Ganglioneuroblastoma (GNB) is a transitional tumor of sympathetic origin that has never been described as primarily involving the orbit. Herein we report an extremely rare case of GNB with primary orbital involvement and its treatment strategies.A 9-year-old girl presented with progressive and recurring right orbital mass for 2 years.Computed tomography (CT) showed a well-defined, well-circumscribed, and homogeneous extraconal soft tissue mass occupying most of the right superior orbital area. Magnetic resonance imaging (MRI) revealed that there was a neoplasm of the right superior orbit molding around the globe with long T1 and T2 signals, and contrast-enhanced MR image showed a heterogeneous enhancement of the mass. Histopathologic examinations were performed after surgery and the characteristics were consistent with a diagnosis of GNB.Surgery was performed and the mass was completely resected.Postoperatively, the patient was on a regular follow-up for 19 months and so far, has had no orbital mass recurrence.Herein we present a rare case of GNB primarily involving the orbit, and the findings showed that GNB could originate from the orbit. The patient underwent surgical tumor resection. The histopathological and immunohistochemical features were consistent with the diagnosis of GNB. For this case, there was no recurrence for 19 months after complete surgical excision of the tumor; however, a regular long-term follow-up is required. The 2 extremes of NTs represent the most malignant neuroblastomas and the most benign ganglioneuromas. In between lie GNBs, which are characterized by a mixture of cells ranging from primitive neuroblasts to well-differentiated ganglion cells within neurofibromatous tissues. GNBs can be further sub-classified into 2 types: nodular and intermixed. It is a transitional tumor of sympathetic origin, which has not yet been described as primarily involving the orbit. The most common sites of GNB origin are the adrenal medulla, extra-adrenal retroperitoneum, and posterior mediastinum. Herein we report a rare case of a 9-year-old girl with a primary GNB of the orbit who responded favorably to surgical treatment.Neuroblastic tumors (NTs) are one of the most common solid tumors in children, which are derived from primitive cells of the sympathetic nervous system and arise from the neural crest.2This case report was approved by the Ethics Committee, and written informed consent was obtained. A 9-year-old girl was referred to our hospital in December 2018 because she presented with progressive and recurring right orbital masses for 2 years  and magnetic resonance imaging (MRI) scans of her orbit were performed Fig. B-E. CT sSurgery was performed under general anesthesia through an anterior orbitotomy via a lid crease incision. The incision was below the arch of the eyebrow and on the surface of the mass. Orbital exploration revealed a circumscribed lesion with red–yellow surface without necrosis Fig. . The masPostoperatively, there was no injury to her visual acuity and no limitation of eyeball movements of the right eye. The patient had a smooth course, and was on regular follow-up for 19 months and so far, has had no orbital mass recurrence.3 NTs differ in their degrees of cellular and extracellular maturation; immature tumors tend to be aggressive and occur in younger patients, whereas mature tumors occur in older children and tend to behave in a benign manner. GNB has an intermediate malignant potential, as it is composed of both mature gangliocytes and immature neuroblasts. The presence of immature tissue in GNB indicates malignancy or potentially malignant behaviors. As 1 type of NT, GNB also arises from the sympathetic chain. The most common sites of origin of GNB, in decreasing order, are the adrenal medulla (35% of cases), extra-adrenal retroperitoneum (30%–35%), and posterior mediastinum (20%); the less common sites are the neck (1%–5%) and pelvis (2%–3%).,6 Case reports on GNBs are extremely rare, especially with orbital involvement. Herein we report a very rare case of GNB with primary orbital involvement. Most GNBs in other sites excrete large amounts of vanillylmandelic acid. Due to the expression of vasoactive intestinal peptide, a syndrome of failure to thrive and intractable watery diarrhea may occur with these tumors. In our case, the patient did not have any other symptoms besides the orbital masses and had normal growth. In addition, the body check and imaging showed no mass in other sites. Based on these findings, we could conclude that this was a rare case of GNB with primary orbital involvement. However, this patient still needs longer follow-up to ensure that there is no metastasis.NTs are a group of tumors that can be seen in the neck, posterior mediastinum, adrenal gland, retroperitoneum, and pelvis. For imaging GNB, MRI and CT scanning are preferred methods. On MRI, GNB is typically heterogeneous, variably enhanced, and of relatively low signal intensity on T1-weighted images and high signal intensity on T2-weighted images. As for this case, it showed atypical images, and the diagnosis mainly relied on histopathologic examination and immunohistochemical staining. In this case, the mass contained both mature ganglion cells and neuroblastoma. H-E staining showed that neuroblasts were immature and undifferentiated sympathetic cells, which were small, rounded in contour, showed little cytoplasm, and possessed darker nuclei and smaller indistinct nucleoli, while ganglion cells were fully mature cells with abundant cytoplasm, rounded contour, and large nuclei with distinct and prominent nucleoli. For immunohistochemical staining, Syn and S-100 are generally positive in Ganglioneuroblastoma. In this case, the histopathologic examination and immunohistochemical staining characteristics were consistent with that of ganglioneuroblastoma.Because GNBs arise in various locations, they have varied appearances and growth patterns. The diagnosis mainly relies on clinical symptoms, imaging, histopathologic examination, and immunohistochemical staining. this case was classified as stage 1. GNB is widely seen as a malignant entity and is treated with multimodal therapy. Complete surgical excision of the tumor is one of the therapeutic options. Decarolis et al showed no disease progression after complete resection and subtotal resection with residuals smaller than 2 cm. Other studies also presented the proposal that resection did not have to be radical for the treatment of GNB, especially when considering surgical complications.,13 However, immature areas of the tumor should be sufficiently resected in the initial surgery since there is a possibility that the large tumor masses contain immature components, which may be sources of disease progression. Chemotherapy seems not to be indicated for patients with localized GNBs and the cytotoxic treatment has no substantial effect, whereas surgery alone is sufficient for the treatment of GNB and does not need to be radical if only minor residuals are left. As for this case, the patients first surgery might have been insufficient to remove the mass and might cause tumor recurrence. However, we could not rule out the possibility that we were dealing with a new tumor.The treatment choice for GNBs mainly relies on the stage of the disease. According to the International Neuroblastoma Staging System (INSS), As reported in other cases, GNB originated from other sites with orbital metastases, and the prognosis was reportedly poor, with one large series of findings with only an 11.2% survival rate. However, in our case, the patient had no sign of orbital mass recurrence and metastasis until now. We suppose that this was because it was a localized primary orbital involved GNB without tumor metastasis, which could be completely resected and the Ki67 labeling index was 1%. However, it should be emphasized that regular long-term follow-up is warranted.GNB, histologically containing primitive neuroblasts, is therefore considered malignant or potentially malignant. The prognosis is uncertain, as they can undergo spontaneous regression or spontaneous differentiation to benign neoplasms or exhibit malignant behaviors, especially in older children.In summary, we present a rare case of GNB primarily involving the orbit. The patient underwent surgical tumor excision, and the histopathological features were consistent with the diagnosis of GNB. For the diagnosis of this case, imaging of the mass might not show typical images, and the diagnosis mainly relied on histopathologic examination and immunohistochemical staining. For this case, there was no recurrence for 19 months after complete surgical excision of the tumor, but the patient should undergo a regular long-term follow-up.YZ collected the data of the patient, consulted literatures, dealt with the Figs and wrote the manuscript; WMH was the Consultant in charge for the case, established the diagnosis, performed the surgery and approved the submitted version. All authors read and approved the final manuscript.Data curation: Yi Zhang.Formal analysis: Yi Zhang.Project administration: Weimin He.Supervision: Weimin He.Writing – original draft: Yi Zhang.Writing – review & editing: Weimin He.


Acute pancreatitis is a common clinical disorder of the pancreas that can present with walled-abdominal pain and vomiting. We report a case of a 45-year-old man with alcohol-related acute pancreatitis who developed a large walled-off pancreatic necrosis. Incidentally, the patient had a small gastrointestinal stromal tumor in the adjoining wall of the stomach that was missed on preoperative radiological imaging. A small submucosal lesion was detected during cystogastrostomy and was excised. The diagnosis of a gastrointestinal stromal tumor was confirmed on histopathology. Acute pancreatitis is a commonly encountered pancreatic disease in clinical practice. Most cases are mild but about 10%-20% of cases can develop severe acute pancreatitis. Severe acute pancreatitis is associated with significant morbidities and mortality. Walled-off pancreatic necrosis (WOPN) is a frequent sequela of severe acute pancreatitis. Most of these patients have persistent abdominal pain, fever, vomiting, or jaundice [A 45-year-old male presented to our emergency department with a two-day history of melena, fever, and abdominal pain. He had a past history of severe anemia (hemoglobin (Hb) - 5 gm/dl) requiring four units of blood transfusion three months back. At the same time, he was diagnosed with DVT for which he was taking oral anticoagulants. He was a chronic alcohol abuser consuming about one quarter of alcohol every day for 15 years. On clinical examination, the patient was pale, tachypneic, and had tachycardia with normal blood pressure. There was no calf tenderness or pedal edema. An ill-defined, non-tender lump was palpable in the epigastric and left hypochondriac region. Blood investigations revealed severe anemia and coagulopathy ). Contrast-enhanced computed tomography (CECT) of the abdomen revealed a well-defined, bilobed WOPN of 8 x 8 x 11 cm with multiple air foci within it. The part of WOPN abutting the gastric wall showed increased focal vascularity suspected to be due to collateral formation , bilateral common iliac veins, and left external iliac vein suggestive of thrombosis. Esophagogastroscopy found a large pulsatile bulge in the gastric fundus with a mild irregularity of the overlying mucosa. Since our center did not have an endoscopic ultrasound, and there was a risk of bleeding due to coagulopathy and extensive collaterals during endoscopic drainage without endoscopic ultrasound, surgery was planned.On laparotomy, there was a large collection in the retrogastric space pushing the stomach anteriorly. After anterior gastrotomy, a 3 cm prominent bulge was present in the posterior wall of the stomach. The collection was punctured through the posterior gastric wall, and about 500 ml of foul-smelling pus was drained. While creating the cystogastrostomy opening, a 3 cm submucosal lesion was noted in the gastric wall Figure . The lesHistopathological examination of the submucosal lesion revealed GIST arising from the stomach Figure .Imatinib therapy was not started due to the small size and complete resection of the lesion. At the last follow-up at 17 months, the patient was symptom-free and on oral anticoagulation.GISTs are the most common mesenchymal tumors of the gastrointestinal tract, with the most common site being the stomach . Rarely,Another unique feature of the index case was the presence of extensive DVT. Venous thrombosis involving portosplenic circulation is frequently present in patients with pancreatic necrosis due to various factors such as mass effect from the inflamed pancreas, stasis, and increased levels of procoagulant inflammatory mediators . Venous The index case had severe anemia on presentation mainly due to recurrent bleeding from the eroded gastric mucosa overlying the WOPN. However, there can be multiple contributory factors responsible for anemia in WOPN or acute necrotizing pancreatitis. These factors include bleeding pseudoaneurysms , hemorrhThe treatment of WOPN includes medical therapy with antibiotics, percutaneous drainage, endoscopic transgastric drainage, and surgery. Small WOPN (< 5 cm) resolves spontaneously with medical therapy. The preferred procedure for a large WOPN (> 5 cm) is endoscopic ultrasound-guided transgastric drainage. However, the presence of extensive collaterals, pseudoaneurysm, and hemodynamic instability are relative contraindications for endoscopic drainage. Moreover, endoscopic drainage requires advanced endoscopic equipment and expertise. Open or laparoscopic internal drainage is the treatment of choice in most cases. In the present case, due to coagulopathy, the presence of collaterals, and the lack of equipment, surgical drainage was performed.WOPN can lead to various uncommon complications such as anemia and extensive deep venous thrombosis. CECT or endoscopic ultrasound should be routinely performed and carefully studied to rule out the presence of any incidental co-existent submucosal lesions, such as GIST, before planning the treatment strategy.


