large scalp defects. The risks of neonatal infection include low gestational age, inhaled amniotic fluid, and intrapartum fever. Neonatal scalp mass is common in clinical practice. A cephalohematoma is often found after birth canal compression and tractive fetus. Abscess following infection of scalp hematoma is extremely rare. The factors affecting infection include direct hematogenous seeding, scalp contusion, and fetal monitoring. Surgery and anti-infection treatment are necessary for abscess. Here, we present an infected scalp hematoma with abscess formation and conservative treatment of large scalp defects in a full-term infant.Neonatal sepsis associated with microorganisms from the mother can be lethal.2Written informed consent was obtained from the parents of the patient for publication of this case report and any accompanying images. A copy of the written consent form is available for review by the editor of this journal.9/L, neutrophilic granulocyte percentage (NEUT%) was 72.1%, procalcitonin (PCT) was 0.3 μg/L, but the blood culture was not back. Chest radiography showed changes to the lung texture and enlargement of the lungs. Fever was considered for neonatal pneumonia or hematoma absorption. Cefuroxime sodium (25 mg/kg every 8 hours) was administered by intravenous infusion. Polypnea developed and fever (T40°C) persisted on Day 5 of life, physical and drug for symptomatic treatment. WBC count was 18.74 × 109/L, CRP increased to 163 mg/L, PCT was 1.5 μg/L, and the results of lumbar puncture were normal. E coli that extended-spectrum β-lactamases was positive resistance to cefuroxime sodium and levofloxacin and sensitive to piperacillin sulbactam and meropenem, was detected in blood culture and sputum. Chest radiography showed changes in lung texture and lung enlargement. Biochemical examination was performed on the puncture fluid of the mass. The skin of the mass became discolored locally during anti-infective therapy. Piperacillin sulbactam (280 mg every 6 hours) was administered by intravenous infusion. On the second day after administration of antibiotics, the body temperature dropped. At 9 days after birth, pus burst out from the discolored skin (Fig. E coli was detected in the pus and puncture fluid cultured. The scalp abscess was cut open, local necrotic tissue was removed, and pus was cultured before and after the surgery. Culture indicated Escherichia coli showed the same antibiotic sensitivity as the isolate from the blood culture. After the operation, there were 2 major scalp defects, 3 × 2.5 × 0.5 cm and 1.5 × 1.0 × 0.7 cm (Fig. A full-term infant girl was born by spontaneous vaginal delivery to a 27-year-old mother in a rural hospital. The infant's birth weight was 3.02 kg. After birth, a giant mass was observed over the parietal area. For treatment of the scalp mass, she was transferred to Mianyang Central Hospital. There was no intrauterine distress or premature rupture of membranes in prenatal examination. Breathing, amniotic fluid, umbilical cord, and placenta showed no abnormalities intrapartum. APGAR scores were unknown. A mass about 10 × 10 cm in size was found over the parietal area, and there was no scalp wound. The mass was soft, elastic, and high tension, not extending beyond the cranial suture. Blood analysis and coagulation function were within normal limits. B-mode ultrasound indicated the scalp mass was liquid, the size was about 10.8 × 11.2 × 2.6 cm (Fig. 3E coli was not multi-drug resistant. In this paper, fever appeared on the third day after birth, and blood culture detected multidrug-resistant E coli was found in skin ulceration secretion culture and surgical incision of pus culture on Day 9 of life. These suggest that the blood-borne pathogen infection and sepsis are closely associated with large abscesses of the scalp. The scalp abscess is usually confined to scalp tissue but it may be accompanied by osteomyelitis or intracranial infection.,6 MR and lumbar puncture examinations were normal. Hematomas can sometimes combine abscesses, so a culture should be performed of any secretions or puncture fluid. Due to past overuse of antibiotics in China, there are more and more superbugs infecting patients. It is very important to select antibiotics according to the culture results. Although the structures of subcutaneous abscesses, subgaleal abscesses, and subperiosteal abscesses are different, surgical debridement and sensitive antibiotics are key