Multiple point duodenal atresia is an extremely rare condition with atretic segments in either two or three sites of the duodenum. We report a newborn male patient who presented to our institution with bilious vomiting, nonpassage of meconium, mild abdominal distension, and a palpable epigastric abdominal mass ∼1 × 1 cm. A faint double bubble was found on abdominal X-ray. On exploratory laparotomy, a duodenal cyst due to double duodenal atresia was found and a typical diamond-shaped duodeno-duodenostomy was created. A postoperative contrast study revealed passage of the contrast media into distal intestine. However, the patient died 2 weeks later due to uncontrolled sepsis and pneumonia. Despite the fact that multiple-point duodenal atresia is a rare condition, it should be considered as a differential diagnosis to avoid missed pathology. Multiple duodenal atresia is an extremely rare condition that may be either double or triple. To our knowledge, only 24 papers in the English literature reported this anomaly. Of these, two patients had triple duodenal atresia and the remainder had a double duodenal atresia.Duodenal atresia is thought to occur by failure of embryological recanalization. However, a multiple atresia is difficult to explain with this theory. Yoshida and Migita described it as a result of a malrotation that causes a twist and results in pressure necrosis and atresia in more than one point.This rare deformity has different presentations ranging from neonatal intestinal obstruction in case of complete atresia to infantile presentation with incomplete intestinal obstruction. This is associated with or without a palpable abdominal mass.A male newborn  presented with bilious vomiting. Clinical examination revealed mild epigastric distension with the passage of mucous on digital rectal examination. A palpable epigastric firm mass measuring 1 × 1 cm was found. General examination revealed severe respiratory distress due to meconium aspiration pneumonia with associated signs of sepsis; however, there was no sign of peritonitis. Meconium aspiration pneumonia was diagnosed after reviewing the records of delivery that revealed meconium-stained amniotic fluid in association with immediate postnatal respiratory distress.Laboratory investigations revealed hypochromic microcytic anemia with moderate leukocytosis in association with mild hypokalemia and metabolic alkalosis. Hanging abdominal X-ray showed a hugely distended stomach with a faint double bubble sign and absent gas distribution in the remaining abdomen as shown inThe mother had an antenatal ultrasound that revealed full stomach in association with polyhydramnios.After stabilization of the septic patient, a right supraumbilical explorative laparotomy was performed. We performed a Kocher maneuver and found a cyst between the first and the second part of the duodenum as shown inA longitudinal duodenotomy distal to the cyst was performed and the ampulla of Vater was identified. We advanced a 6-inch French tube through this incision into the distal intestine with injection of warm saline to exclude further distal intestinal obstructions. Then transverse duodenotomy proximal to the cyst was applied and we found a duodenal diaphragm at the proximal end of the cyst causing partial duodenal obstruction; this diaphragm was resected. Another complete duodenal atresia was found at the distal end of the cyst that explained the development of this cyst between a proximal partial obstruction and a distal complete obstruction. This cyst was bypassed by a diamond-shaped duodeno-duodenal anastomosis between the transverse duodenotomy proximal to it and the longitudinal duodenotomy distal to it.Stool was passed on the 5th postoperative day; however, the child still had bilious reflux, so we performed a contrast passage study, which showed that the contrast passed to the distal intestine freely. However, with a remaining proximal dilatation the decision to delay oral intake was taken .Unfortunately, the baby progressively deteriorated due to the meconium aspiration pneumonia that was resistant to antibiotic treatment. The condition was associated with severe uncontrollable sepsis that was progressed to septic shock, disseminated intravascular coagulation (DIC), and multiorgan failure that ended with death of the child 15 days postoperativelyDouble duodenal atresia is an extremely rare condition occurring in 1:5,000 to 1:10,000 live births; Stringer et al published a series of four patients and the postoperative survival was reported in three cases and only one case died due to the associated congenital heart disease.It usually occurs as a sporadic congenital anomaly and only two reports documented its presence in siblings.Besides the previously mentioned presentations, double duodenal atresia may present postoperatively after the treatment of a first point duodenal obstruction by intestinal obstruction, perforation, and/or abdominal cyst. This usually develops if the patency of the distal intestine was not ensured during the first operation.In our case, a cystic dilatation between the first and the second part of the duodenum was found during operative exploration. This is consistent with other similar studies discussing the same topic like that one belonging to Stringer et al, which was published in 1992.Endoscopic incision of double duodenal webs has been advocated by some surgeons as a new treatment modality in infants presenting with partial double duodenal diaphragms. However, it is not feasible in newborns because a minimum weight of ∼8 kg is mandatory for endoscopic intervention as reported by Barabino et al. These authors recorded a successful endoscopic release of duodenal web in a 11-month-old baby (8 kg).Reviewing the records of our patient during delivery revealed that delayed cord clamping was not done in this condition; this may be the cause of hypochromic microcytic anemia. Our patient died 2 weeks postoperatively from meconium aspiration pneumonia that was resistant to antibiotic treatment and associated with uncontrollable sepsis. The condition progressed to septic shock, DIC, and multiorgan failure that ended with death of the child. Surgical exploration and anastomosis are usually curative in all cases. Mortality is only known due to other causes, such as associated cardiac malformations or pneumonia as in our patient.Double duodenal atresia is a rare condition. Operative detection of one atresia does not preclude the presence of further distal one, so thorough examination of the distal intestine with a tube and warm saline injection in case of intestinal atresia is mandatory to exclude other distal obstructing lesions.


Endolymphatic sac tumor (ELST) is one of neuroectodermal tumor which arising from endolymphatic sac and duct. It is actually quite rare, with less than 200 cases reported. Although ELST presents benign appearance in histopathology, it can present aggressive destructive behavior in clinical. The cornerstone of treatment for ELST is complete surgical excision. However, it is almost impossible to completely resect the advanced stage tumor. There is still controversy about other treatments, such as radiotherapy and gamma knife surgery.A 47-year-old man was admitted in The First Affiliated Hospital of Fujian Medical University with a 7-year history of progressive hearing loss and near 6-month repeated attacks of headache. Preoperative CT revealed a massive intracranial lesion and associated hydrocephalus. MR scanning demonstrated a 7.2 cm × 4.6 cm × 4.2 cm bulky mass located in left-sided posterior cranial fossa and temporo-occipital region which showed hyperintensity on T1-weighted images and mixed signal intensity on T2-weighted images. There was no neither clinical manifestation nor family history of Von Hippel–Lindau syndrome (VHL).Due to the mass that was large and invading the bone of skull base, it was difficult to extirpate surgically, so the ventriculoperitoneal shunt combined with local biopsy was performed. The postoperative pathology and immunohistochemical findings confirmed the lesion was an endolymphatic sac tumor. After operation, the patient regularly received radiotherapy.The widely accepted management of ELST is complete surgical resection. However, it is difficult for surgeons to achieve radical resection with late-stage ELST. Currently, there is much dispute about the role of radiotherapy for the management of ELST in academic circles. In this case where the mass cannot be surgical removed, radiotherapy has the curative effect for ELST in terms of disease control and quality of life. Endolymphatic sac tumor (ELST) is an exceedingly rare tumor of the epithelium of endolymphatic ducts and sacs (EDS) with less than 200 cases reported. Although histologically benign, ELST presents clinically invasion behavior because of the disease’s potential for locally infiltration and destruction of bone. It can be occurred in isolation or rather, in simultaneity with Von Hippel–Lindau syndrome (VHL) . The incThe patient was a 47-year-old man, from The First Affiliated Hospital of Fujian Medical University, Department of Neurosurgery with the chief complaint of progressive left hearing impairment for almost 7 years. Over the past 6 months, the symptom was aggravating that accompanied with repeated occipital headaches. On physical examination, the left ear entirely suffer hearing loss. The hearing of the right ear was normal. The patient presented facial paralysis (House-Brackmann grade II) and hypoglossus paralysis. There was no tinnitus, otalgia, otorrhea, vertigo, and history of trauma or surgeries. In addition, the patient had no definite symptoms or family history of VHL disease.Cerebral computed tomography (CT) findings revealed a bulky enhancing soft tissue mass (maximum transverse diameter was 7.2 cm × 4.6 cm) of left-sided posterior cranial fossa and temporo-occipital region tha t had eroded the skull base bone of middle and posterior cranial fossa Fig. a, b. LinBecause of the tumor that was too large which invading nearby structures, it was difficult to excise completely. Finally, the ventriculoperitoneal shunt combined with local biopsy was performed to cure the hydrocephalus. Postoperative pathology analysis revealed that the tumor cells mainly showed papillary arrangements Fig. a. At higThere was no neurologic deficit in the early stage after operation, and the patient got out of the hospital at 7 days postoperatively. Adjuvant radiotherapy was given for palliative treatment postoperatively. In his postoperative 12-month follow-up, interval re-examination MRI scan revealed that the mass remained progression-free and was not leaded to any problem.Endolymphatic sac tumor is a rare form of benign tumor arising from endolymphatic duct and sac (EDS) . It has ELST can be occurred in isolation or rather, in simultaneity with Von Hippel–Lindau (VHL) disease. VHL disease was named for the German ophthalmologist Eugene Von Hippel and the Swedish pathologist Avrid Lindau, who respectively described the disorders in 1904 and 1927, but it was not widely recognized until 1970s. VHL disease is an autosomal dominant genetic disorder characterized by cysts and benign tumors of multiple systems with potential for subsequent malignant transformation. By researching the genomics and proteomics, the mechanism of VHL disease has been identified. The development of VHL disease has been attributed to mutations of the Von Hippel–Lindau tumor suppressor (VHL) gene which encodes a tumor suppressor, VHL protein (pVHL), located at the short arm of chromosome 3 3p25-p26) [-p26 [5].The ubiquitous clinical symptoms of ELST are one-sided sensorineural hearing loss (SNHL) which may mimick Meniere’s disease, and its mechanism attributes to progressive of tumor associated with either the pressure of adjoining auditory structures or endolymphatic hydrops. It is a gradual process, although acute onset has also been reported. Patients may also present tinnitus, vertigo, aural fullness, and cranial neuropathies, which consist of cranial nerve paralysis, jugular foramen syndrome , and cerebellopontine angle syndrome  , 4, 8.Representative radiographic findings of ELST are displayed as destruction of bone and markedly inhomogeneous enhancement on CT scan. The neoplasm is situated in the region between posteromedial petrous bone and the sigmoid sinus, which intratumor and posterior rim calcification can be often seen. On MRI, ELST reveals heterogenous signal intensity on T1WI and T2WI. The hemorrhagic areas with sediment of methemoglobin and hemosiderin are markedly hyperintense on T1WI and T2WI. The hypointense areas may attribute to local necrosis, calcifications, or residual bone. In enhanced scan, ELST shows a heterogeneous intensity on T1WI and displays flow voids on T2WI , 10. AngThe major characteristics of pathology of ELST are low-grade, locally destructive, richly vascularized, ill-defined margins. The gross of ELST representatively appears as soft, reddish-blue polypoid lesion; microscopically, ELST shows two fundamental forms: papillary and follicular. The papillary pattern is arranged by simple cuboidal or columnar neoplastic epithelium cells. By comparison, the follicular pattern exhibits cystic filled with colloid protein material. Some cases had been reported that ELST may present both patterns , 13. CytThe histological differential diagnoses of ELST include middle ear carcinoma, choroid plexus papilloma, paraganglioma, papillary ependymoma, metastatic renal cell carcinoma, and metastatic thyroid papillary carcinoma. The foremost of those is the middle ear carcinoma which expands within mesotympanum, without causing bone invasion and destruction. However, a middle ear carcinoma is different than an ELST in that its cells show distinct pleomorphism and more conspicuous mitotic activity. The differences between ELST and the remaining entities previously described can be distinguished by immunohistochemical staining. ELST is considered to originate from intratemporal portion of the endolymphatic sac which is of neuroectodermal embryogenesis , 14; theSurgery is still recognized as the cornerstone of treatment which there was no ever reported recurrences after complete surgical resection, whereas subtotal resection may result in local recurrence. But, entire resection of a late-stage ELST is often not possible due to the anatomic complexity and distinct patterns of extension. The common choices of the operative approach include the transmastoid, posterior fossa, or retrolabyrinthine-transdural approaches , 17. TheIt has been recommended that at least 10 years of follow-up investigations of ELST patients should consist of annual MRI scanning. However, there is no consensus as to what follow-up should be arranged for ELST patients, although genetic evaluation is deemed necessary to evaluate the future development. There is no treatment protocol for the patients of unresectable or subtotal resections, into which research should be urgently needed, especially late-stage ELST.We present this rare case to aim at the better understanding and awareness of this extremely rare neoplasm. Although surgery excision, as standard treatment has been widely accepted, there has been controversial in unresectable cases. Due to the lack of evidence in the literature concerning the role of radiotherapy of unresectable ELST, we would like to put forward that radiotherapy could also control condition progressing; however, there is need to be long follow-up to assess the long term effect of radiotherapy. A multidisciplinary treatment approach including neurosurgeons, radiologists, neuropathologists, and radiation oncologists should be provided to patients with ELST to achieve adequate control of these locally aggressive tumors.


Streptococcus dysgalactiae. Through this case, we want to showcase this rare cause of IE and also highlight systemic embolization as its possible initial presentation.Infective endocarditis is a rare disease and is associated with a high mortality rate. The following case describes a 56-year-old gentleman who presented to our hospital with a pulseless left leg concerning for acute limb ischemia, which was managed with emergent revascularization. His subsequent workup revealed IE due to a rare organism known as  Staphylococcus aureus, with this organism representing 31% of all IE cases [Infective endocarditis (IE) is a rare and life-threatening disease. There has been an increase in the incidence of IE in the previous decade, with the incidence being 15 per 100,000 population in 2011 in the United States [The most common presenting feature of endocarditis is fever (96%), with the vascular embolic event being a presenting feature in less than 17% of the cases . EmbolizStreptococcus dysgalactiae.We present a case of acute IE with the initial presentation of systemic embolization from a rare cause of IE, ⸰F), normotensive, tachycardic (117 beats/minute), and saturating well on room air.A 56-year-old gentleman with a past medical history of untreated hepatitis C infection, osteomyelitis with right fourth toe amputation (wound cultures positive for beta-hemolytic G streptococci), and alcohol abuse (12 beers per day) presented with paresthesias and a pulseless left leg. He was noted to be febrile (102.93/uL and later peaked at 33×103/uL), thrombocytopenia (70×103/uL), international normalized ratio <1.5, and creatinine 1.22 mg/dL. Two sets of blood cultures were positive for Streptococcus dysgalactiae. CT angiogram of the left limb  is a human pathogen that belongs to Lancefield groups C and G, which forms large beta-hemolytic colonies on blood agar [Streptococcus dysgalactiae.se of IE . Streptoood agar . Many clood agar . Our casStaphylococcus aureus, Candida species, HACEK organisms, and Abiotrophia organisms [Systemic embolization of vegetation occurs in 22%-50% of cases with IE, with the highest embolic complications seen in people with the involvement of the mitral and aortic valves with rganisms . In a strganisms . Our casAn echocardiogram is an important tool in the diagnosis of IE. Transthoracic echocardiogram and, if high suspicion is present, a transesophageal echocardiogram can be obtained to demonstrate the presence of an oscillating intracardiac mass, annular abscess, prosthetic valve dehiscence, or a newly developed valvular regurgitation, which fulfill the major criteria for IE . The diaBased on the guidelines of the American Heart Association, surgical intervention is essential in patients with persistent vegetation after embolization, >10 mm size of the anterior mitral leaflet vegetation, one or more embolic events during the first two weeks of antimicrobial therapy, or an increase in vegetation size despite therapy as a potential need for surgical intervention [Streptococcus dysgalactiae. We also want to emphasize the importance of prompt recognition and management of IE. Despite multiple comorbidities, the underlying infectious source was controlled by surgical intervention in our patient leading to stabilization and ultimately increasing longevity.Through this case, we hope to highlight the diversity in initial presentations of IE, especially when caused by an atypical organism such as