to treatment.Scalp hematoma occurs most commonly after blunt instrument injury, premature delivery, primigravida delivery, or error by a midwife. The body often reabsorbs a scalp hematoma without intervention, but some large hematomas require puncture extraction and compression bandaging. A few hematomas need to be cut open to remove the hematoma and stanch the flow of any fluids. Neonatal scalp hematoma can be complicated by anemia, hypovolemic shock, infection, and other problems. In the absence of such risk factors as aspiration, scalp contusion, and fetal monitoring, bacterial infections in giant neonatal scalp hematoma are extremely rare. Currently, only one case of infected scalp hematoma associated with sepsis in neonates has been reported, and the  Due to the special dome morphology of the scalp, methods of scalp reconstruction mainly include direct suturing, scalp expansion, and free flap.,9,10 Since direct suturing requires a wide separation of loose connective tissue, most scalp defects cannot be sutured directly like skin defects in other parts of the body, except for a small number of small avulsion wounds. Tissue expanders are one way to treat large-area skin defects. Scalp expansion has the disadvantages of local skin necrosis, dilator exposure, local infection, and insufficient skin expansion, and treatment may require multiple surgeries. Free flap can be highly traumatic, time-consuming, and costly. In this paper, we describe a new method that is simpler, involves less pain, and reduces expenses. When changing fresh dressing for draining abscess, sterile auxiliary material is used to pull on the skin around the defect and reduce its area and ultimately repair the skin. This technique significantly shortens the treatment cycle, and reduces the need for unnecessary and expensive medical treatment.Scalp defects are a common complication after debridement of scalp abscess. There has been one case reported of scalp abscess associated with sepsis. This report did not list the size of the scalp defect or any subsequent treatment.Conceptualization: Zongping Li.Investigation: Liling Yang, Xu Yang.Methodology: Hongyuan Liu.Project administration: Zongping Li.Resources: Yan Zhang, Jia Chen.Supervision: Jia Chen.Validation: Yan Zhang.Writing – original draft: Hongyuan Liu, Xu Yang, Jia Chen.Writing – review & editing: Hongyuan Liu.Hongyuan Liu orcid: 0000-0001-9580-9715.


Although postoperative abdominal drains are useful in therapeutic settings, their prophylactic role is debatable. We herein describe the case of a 30-year-old male who underwent bile duct resection with hepaticojejunostomy for cholangiocarcinoma. On postoperative day four, the patient developed biliary peritonitis. Explorative laparotomy revealed an obstruction of the afferent limb caused by an intestinal loop around a Jackson-Pratt (JP) drain. Removal of the drain resolved the obstruction which led to a significant improvement of the patient’s clinical state. To the best of our knowledge, this is the second report of a bowel obstruction from a surgical drain. When placing abdominal drains, surgeons must take into consideration their indication as well as possible related complications, including intestinal obstruction.  Prophylactic intra-abdominal drain placement following surgery aims at draining intraperitoneal collections and reducing the risk of subsequent infection . HoweverA 30-year-old male with no significant past medical or surgical history presented with cholestatic jaundice. An abdominal ultrasonography (USG) revealed an 11-mm obstructive lesion in the common bile duct with intrahepatic duct dilatation. An attempt at stent placement to relieve jaundice failed during endoscopic retrograde cholangiopancreatography (ERCP). A magnetic resonance cholangiopancreatography (MRCP) subsequently showed no gallstones but revealed a 7.0 mm segmental stenosis of the common hepatic duct with intrahepatic bile duct dilatation. The lesion was further characterized with an endoscopic ultrasonography (EUS), revealing a 19.0 mm lesion at the common hepatic duct, suspicious for a cholangiocarcinoma (Klatskin tumor). Thoracic, abdominal, and pelvic scans revealed no evidence of metastatic disease and no vascular involvement. The tumour was therefore deemed resectable. The patient underwent an extended right hepatectomy, cholecystectomy, extrahepatic duct resection, and lymphadenectomy, followed by a Roux-en-Y