Splenic vein stenosis and occlusion, which are known causes of left-sided portal hypertension, often occur secondary to trauma, pancreatitis, or invasion or compression by pancreatic tumors. However, few reports have described idiopathic splenic vein stenosis.A 70-year-old man was referred to our hospital for examination of isolated gastric varices. He had no history of liver disease, pancreatitis, or abdominal trauma. Computed tomography revealed stenosis of almost the entire length of the splenic vein, and development of gastric fundal and short gastric varices. No inflammatory changes or neoplastic lesions of the pancreas were observed in any imaging study. The patient was diagnosed with left-sided portal hypertension caused by idiopathic splenic vein stenosis, and splenectomy was performed. The postoperative course was smooth, and improvement of the gastric varices was shown by upper gastrointestinal endoscopy at 3 months after the operation.Idiopathic splenic vein stenosis is an extremely rare cause of left-sided portal hypertension. Splenectomy is one of the most effective treatments for left-sided portal hypertension caused by idiopathic splenic vein stenosis. Left-sided portal hypertension (LSPH) is characterized by the flow of splenic venous blood into the portal trunk via the collateral circulation due to narrowing or obstruction of the splenic vein. Although splenic vein stenosis or occlusion is often caused by trauma, pancreatitis, or invasion or compression by a pancreatic tumor, few reports have described idiopathic splenic vein stenosis. Herein, we report a case of LSPH caused by idiopathic splenic vein stenosis.A 70-year-old man presented with the chief complaint of epigastric discomfort. Upper gastrointestinal endoscopy showed isolated gastric varices Fig. , and theBased on these findings, we diagnosed the patient with LSPH caused by idiopathic splenic vein stenosis. Splenectomy was performed because of concerns about the risk of bleeding from the gastric varices. No obvious abnormality was detected around the splenic vein. The liver surface was smooth and the edges were sharp, while the spleen weighed 120 g and was macroscopically almost normal. Histopathological examination showed slightly conspicuous sinus hyperplasia, but no obvious findings of neoplastic lesions or vasculitis, including around the splenic vein. Congo red staining was also performed, but no obvious amyloid deposits were observed. The postoperative course was smooth, and upper gastrointestinal endoscopy performed 3 months after the operation showed that the gastric varices had disappeared  stenosis of the splenic vein with post-stenotic dilatation; (2) no evidence of a cause of splenic vein stenosis, such as pancreatitis, pancreatic tumor, cysts, thrombosis, arterial aneurysm, retroperitoneal fibrosis, or iatrogenic injury; (3) normal liver function tests, normal appearance of the liver by imaging studies, and/or no evidence of hepatitis or cirrhosis by histological examination; and (4) presence of gastric varices and multiple collateral vessels leading from the spleen to the stomach via the short gastric veins with hepatopetal blood flow. In the present case, although the first event was different, the diagnostic method was the same. Regarding treatment, although splenectomy was performed in the first case, while endoscopic treatment was performed in the pregnant case, surgical treatment would have been performed if this patient had consented.Gastric varices are generally asymptomatic and are often identified incidentally. The most common clinical symptom in symptomatic patients with LSPH is gastrointestinal bleeding from a ruptured gastric varix. Takagi et al. reported  that theSplenectomy is considered an effective treatment for patients with uncontrollable bleeding. In symptomatic patients, splenectomy is associated with correction of potentially fatal bleeding, prevention of hypersplenism, and a possible reduction or elimination of future bleeding episodes. Wang et al. reported  that surIn any case, the management of LSPH should be directed at the splenic side of the portal circulation, because pressure is increased only on that side . Unlike It is also important to consider postoperative complications after splenectomy. Peculiar complications include pancreatic fistula and overwhelming post-splenectomy infection (OPSI) , 15, whiIn patients for whom surgical treatment is high risk, splenic artery embolization has been reported as an alternative treatment option, and in patients with splenic vein stenosis without occlusion, stent implantation in the splenic vein has been reported. However, these treatments may lead to splenic abscesses or delayed splenic perforation , and theWe report a case of LSPH caused by idiopathic splenic vein stenosis that was improved by splenectomy. There are few reports of idiopathic splenic vein stenosis, and this condition can sometimes be fatal. Splenectomy is one of the most effective treatments for patients with intractable bleeding and recurrence from gastric varices.


Inverted papilloma is a rare condition of the middle ear. In this paper, the authors present a case report of a patient at a Midwestern health system with inverted papilloma. To supplement the case report, a literature review was also performed to identify clinical trends predisposing such cases to recurrence, malignant transformation, and response to radiation. In addition, the authors also propose a surveillance algorithm derived from this case and previously published surveillance strategies.The authors present a rare case of inverted papilloma of the middle ear. To the authors’ knowledge, this is the youngest case presentation (mid-teenage years) of this condition to have been reported in the literature. The patient underwent surgical excision, had recurrence, and has been disease free since revision surgery.Our literature review identified 25 cases previously published with ours being the 26th. An inadequate number of cases exist to abstract statically relevant clinical trends in presentation and tumor behavior. Additionally, no tumor characteristics have been identified that predispose tumors to future malignant transformation. No assessments can be made regarding the benefits of radiation therapy. Most cases to date have been surveyed with a combination of CT, MRI, and clinical follow-up.Inverted papillomas of the middle ear space are rare. Although this case report adds to the literature, additional cases are needed to draw statistically relevant clinical characteristics and responses to medical and surgical therapy. Inverted papillomas are benign tumors typically found in the nasal cavity. These locally aggressive tumors have a potential for malignant transformation. Presentations of inverted papillomas in the middle ear space are rare, with the previous literature reporting as few as 23 cases total.A female in her mid-teens presented with a chief complaint of hearing loss. Initial otoscopic examination revealed a bulging tympanic membrane with an inflamed mass occupying the middle ear space. Nasopharyngoscopy  did not reveal any sinonasal masses or lesions. A hearing test demonstrated a unilateral, profound hearing loss. A CT scan of the temporal bones showed nonspecific, complete opacification of the middle ear and mastoid on the affected side.  An additional MRI scan was obtained showing an enhanced soft tissue mass centered within the left middle ear cavity. No intracranial involvement was noted.As seen in Figure 3, there was a proliferation of thickened transitional-type epithelium with an inverted growth pattern, forming well-circumscribed lobules and glands that emptied onto the luminal surface. No evidence of infiltrative growth or necrosis was seen. On higher power (inset) the neoplastic cells had features of columnar and stratified squamous cells lacking significant mitotic activity or nuclear pleomorphism. Intraepithelial polymorphonuclear neutrophilic granulocytes were noted, which focally form microabscesses.The patient was brought to the operating room for a middle ear exploration and biopsy. A red, flesh-like mass was noted to be completely occupying the middle ear space. The mass appeared to be highly vascularized with finger-like projections extending radially. At the time of this exploration, the ossicular chain (i.e. the hearing bones) was completely encompassed in tumor and we were unable to ascertain proper anatomy and movement.The Eustachian tube, , could not be adequately viewed. A specimen of the mass was taken for pathologic review. Final pathology was interpreted as an inverted papilloma with no evidence of dysplasia .After further discussion and planning by the authors, the patient underwent a surgical middle ear exploration with removal of the lesion and hopeful exteriorization. During the case, a 1 cm area of erosion of the bony eustachian tube was noted and subsequently, packed. The tumor was completely excised with the exception of two areas. A microscopic tumor was left as it was overlying facial nerve. Also, several microscopic tumor fragments were left affixed to the stapes around the oval window. Postoperatively, the patient did very well with no facial nerve weakness, although her hearing remained poor on the diseased side.After 18 months of follow-up observation, she developed further hearing loss and ear drainage in the affected ear. A subsequent MRI demonstrated a tumor enhancement in the left mastoid region and left middle ear cavity. In addition, no enhancing lesions in either internal auditory canal were observed. No other lesions were noted in either the nasopharynx or neck.The patient then underwent a revision modified radical mastoidectomy. Granulation tissue was noted in the mastoid cavity. There were some areas of inflammation and pockets of purulent  material, which were removed. Biopsies were obtained from the remnant tissue around the stapes and the facial nerve and were confirmed to be inverted papilloma. Small areas of remnant tumor along the facial nerve were left alone. She had normal postoperative facial nerve functioning. Her ear canal has since become fibrosed, scarred, and created an overclosed ear canal.The patient has been asymptomatic since revision surgery with stable hearing loss. A pair of postoperative MRI films performed at one and six months after the second surgery showed a clear mastoid cavity with no evidence of recurrence.th. Publication dates ranged from 1987-2016.The authors conducted a literature review to identify a total of 25 previously published cases of inverted papilloma of the middle ear and mastoid cavity, ours being the 26Hearing loss appears to be the most common presenting symptom. Interestingly, there appears to be no correlation between severity of presenting symptoms and chance of recurrence. In 2012, Jones et al. described a case that presented with complete facial nerve paralysis, although no recurrence after resection was reported.Additional discrepancies exists in the literature regarding whether or not radiation therapy can decrease the probability of disease recurrence. Although radiation therapy has been shown to be an effective means of local control in some instances of sinonasal inverted papillomas,Conflicts also exist regarding whether or not a history of sinonasal papilloma predisposes patients to more aggressive malignant forms of ear papillomas. Several previous reports have described patients with a history of nasal papilloma with malignancy as the original otologic histology.In this presented case of a patient in her mid-teens, many factors may have been relevant to her surgical outcome. During her first tympanomastoidectomy, the decision had been made to leave the tumor over her facial nerve and around the stapes alone to avoid facial nerve damage and deafness respectively. At the time, it was not known how aggressive the tumor was and the decision for observation was made.During the second surgery, meticulous care was taken to remove every part of the tumor around these areas. At the time of this publication the patient is approximately 18 months out from her last surgery and disease free, although time will tell concerning the aggressive nature of her disease. Our original decision to forego radiotherapy on this patient was made due to her young age and the lack of supporting evidence in the literature. We would have chosen to radiate the area if there had been any evidence of malignant transformation, further erosion, or spread of tumor.We plan to continue following the patient with serial MRIs at six-month intervals and physical exams including nasopharyngoscopy. Additional cases must be identified and published to draw statistically relevant clinical characteristics and responses to various medical and surgical therapies. Future publications that identify different presentation trends, treatment regimens, and surveillance protocols may lead to more evidenced-based patient care for this rare condition. The authors suggest that any patient with chronic ear drainage, hearing loss without obvious cause, or chronic otalgia (i.e. earache) be referred to an otolaryngologist for further evaluation.The authors declare no conflict of interest.


A Spigelian hernia is a very rare hernia, making up approximately 0.1% of all abdominal wall hernias. This hernia goes through a defect in the Spigelian fascia which is the part of the transversus abdominis aponeurosis lateral to the rectus muscle, often at the level of the arcuate line, where the fascia is widest and weakest. We present the case of a 77-year-old female with no past surgical history who presented to our teaching hospital with high-grade small bowel obstruction secondary to an incarcerated Spigelian hernia. She was taken to the operating room for a laparotomy and a portion of the small bowel mesentery was found to be strangulated. The hernia was reduced, and the defect was repaired primarily. The diagnosis of a Spigelian hernia can often be difficult to diagnose on history and physical examination alone, but computed tomography (CT) imaging can be a valuable adjunct in diagnosis. Prompt surgical treatment should ensue when the diagnosis of high-grade bowel obstruction is made in a patient with imaging findings consistent with a Spigelian hernia. The Spigelian fascia is the part of the transversus abdominis aponeurosis lateral to the rectus muscle, often at the level of the arcuate line, where the fascia is widest and weakest . HerniasWe present a rare case of an incarcerated Spigelian hernia causing high-grade bowel obstruction in a 77-year-old patient presenting with nausea, vomiting, and abdominal pain.A 77-year-old female with a past medical history of type one diabetes mellitus and hypertension presented to our teaching institution with two days of nausea, vomiting, and obstipation. She reported diffuse abdominal pain and bloating. She had no history of prior abdominal surgeries. She was hemodynamically stable on presentation. Her abdominal exam revealed a distended abdomen with generalized tenderness to palpation without rebound or guarding. She had no palpable hernias. Her laboratory work was largely unremarkable. She had a computed tomography (CT) scan performed which showed a high-grade bowel obstruction with a transition point at a right-sided Spigelian hernia Figures -2. She wA Spigelian hernia, which was first described by Klinkosch in 1764, accounts for about 0.1% of all abdominal hernias . This isA complicated obstructed spigelian hernia is an uncommon diagnosis. It is unusual to find mesentery of small-bowel being incarcerated in this Spigelian hernia defect leading to acute small-bowel obstruction. In an emergency setting, reduction of the hernia is usually sufficient if the bowel loops appear viable post reduction and CT scan confirms the diagnosis.


Concomitant cemento-osseous dysplasia (COD) and aneurysmal bone cyst (ABC) are rare in the head and neck region. In our search of the English language literature, we found only one case report describing the simultaneous occurrence of COD and ABC in the head and neck region. Here, we report a case of COD associated with ABC. Further, we performed a systematic search of the literature to identify studies on patients with COD associated with nonepithelial lined cysts of the jaws.The patient was a 32-year-old woman who was referred from a private dental clinic because of a cystic lesion below the mandibular right first molar. She had no pain or significant systemic disease. After performing panoramic radiography and cone-beam computed tomography, the imaging diagnosis was COD with a cystic lesion, such as ABC or solitary bone cyst. Excisional biopsy was performed, which revealed concomitant COD and ABC.This case of ABC associated with COD provides insight for the diagnostic process of radiographically mixed lesions with cystic changes. Cemento-osseous dysplasia (COD) is a fibro-osseous lesion occurring in the tooth-bearing area of the jaws –3. The eThe aneurysmal bone cyst (ABC) is a benign, osteolytic, rapidly growing, expansile lesion of the bone, which predominantly occurs in the metaphysis of long bones, such as the femur, tibia, and spine , 8. Its Nonepithelial lined cysts occasionally occur along with various bone lesions, mainly fibrous dysplasia, giant cell tumor, chondroblastoma, ossifying fibroma, benign osteoblastoma, COD, fibrous histiocytoma, fibrosarcoma . These cThe patient was a 32-year-old Korean woman referred because of a cystic lesion below the mandibular right first molar at a local dental clinic. She had no pain or significant systemic disease. Several clinical tests, including electric pulp test, were performed and the affected tooth showed the presence of pulp vitality. There was no tooth mobility.Panoramic radiography revealed a round, mixed lesion below the mandibular right first molar Fig. . The borIn the additionally obtained cone-beam computed tomography scan, a slightly ovoid lesion was observed anteriorly and backwards Fig. . LikewisSurgical excision and histopathologic examination were performed. Histopathologically, stromal tissue was composed of spindle-shaped fibroblasts with small blood vessels Fig. . Cystic One-year follow-up was done and there was no evidence of recurrence. Normal bone healing was seen without pathological findings.A systematic search of the literature was performed to identify studies reporting patients with COD associated with nonepithelial lined cysts of the jaws. English- and non-English-language papers were searched in PubMed/MEDLINE/Google Scholar databases and the gray literature using a combination of terms containing “cemento-osseous dysplasia” and containing “aneurysmal bone cyst” or “simple bone cyst” or “traumatic bone cyst” or “cyst” or “cystic lesion.” The search was carried out using the literature from 1985 to date. The focus of each reference varied, including a series of patients, case reports, and articles investigating the imaging appearance. Papers that reported epithelial lined cysts, such as a periapical cyst or dentigerous cyst, were excluded. We reviewed 116 papers and finally selected 14. Table We found 46 patients with a female predilection (43 women vs 3 men). Forty-five patients had concomitant COD and SBC, while only one patient had concomitant COD and ABC. The mean age was 43.47 years, ranging from 11 to 66 years. The mandibular posterior area  was the most frequently reported site . Clinically, 11 patients presented with pain, while 12 patients presented with no pain. The pain status of 23 patients was not mentioned.Both COD and ABC are non-neoplastic intraosseous lesions. The concomitant occurrence of these lesions could be explained by the probable pathogenesis of ABC. The stroma of COD often displayed characteristic cavernous-like vascularity that was almost always associated with bony trabeculae . Free heOur literature search demonstrated only one case report of concomitant COD and ABC , so an aCompared to one previously reported concomitant COD and ABC case, our patient showed the anterior part of the mixed lesion and the posterior cystic portion. Buccolingual expansion was unclear in the cystic portion. Since the cystic portion was small, it was difficult to distinguish ABC or SBC in the radiologic findings. Pathologically, the COD portion was observed on one side, and most of the lesions showed the characteristics of ABC. The part where the COD was clearly diagnosable was considered to be the anterior part of the lesion observed in the radiologic mixed lesion.The imaging findings of the previous case showed the characteristics of ABC with clear buccolingual expansion. In the pathologic findings presented, the characteristics of COD were generally observed, and in some of them, the ABC findings were visible. Both lesions were diagnosed as concomitant COD and ABC, but the imaging and pathologic findings showed some differences among similar tendencies. Although there are various limitations to generalization due to the small number of cases, so, but we are expecting to better understand and diagnose nonepithelial cystic lesions associated with COD by reporting this case, in which an ABC and COD were present in association.


Primary spindle cell sarcoma is a rare tumour. The presentation of acute intra-abdominal bleeding from a metastatic spindle cell tumour has not been previously reported. We report a case of a 40-year-old woman with a history of curative resection of the medial compartment of the right thigh for spindle cell sarcoma presenting with an acute onset abdominal pain and haemorrhagic shock after 5 uneventful years. Emergency exploratory laparotomy was conducted that revealed a retropancreatic mass which had ruptured along its inferior border. Histological evaluation revealed a metastatic deposit of the spindle cell sarcoma. In cases of spontaneous abdominal haemorrhage, it is important to consider the possibility of a ruptured metastatic deposit among the differentials especially in patients with a history of malignancies. Moreover, this is the first reported case of metastatic malignant spindle cell sarcoma presenting with intra-abdominal haemorrhage. Primary spindle cell sarcoma is a rare soft-tissue tumour that lacks a specific cell line of differentiation and is among the least reported tumours in the world . Being pA 40-year-old previously healthy woman underwent a curative resection of the medical compartment of the right thigh for a malignant spindle cell tumour. Due to clear resection margins and absence of any metastatic deposits in imaging, she was not given any adjuvant therapy. She was followed up for 5 years, and she was otherwise well and asymptomatic without any evidence of local recurrence.After a period of 5 years, she developed an acute onset generalized severe abdominal pain and collapsed. She did not have any external bleeding manifestations such as haematemesis or melaena. There was no history of abdominal trauma. She was immediately admitted to the emergency department, and she was found to be in class III haemorrhagic shock with a pulse rate of 125 beats per minute and a blood pressure of 90/60 mmHg. Her abdomen was distended and diffusely tender. She was immediately resuscitated with intravenous crystalloid boluses and blood transfusion. Her serum beta-Human Chorionic Gonadotropin (beta-HCG) was negative, excluding a ruptured ectopic pregnancy. Her full blood count revealed a haemoglobin level of 5 g/dl, and a massive transfusion protocol was initiated. Her other basic investigations and clotting profile were within normal limits. Despite resuscitation, her vital parameters did not improve and her abdominal girth was gradually increasing. The abdominal ultrasound scan revealed moderate amount of free fluid with a mass around the proximal part of the pancreas. There was no evidence of abdominal aortic aneurysm, and other solid organs were normal. Hence, a decision was taken to proceed with an emergency exploratory laparotomy. A computed tomography was not done as the patient was haemodynamically unstable.A midline laparotomy was performed. There was 4.5 litres of haemoperitoneum, and a linear tear in the gastrocolic omentum with a haematoma in the lesser sac. Active bleeding was noted from a nidus which was situated posterior to the body of the pancreas, and it had ruptured through the inferior border of the pancreas. The rest of the pancreas and other abdominal organs looked normal. Bleeding was controlled by ligation and local application of haemostatic agents. A biopsy was taken for histopathological analysis. The pancreas and the rest of the organs were macroscopically normal.The histological analysis revealed an infiltrating tumour composed of fascicles of spindle cells with oval, vesicular nuclei with the scanty eosinophilic cytoplasm. There were areas containing cells with moderate to severe nuclear pleomorphism and increased mitotic activity (13/10 high power field). The prominent storiform pattern was evident . The focFollowing surgery, the patient was managed in the intensive care unit for 4 days, and thereafter, the patient recovered and was discharged on day 7. She was offered pancreatectomy with excision of the tumour but she refused surgery. She was referred to the oncologist and was commenced on adjuvant chemotherapy. She received a course of Ifosfamide and Doxorubicin. Contrast-enhanced computed tomography was performed before the commencement of chemotherapy which showed a 7 × 4.8 × 4.9 cm mass lesion with a thin wall containing hypodense material, attached to the inferior border of the pancreas . There wSpindle cell neoplasms encompass a wide diversity in clinicopathologic and biological heterogeneity . PrimaryIn the reported literature, primary tumour has been treated aggressively with surgical therapy, along with adjuvant chemoradiotherapy in high-risk patients. In a population-based analysis including 3299 patients, around 60% of the overall cases were treated with surgery alone, and despite different surgical modalities, they had a relatively favourable disease-specific and overall survival rates compared to the nonsurgical group [Similarly in our case, the patient underwent curative resection of the medial compartment alone as a treatment modality without conferring the patient to adjuvant therapy. Even though there was no evidence of metastatic disease at the time of surgery, the patient presented with complications of metastatic disease after 5 years.Metastatic tumour presenting with massive intra-abdominal bleeding is a relatively rare phenomenon, and spindle cell sarcoma presenting with such phenomena is even rarer. Bhardwaj et al. describes a case of metastatic squamous carcinoma of the lung presenting with upper gastrointestinal bleeding . Joy et In our patient, proper preoperative work-up was not possible as the presentation was an emergency, and the priority was to achieve haemostasis and stabilization rather than tumour excision. Due to the haemodynamic instability and the difficult anatomical location of the tumour, curative dissection was not attempted, and a biopsy was taken to confirm the diagnosis. Retroperitoneal metastasis is very rare in this tumour and mostly occurs in the lungs because of haematogenous spread.Although rare, it is important to consider the possibility of a ruptured metastatic deposit among the differential diagnoses in an event of a spontaneous abdominal haemorrhage. Obtaining a detailed history is important to detect previous history of malignancies that could give rise to this rare complication. Furthermore, this may be the first presentation of a tumour in an otherwise healthy person.We describe a middle-aged female with a history of curative medial compartment resection of the thigh for a malignant spindle cell tumour later presenting with massive intra-abdominal haemorrhage due to metastatic deposits. In cases of spontaneous abdominal haemorrhage, especially in patients with a history of malignancies, it is important to consider the rare differential diagnosis of a ruptured metastatic deposit.