hepaticojejunostomy Figure .Two prophylactic JP drains were placed at the right upper quadrant around the anastomoses to monitor for postoperative anastomotic leaks. The procedure was successfully performed and well tolerated by the patient without any immediate post-operative complications.The pathology report later confirmed a perihilar extrahepatic bile duct adenocarcinoma. No evidence of lymph node involvement or metastatic disease was found.On postoperative day four, the patient became febrile and complained of abdominal pain and vomiting. Physical examination revealed diffuse rebound tenderness was present, with maximal tenderness at the upper right quadrant. Bile was noted in both JP drains. Elevated cholestatic markers were noted on laboratory results. An urgent abdominal CT scan revealed a severe dilation of the proximal jejunum (up to 5.2 cm) with a possible transition point at the level of the proximal jejunum. A hypodense zone suggesting a perianastomotic collection of 5.0 cm x 3.0 cm was noted. The radiological images suggested an anastomotic leak secondary to the obstruction of the afferent intestinal loop, making biliary peritonitis the most likely diagnosis. The patient underwent urgent exploratory laparotomy. A significant intestinal distention was noted upon opening of the abdomen. Surprisingly, the proximal jejunum was looped around one of the JP drains close to the hepaticojejunostomy Figures -4. Removal of the drain led to the spontaneous resolution of the obstruction and resulted in normal drainage of the intestinal content into the distal intestine. The patient was hospitalized for several weeks due to multiple complications, including gastroparesis and transient hepatic insufficiency. After significant clinical improvement, the patient was discharged from the hospital with a closely scheduled follow-up appointment.The use of surgical drains has been practiced for many years and was even described in the era of Hippocrates for drainage of empyema and ascites . In the Prophylactic drains address intraperitoneal collections such as bile, blood, ascites and intestinal or pancreatic secretions. The accumulation of such biological liquids could lead to intraabdominal infection . Bile anHowever, despite its advantages, placement of a prophylactic drain remains debated in many instances. While it does not offer clinically significant benefits for most abdominal surgical procedures  , 3-4, 6,Additionally, abdominal drains could be associated with complications, including hemorrhage, bacterial infection, hernia formation, foreign body reactions, and postoperative pain , 7-8. OtBowel obstruction is another very rare complication from intraabdominal drains. Shah et al. reported a case of a 78-year old male whose small bowel was twisted around a JP drain after undergoing low anterior resection with loop ileostomy for rectal cancer . The obsTo the best of our knowledge, this is the fourth case report ever published in the literature on intestinal obstruction caused by a surgical drain and the first such case following a hepato-pancreato-biliary procedure Table .On preoperative imaging, a close trajectory of the JP alongside the afferent loop was noted without clear evidence pointing out the drain as the cause of the intestinal obstruction. Indeed, the etiology of the obstruction on imaging was only noted retrospectively. This case serves as an example of how unlikely it is to suspect a drain as a cause of intestinal obstructions. All initial interventions of the four cases (including the following report) on intestinal obstruction from an abdominal drain were elective procedures. Of the four published cases on intestinal obstruction from an abdominal drain, only one case resolved nonoperatively . Indeed,Although therapeutic drainage of intraabdominal collections is beneficial, the role of prophylactic drainage following abdominal interventions remains controversial. There is currently growing evidence that prophylactic drain placement is not beneficial after different surgical interventions. Placement of prophylactic drains must be individualized. When placing them, the surgeon must take into consideration the possible related complications, including hemorrhage, infection, and rarely intestinal obstruction. Finally, with advances in surgical technique, easy access to antibiotics, radiological imaging and drainage, indications for routine prophylactic drains should be re-evaluated.