Amyloidosis is characterized by extracellular deposition of the amyloid protein. It can affect multiple organ systems but it most commonly affects the heart, kidney and gastrointestinal (GI) tract. It can occur sporadically or in association with other conditions like multiple myeloma, chronic inflammatory diseases, infections etc. Its involvement of the gastrointestinal tract is rare and diffuse. It has variable manifestations in GI tract from involving the stomach to the large bowel including liver. We present a case of 55 year old Caucasian male with recent diagnosis of multiple myeloma who presented with recurrent episodes of small bowel obstruction which was later found to be caused by amyloid deposition. Amyloidosis is characterized by extracellular deposition of amyloid protein. There are two most common forms of amyloidosis. The AL amyloidosis which is most commonly associated with monoclonal free light chains deposition, which was also previously called primary amyloidosis is most common. The AA amyloidosis  is secondary to chronic inflammation in the body due to infection, rheumatoid arthritis etc. AL amyloidosis most commonly involves heart, kidney, liver and gastrointestinal tract . AmyloidA 55 year old Caucasian male with past medical history of multiple myeloma (recently diagnosed) currently on chemotherapy with Velcade +Cytoxan and dexamethasone, chronic kidney disease, hypertension, atrial flutter s/p ablation, melanoma of the left thigh status post-surgical resection with negative lymph nodes and in remission presented to the hospital with chief complaints of multiple episodes of nausea and vomiting, diffuse abdominal pain, subjective fevers and chills from past 2 days. Last bowel movement was 2 days ago. On presentation, his vitals were stable. Labs including complete blood count, comprehensive metabolic panel was normal. CT abdomen/pelvis without contrast revealed partial small bowel obstruction. This was followed by small bowel follow through which revealed non- specific thickening of proximal small bowel loops. This was his third hospitalization with same presentation of small bowel obstruction. He was admitted one month ago and 2 weeks ago with similar presentation. During the initial hospitalization, he underwent exploratory laparotomy with no findings to suggest mechanical small bowel obstruction. Subsequently, he had an esophagogastroduodenoscopy (EGD) with push enteroscopy which identified enteritis and a Mallory-Weiss tear Figure . Small bOn this admission, trial of nasogastric (NG) tube was given and EGD with push enteroscopy was repeated which revealed non erosive esophagitis, mild gastritis and severe duodenitis; biopsies were obtained. Congo red staining of the biopsy revealed amyloidosis of the small bowel. However, the gastric biopsy did not reveal any evidence of amyloidosis Figure -4. The pOur case highlights the importance of the amyloidosis as a cause of intestinal pseudo -obstruction in patients with diagnosed multiple myeloma. Intestinal pseudo-obstruction is a well-known complication of amyloidosis and it should always be kept in mind in patient with multiple myeloma presenting with ileus .From our review of literature, we found 33 cases of amyloidosis reported as intestinal pseudo-obstruction. Amyloidosis is characterized by extracellular deposition of abnormal protein. It involves multiple organs, most common being the heart, peripheral nerves and kidneys; however its involvement of the GI tract is mostly subclinical ,5. It haDiagnosis of amyloid, irrespective of the type, depends upon clinical, laboratory and histological findings. Although endoscopic and radiological findings also assist in the diagnosis, they are very non-specific. Endoscopic findings include mucosal fragility, diffuse lesions such as ulcers, erosions, granular or plaque like mucosa of the stomach or small intestine, large duodenal folds, peripyloric ulcers and polypoid protrusions ,8. The rNo specific treatment for management of GI complications in systemic amyloidosis are available and treatment should be directed at symptomatic management. It is important to assure adequate nutrition and hydration for patients with motility disorders. Using pharmacological agents like ondansetron, granisetron, or prochlorperazine and using loperamide, lomotil for diarrhea can be helpful ,10.Overall, systemic amyloidosis has a very poor prognosis. The median survival in untreated patients is <2 years . The mosThis work has been presented at the 2019 ACG Annual Meeting .The presence of amyloidosis in GI tract is rare and can have a variety of presentations. A high index of suspicion is required in patients with multiple myeloma and other chronic inflammatory conditions who present with gastrointestinal problems. In patients, without a history of systemic amyloidosis, gastrointestinal amyloidosis should be suspected in case of unexplained weight loss, autonomic dysfunction, proteinuria or malabsorption. Treatment involves treating the underlying cause. Untreated patients carry a very poor prognosis.


Dear Editor,A 5-year-old male patient with a history of frequent falls and difficulty in speaking since two years of age presented with a two-month history of left peripheral facial paralysis, together with intractable vomiting and slurred speech. The mother reported that he had been born (at term) with perinatal asphyxia and had been admitted to the neonatal intensive care unit. Physical examination also showed left-sided paresis of the face, arm, and leg, as well as mild left-sided dysmetria. Magnetic resonance imaging of the brain showed a predominantly solid, infiltrative, expansile lesion in the brainstem, with a signal that was isointense on T1-weighted images and slightly hyperintense on T2-weighted images, without contrast enhancement or restricted diffusion . The les. They typically occur in middle-aged or elderly individuals, having a slight predilection for males-3. In 50-60% of cases, they are found in the fourth ventricle, although they can also arise in the lateral ventricles, third ventricle, and septum pellucidum, as well as in the cerebral, cerebellar, and medullary parenchyma,2,4,5. Intracranial subependymomas are usually asymptomatic, generating symptoms only if they obstruct the flow of cerebrospinal fluid or compress the adjacent structures. Histopathologically, they are characteristically fleshy, white, hypovascular, and well-defined, composed of scattered groups of uniform cells, with cilia and microvilli, oval nuclei with little or no mitosis, and a matrix of dense fibrillar material, often accompanied by microcysts,3.Subependymomas are tumors of uncertain origin, classified as grade I by the World Health Organization, and are typically infiltrative. Among individuals with subependymoma who are under 14 years of age, the complete resection rates are lower and progression-free survival is shorter, especially when the lesion is infiltrative, younger age and infiltration therefore being factors associated with a worse prognosis.When they occur in young individuals, subependymomas are usually mixed tumors, appearing in conjunction with ependymomas or astrocytomas-10. On magnetic resonance imaging, they are typically solitary, circumscribed, solid-microcystic, intraventricular, and exophytic,4,5. In comparison with the white matter, they present a signal that is isointense to hypointense on T1-weighted images and hyperintense on T2-weighted images, in some cases presenting hypointense foci in susceptibility-weighted sequences, due to calcifications or, more rarely, bleeding,4,5.Such lesions typically show little or no contrast enhancement and rarely show any perilesional edema or restricted diffusion,4,5. The main differential imaging diagnoses of intracranial subependymoma include ependymoma, medulloblastoma, astrocytoma, and central neurocytoma, although diffuse glioma is the main differential diagnosis when the lesion is when infiltrative.Expansile lesions in the nervous system have been the subject of recent studies in the radiology literature of Brazil. When the tumor is in a critical region, there is no need for extensive resection, given that even partial resection has been shown to result in a favorable outcome, especially if followed by radiosurgery and radiotherapy. In conclusion, the case presented here was one of an unusual presentation of subependymoma, with characteristics indicative of a poorer prognosis. Such characteristics should be taken into consideration in imaging studies.Surgical management is adopted only when a subependymoma is symptomatic, all other cases being monitored


Trigger point blocks are now widely practiced, especially in pain treatment. Among the complications of lumbar trigger point injection, reports of medically induced kidney injury are very rare, and diagnosis during emergency treatment is rare.A 78-year-old woman on antiplatelet medication following a stroke was diagnosed with treatable type A aortic dissection at another hospital after undergoing lumbar trigger point injection. On arrival at our hospital, there were no signs of hemodynamic deterioration. Additional careful medical re-interview and ultrasonography by anesthesiologists enabled a definitive diagnosis of acute kidney damage and hematoma caused by lumbar trigger point injection, and aortic dissection surgery was abandoned.This clinical case demonstrates the importance of awareness of potential kidney injury and hematoma during lumbar trigger point injection. Trigger point injection therapy is a common treatment for chronic pain in primary medicine and is in general use by family doctors and orthopedists for chronic low back pain. Although several complications have been reported for this technique , acute kIn this report, we present a case in which additional careful medical re-interview and ultrasonography enabled a definitive diagnosis of acute renal failure and hematoma caused by lumbar trigger point injection, which was subsequently treated conservatively.We obtained approval from the institutional ethics committee of Asahikawa City Hospital and written informed consent from the patient for publication of this case report. The study followed the CARE guidelines and the appropriate EQUATOR guidelines.The patient was a 78-year-old-woman with a previous history of cerebral infarction and hyperlipidemia who was taking therapeutic aspirin  without other anticoagulants. She complained of uncomfortableness and a sudden, new onset of low back but no chest pain 30 minutes after receiving lumbar trigger point injection of lidocaine by her family doctor. She was referred to a primary emergency hospital with a suspicious diagnosis of aortic dissection. She was conscious with systolic blood pressure around 170 mmHg with a left-right difference of 30 mmHg on arrival at the hospital. Contrast-enhanced computed tomography (CT) revealed Stanford type A aortic dissection and she was transferred to our hospital. She remained conscious with stable vital signs throughout this time.Since the patient was scheduled for emergency surgery for type A aortic dissection, we noticed something unusual while conducting our additional detailed medical interview and physical examination in preparation for the surgery. Based on the interview, renal hematoma was suspected because of localized lumbar pain and a trigger block puncture mark in the same area. We suspected that her symptoms were related to the lumbar trigger point injection. Ultrasonography revealed a hypoechoic lesion displacing the left kidney, approximately 3 cm beneath the injection site, suggesting left renal hematoma caused by trigger point injection, as indicated by the white arrows in Fig. Further investigations revealed no progression of anemia, no increase in blood staining on CT, and no active bleeding, and conservative therapy was initiated with antihypertensive treatment for the thrombotic obstructive type A aortic dissection. After diagnosis of the renal hematoma, aspirin administration was discontinued. Only non-steroidal anti-inflammatory analgesics and antibiotics were prescribed for the renal hematoma. She was discharged from the hospital without sequelae 2 weeks after admission.Renal hematoma is a rare complication of lumbar trigger point block, although it is an important treatment in the pain clinic. We experienced a case in which careful medical re-interview and ultrasonography enabled a definitive diagnosis of acute kidney injury and hematoma caused by lumbar trigger point injection. Among the 276 claims associated with invasive procedures for chronic pain management in the American Society of Anesthesiologists Closed Claims Project , 17 invoRegarding the trigger point injection performed by the previous doctor, whether it was done blindly or with ultrasonography-guided technique, how long the needle was, and other detailed information was unknown because it was not reported to us. Therefore, we could not discuss the technique in this case. In the present case, the renal hematoma was a complication of trigger block injection and was not associated with aortic dissection; moreover, the hematoma appeared to be iatrogenic, caused by prolonged bleeding due to antiplatelet therapy. Non-traumatic renal hematoma can be classified into three types: internal, subcapsular, and perirenal . Non-traUltrasonography is useful for visualizing free fluid in the trauma setting but is inferior to CT in terms of resolution and ability to accurately characterize renal injury , 9. It iIn the dorsal subcutaneous to renal anatomy, structures located at a thickness of approximately 3 cm beneath the subcutaneous tissue and fat include the latissimus dorsi, quadratus lumborum, and transverse abdominal muscles; the inferior surface of the diaphragm above the kidney; and Gerota’s fascia . BetweenThe present treatment strategy was conservative and included follow-up observation because renal capsular hematoma is sometimes accompanied by bleeding. Our careful re-interview and primary ultrasonography enabled accurate identification of the cause of bleeding in this patient and guided the course of appropriate treatment.In conclusion, hematoma caused by lumbar trigger point injection is a relatively rare condition. Our case report highlights the importance of careful medical re-interview and ultrasonography at the primary medical examination.


Primary central nervous system lymphoma (PCNSL) rarely manifests in immunocompetent patients. In such cases, these lesions may mimic more common intracranial bleeding or tumors. We present the case of an elderly patient who presented with a presumed chronic subdural hematoma (SDH); upon surgical intervention, an occult mass was discovered with no evidence of associated hematoma. Biopsy and immunohistochemistry demonstrated PCNSL. Literature review identified six other cases of PCNSL in immunocompetent adults that were initially suspected to be SDH but were finally diagnosed with PCNSL. Our literature review highlights the rarity these cases and the importance of distinguishing intracranial bleeds from PCNSL, as the latter can be treated with chemoradiation with good clinical outcomes. Primary central nervous system lymphoma (PCNSL) is a rare variant of non-Hodgkin’s lymphoma (NHL), which is restricted to the brain, leptomeninges, cranial nerves, spinal cord, or intraocular compartment without involvement of other organ systems. PCNSL comprises 1%-3% of all central nervous system (CNS) tumors and only 2%-3% of NHL cases . -12. 8-12Although our literature review focuses on immunocompetent patients with PCNSL, we recognize that SCNSL can also present like a subdural bleed in patients with a history of B-cell lymphoma. Gonzalez-Bonet et al. described the case of a 71-year-old male who presented with a two-week history of neurological decline after a fall with head impact . AlthougAnother clinical entity to consider is PCNSL presenting with intracranial hemorrhage, which can obscure the lymphoma tumor on neuroimaging. Barrios et al. described the case of a 40-year-old woman who experienced sudden-onset neurological deterioration and was found to have a SDH from a ruptured dural lymphoma lesion on autopsy, along with multiorgan dissemination of small B-cell lymphoma . AlthougPCNSL in immunocompetent patients remains an uncommon diagnosis. As there is low clinical suspicion for such cases, these lesions can easily mimic more typical entities. We present the rare case of a patient with long-standing PCNSL whose clinical course and imaging studies mimicked SDH for several years. Our patient and six others identified via literature review demonstrate how correct diagnosis and appropriate management of PCNSL can result in good clinical outcomes, especially in those with low-grade B-cell lymphomas. We further consider PCNSL presenting with concomitant intracranial bleed, SCNSL mimicking SDH, and other types of intracranial tumors that PCNSL can mimic. Our findings illustrate the rarity of PCNSL mimicking intracranial bleed and the importance of obtaining histopathological diagnosis in these cases.


Angiosarcoma is a rare malignant tumors. The objective of this study is to report a patient who suffered from a progressive low back pain and left lower extremities radiation pain for about 8 months, After diagnoses, this was identified as an extremely rare case of primary multiple angiosarcoma of vertebra.A 54-year-old man with a history of 2-year hypertension and 8-year diabetes, both of which were well controlled by drug management. Lately, he suffered from a progressive low back pain and left lower extremities radiation pain for about 8 months.Magnetic resonance imaging of lumbar showed a clear pathological fracture and primary multiple angiosarcoma of all vertebra. Postoperative pathology and High-throughput sequencing confirmed the diagnosis of primary multiple angiosarcoma of vertebra.The patient underwent minimally invasive pedicle screw fixation combined with bone cement augmentation for the purpose of stabilizing the damaged vertebrae. Following operation, he received both radiotherapy and chemotherapy for a period of time.The operation has achieved positive results in relieving pain and stabilizing the spine. No wound problem or operative complications occurred after operation. The patient reported an obvious remission of low back pain and was only capable to perform restricted physiological activities. A long-term palliative radiotherapy and chemotherapy were performed after operation. Unfortunately, the patient died 18 months later.This article emphasizes primary multiple angiosarcoma of vertebra. Despite being rare, it should be part of the differential when the patient manifested back pain and radiculopathy. We recommended the minimally invasive pedicle screw fixation for angiosarcoma of vertebra. Osteoplasty by bone cement augmentation was also an ideal choice for surgical treatment. It also advocates the use of specific targeted radiotherapy drugs based on gene analysis of tumors. Over 50% of the cases occur in the head and neck, making up 0.1% of the head or neck malignant tumors, At present, there remains no standard for treatment. The common practice includes surgical excision, radiotherapy and chemotherapy. However, the effect of chemotherapy has yet to be evaluated. We found a case of vertebral angiosarcoma. We first used percutaneous vertebro plasty to stabilize the vertebral body, and then selected targeted drugs according to the results of DNA sequencing.Tumor tissues are highly differentiated and have a high rate of metastasis and recurrence. Conventional imaging examination is incapable to make accurate diagnosis or provide valuable clues, which makes the process of diagnosis and treatment of angiosarcoma complicated.2In December of 2016, a 54-year-old man with a history of 2-year hypertension and 8-year diabetes, both of which were well controlled by drug management, suffered from a progressive low back pain and left lower extremities radiation pain for about 8 months. The pain in his back could reach 8 points using visual analog scale and could not be alleviated with rest and hot compresses. The imaging examination revealed a multiple spinal pathological fracture with a bone lytic lesion located in the all spinal vertebral body , The shoulder joint consisted of the humerus and clavicle , and pelvis bone  and TP53 exon 7  were detected, which indicated that the drugs targeting p53 and Nf1, including MEK inhibitor trametinib and cobimetinib, as well as mTOR inhibitor everolimus and temsirolimus had a possibility to be suited to his treatment.Sequenom MassARRAY system (Sequenom) based on time-of-flight mass spectrometry was performed for the detection of single nucleotide polymorphisms (SNPs) in the genes associated with sensitivity to chemotherapeutic agents. The potential SNPs were detected in multiples genes related to chemotherapeutic sensitivity, including X-Ray Repair Cross Complementing 1 (XRCC1) rs1799782G>A, methylenetetrahydrofolate reductase (MTHFR) rs1801133 AA, P-glycoprotein (ABCB1) rs1045642 GG, rs2032582 A>C, and cytochrome P450 2D6 (CYP2D6) rs1065852 AA. These SNPs were indicative of increased sensitivity to paclitaxel, carboplatin, cisplatin, and oxaliplatin, and reduced sensitivity to tamoxifen, vincristine, and methotrexate.Preoperative assessments included echocardiogram,electrocardiogram, and chest X-ray. Lumbar magnetic resonance imaging was ordered to visualize the metastatic lesions, to assess the stability of the vertebral column, and to aid in the formulation of a surgical approach. Considering multiple pathological fractures of the spine, minimally invasive pedicle screw fixation combined with percutaneous vertebro plasty was scheduled for him. In brief, posterior circumferential decompression from T7 toT11 internal fixation was performed. Meanwhile, the T7 and L1 vertebral body was injected with bone cement. The visual inspection using the intraoperative fluoroscopy showed the optimal position of all pedicle screws. The incision was closed and the intraoperative blood loss was approximately 400 mL.Thus, erythrocyte 4U was used. Postoperatively, the patient was referred to the regular ward.The surgical biopsy of the lesion and immunohistochemical examination were conducted to confirm primary angiosarcoma of vertebra Figs.  and 4. POne week after the operation, the patient's muscle strength of lower extremities improved to grade V compared to the preoperative status, and the symptoms were relieved significantly. Moreover, visual analog scale score of his back pain improved to 0 to 1 points compared to the preoperative status, 7 to 8 points. The patient reported an obvious remission of low back pain and was only capable to perform restricted physiological activities. The palliative radiotherapy and chemotherapy were performed for a long period of time after operation. Unfortunately, the patient died 18 months later.3 In this case, X-ray and CT showed the multiple bone destruction in the patient's body, especially in the spine. We thought it was a multiple myeloma of the lumbar spine at that time, but subsequent pathological biopsy confirmed that the tumor was indeed a primary angiosarcoma. The postoperative pathological tissue sections were characterized by a typical vascular endothelial malignant tumor, where the cells were large cubic and polygonal with eosinophilic cytoplasm and light nucleus. Abnormal mitosis was observed in a large number of cells. It was disorderly and heteromorphic, >2 /10 HPF, immature blood vessels . Following the analysis conducted of its clinical and pathological characteristics, consideration was given to the long-term inflammatory stimulation. It led to the occurrence of angiosarcoma.Angiosarcoma/hemangiosarcoma in vascular endothelial cells and lymphangiosarcoma stems from lymphatic endothelial cells. There are no reliable morphological and molecular biological indicators to distinguish between the 2, as a result of which they are collectively known as angiosarcoma. It is now believed that the occurrence of angiosarcoma is related to chronic chronic lymphedema and ionization. Radiation, chemical contact, trauma and chronic infection are related to. That means the potential of using these therapeutic drugs against this angiosarcom. Previous studies have shown that genotype A/XRCCI Arg399Gin has a higher probability of responding positively to platinum-based therapy in patients with non-small cell lung cancer, suggesting that the SNP mutation leads to DNA repair defects and increased effectiveness of platinum-based chemotherapy. XRCC1 is a scaffold protein involved in repair of single strand breaks after BER. Some studies have shown that XRCC1 Arg399Gln is significantly associated with the prognosis of non-small cell lung cancer. XRCC1 Arg399G/G is significantly associated with better critical survival in patients treated with cisplatin, so cisplatin is also a potential therapy.Upon high-throughput sequencing performed for the full exon regions in cancer related genes, a discovery was made of mutations in NF1 exon 32 (c.4183 C>T p.Gln1395X) and TP53 exon 7 . Accordingly, the therapeutics targeting NF1 MEK inhibitors (trametinib and cobimetinib) and mTOR inhibitors (everolimus and temsirolimus) could be applied to the treatment. Sequenom MassARRAY system analysis was conducted of the SNPs in genes related to the sensitivity to chemotherapeutic agents, with potential SNPs detected in XRCC1 rs1799782G>A, MTHFR rs1801133 AA, ABCB1 rs1045642 GG, rs2032582 A>C, and CYP2D6 rs1065852 AA. These genotypes had been reported to be associated with therapeutic effect or toxicity of tamoxifen, which is a weak anti-estrogen and has a significant inhibitory effect on the expression of CYP2D6 gene.Generally speaking, the occurrence of angiosarcoma is very rare. Its clinical manifestations are very similar to those of single glomus tumor and Kaposi sarcoma. Without rich clinical experience, it is difficult to diagnose. This requires advanced diagnostic techniques. Pathological biopsy is the most direct and fundamental diagnostic method and the golden standard of diagnosis. Therefore, it is suggested that the patients suspected of hemangiosarcoma should undergo pathological biopsy as soon as possible to determine the type of disease. After definite diagnosis, it is also necessary to detect the gene level of patients. High-throughput sequencing is 1 aspect, to explore the relationship between genes and disease occurrence, and to use specific anticancer drugs to regulate the effect of specific genes. In order to achieve the effect of targeted therapy, and according to the different tissues and organs targeted treatment, for tumors occurring in limbs, it is suggested that radiotherapy and chemotherapy, tumors occurring in bone tissue, should prevent the progress of bone destruction, maintain a certain bone density to play a role in supporting body weight. In a word, the diagnosis and treatment of hemangiosarcoma are very important. Only by definite diagnosis can targeted treatment be carried out.Conceptualization: Wei Huang, Tao Liu.Investigation: Ruimeng Duan, Depeng Shang.Methodology: Meng Zhang, Yashuai Yuan.Resources: Xiaobing Yu, Meng Zhang.Supervision: Xiaobing Yu, Wei Huang.Writing – original draft: Wei Huang, Mingjia Qu, Xiaobing Yu.Writing – review & editing: Wei Huang, Xiaobing Yu.


Teratoma is a true neoplasm and originates from the three germ cell layers and it can contain any tissue derived from these layers. The location of teratoma is variable according to the age group. In adults, sacrococcygeal teratoma is rare and carries a low risk of malignant transformation. Surgical resection is the mainstay of treatment and is challenging due to tumor location.We are presenting a case report of a 16-year old female referred to our hospital with recurrent attacks of urine retention. Imaging study showed a large sacrococcygeal tumor. It was successfully resected by a combined laparoscopic and posterior approach without any major complication.The combined laparoscopic and posterior approach is a safe surgical technique for resection of the large sacrococcygeal tumor. This surgical method has been published around 10 times in separated reports around the world and for first time in our region. Teratomas are tumors which contain tissues resembling normal derivatives from all the three germ cell layers. These tumors could be benign or malignant, most commonly found in testes and ovaries but also found in retroperitoneum, mediastinum, sacrococcygeal region and intracranial spaces .The sacrococcygeal teratoma is commonly found in the neonate with an incidence of 1/40,000 live births , 3. The Presacral tumors present later than tumors with an external component and are associated with a higher rate of malignant change .In this case report, we are presenting our experience of removing a large sacrococcygeal tumor by a combined laparoscopic and posterior open approach.A 16-year-old Saudi female had multiple emergency department visits for new acute onset of recurrent episodes of urinary retention over few weeks requiring intermittent bladder catheterization. Her workup showed a pelvic mass and was referred to the authors’ outpatient clinic for management mid of 2019. History and physical examination were unremarkable with BMI of 24.6. Pelvic Magnetic resonance imaging (MRI) showed a large multiloculated cystic presacral tumor approximately measuring 11.5 × 8.8 × 12.3 cm. A small part of the tumor was seen crossing the left levator ani muscle into the left ischioanal fossa and insinuating posterior to the last coccygeal joint with no bone destruction or intraspinal extension, Altman type IV. The lesion contained multiple cystic locules some containing mucoid material while others contained hemorrhage. The tumor was displacing the rectum to the right side, the uterus and the bladder anteriorly and stretching the vagina  scan of the pelvis with rectal contrast showed no leak. The drains were removed, and she was discharged home after 15 days.The histopathology showed a mature cystic teratoma measuring around 11 cm Fig. .Fig. 5H&The patient is following in the out-patient clinic with no urinary symptoms. CT scan done at one year show no evidence of recurrence.In Adults, sacrococcygeal teratoma is rare . These tMany patients complained of pain on sitting. The diagnosis of a presacral mass can usually be made with a digital rectal examination .In the pediatric population with sacrococcygeal teratomas, there is a tendency toward malignant change with increasing age. In adults, however, benign tumors are more common. Presacral tumors present later than tumors with an external component and are associated with a higher rate of malignant change .The standard of care for benign tumors is complete resection with negative margins, to relieve the symptoms and prevent malignancy in the future . The retDue to non-specific symptoms, the diagnosis is often made by imaging studies like CT, MRI and Ultrasound. Both CT and MRI had a 100% sensitivity in diagnosing a retrorectal tumor but imaging features alone cannot differentiate between benign and malignant teratomas , 8]. MR. MR8]. MCT scan is very sensitive in showing calcification in more than 50% of malignant tumors, but other studies report calcification on CT in both benign and malignant teratomas . Hence tCT and MRI are excellent investigations defining the extent of sacrococcygeal teratomas, while MRI is better in detection of spinal canal invasion . They alSagar et al. showed the ability of MRI to differentiate between benign and malignant tumors with specificity and sensitivity of 97% and 88% respectively. The corresponding values for preoperative biopsy were 100% and 83% [The serum levels of alpha-fetoprotein (AFP), beta human chorionic gonadotrophin (BHCG) and carbohydrate antigen 19-9 (CA 19-9) can be raised in malignant teratomas. In our patient tumor markers were normal, most likely suggesting a benign teratoma .The cornerstone of surgical management for retrorectal tumors is complete surgical resection , 12. In A combined anterior and posterior approach was used for large tumors that could not be removed through a single incision . SimilarThe largest comprehensive review of surgical management of retrorectal tumors was published by Baek which had 1708 patients with 155 having teratoma. The overall postoperative complications in the open surgical approach were 13.2%. The morbidity associated with the posterior approach was 7.2% with anterior approach was 19.3% and with a combined approach it was 24.7% .Post-operative complications included neurologic bladder 15%, wound infection 11%, dysesthesia 7%, fecal incontinence 7% massive bleeding 4% ureteric injury extensive soft tissue infection, reoperation for bleeding and impotence , 13.The minimally invasive approach by laparoscopic surgery was proposed for the first time in 1995 by Sharpe as laparoscopy provided bright illumination and magnification for deep pelvic tumors posterior to the rectum and helped in better dissection avoiding trauma to rectum pelvic muscle, nerves and control of blood vessels especially the median sacral artery , 15.p < 0.05). Complication rate was 19.8% in MIS and 12.2% in open surgery but was not statistically significant [In one large systemic review, minimally invasive approach (MIS) was used in 83 patients. The hospital stay was shorter than open surgery (4 ± 2 vs 9 ± 7 days The use of laparoscopic approach provided advantages like improved visualization in the narrow pelvis which facilitated the dissection of the cystic lesion from the rectum, avoiding a large incision, less postoperative pain, and wound infections.After comprehensive preoperative planning large sacrococcygeal teratoma extending from the sacral promontory to beyond coccyx can be safely removed by a combined laparoscopic and posterior approach.