Monostotic fibrous dysplasia (MFD) of the lumbar spine represents an exceedingly rare lesion. A 26-year-old patient presented with a progressive osteolytic lesion of the vertebral body L2 and the diagnosis of MFD. A minimally invasive left-sided eXtreme Lateral Interbody Fusion (XLIF) approach with resection of the vertebral body L2 with placement of a mesh cage was performed. No complications were observed perioperatively and the symptoms rapidly improved. Minimally invasive piecemeal resection with a combined dorsolateral approach showed a favorable clinical and radiological outcome and seems to be a safe and reliable technique for MFD. Monostotic fibrous dysplasia (MFD) of the spine represents a rare disease entity of the lumbar spine , 6, 19. In November 2018, a 26-year-old young man with deep-seated pain in the upper lumbar spine and the diagnosis of juvenile fibrous dysplasia of the vertebral body L2 was sent to our outpatient clinic. In 2016, a biopsy of the suspected vertebral body L2 was performed at an outside institution with the diagnosis of FD. Images of the whole body did not revealed any other lesions, so that the diagnosis of a MFD was confirmed. In the clinical course, multiple magnetic resonance images (MRI) were performed and showed a progressive osteolytic mass with medullary expansion, ground-glass matrix, narrow zone of transition, and marginal sclerosis of the second lumbar vertebral body Fig. . AdditioSurgical treatment, namely piecemeal resection of the lesion, was performed via a minimally invasive eXtreme Lateral Interbody Fusion (XLIF) approach on the left side to perform a partial resection of L2. The twelfth rib was partially resected. For vertebral body replacement, a SynMesh-Cage , filled with bone harvested from the iliac crest and the resected twelfth rib, was performed. This was supplemented with demineralized bone matrix . In the same anesthesia, the patient was rotated into the prone position and a minimally invasive posterior transpedicular stabilization from L1 to L3 including lordosing screws at L2 was performed .The patient did not suffer from any perioperative complications. Postoperative CT scan depicted correct positioning of the ventrodorsal instrumentation Fig. . The hisFD is a rare mosaic disorder in which normal bone is replaced by fibro-osseous tissue. FD can occur throughout the skeleton; however, most often the long bones, ribs, and craniofacial bones are affected . AlthougThe authors present a rare case of MFD of the second lumbar vertebra and its unique treatment with a combined minimally invasive dorsolateral approach to prevent progressive deformity. The favorable clinical and radiological outcome in the follow-up period emphasizes the safe and well-practicable surgical treatment to resolve the symptoms in a MFD patient with progressive spinal deformity.


Among the aggressive odontogenic tumors, a few tumors generally ameloblastomas, carry a connotation of being aggressive. But, a rare tumor like central dentinogenic ghost cell tumor (DGCT) can be equally aggressive with a propensity for recurrence. The two cases discussed in this article are divergent in behaviour with features such as presentation, seen in early decades as central intraosseous tumors in the maxilla and mandible. The first case describes an aggressive DGCT, associated with impacted tooth and odontome which showed recurrence into a large solid tumor within a year. The second case arose in accordance with a partially erupted molar which was comparatively innocuous and less aggressive. Both the cases exhibited classic histopathological features. These two unconventional cases of DGCT with a follow-up are being reported here to highlight the variation in behaviour and presentation and also to understand the aggressive nature of the tumor. In the category of odontogenic tumors, ameloblastomas tend to display a great variety of histopathological patterns, but some lesions like calcifying odontogenic cysts (COC) have shown an intriguing evolution pattern in tumor pathology in the passing decades.Gorlin et al. first identified and described COC as an entity of odontogenic origin . He elucPraetorius et al. gave further clarity to the dualistic nature by classifying COC into tumor/solid and cystic lesions with further subclassification as simple, associated with odontome, and associated with ameloblastic proliferation . The WorHere, we present two cases of DGCT. The first case of DGCT seen in a 28-year old male was associated with an impacted canine and odontome; albeit an independent identity as DGCT, it presented as a cyst clinically and was reported as a cystic variety of DGCT. The lesion evolved into a completely solid neoplastic variant of DGCT on recurrence, in other words, type 3 according to Toida et al.'s classification [A 28-year old male patient reported to the clinic with a hard maxillary swelling in the left posterior quadrant. On clinical examination, the swelling was seen associated with the premolars and first molar in the left maxillary quadrant . The swerd of the root  stained sections showed odontogenic cystic epithelium with tall columnar basal cells with polarized hyperchromatic nuclei. Suprabasal cells resembling the stellate reticulum-like cells and ghost cells were evident . The conThe decalcified H&E-stained sections of the hard mass showed dentin, cementum, and ectomesenchymal tissues similar to the dental papilla. All these tissues were arranged haphazardly representing a composite complex odontome . A diagnFollow-up led to the disclosure of recurrence in the maxillary antral floor in the form of a solid tumor (DGCT) after 11 months, but the patient was asymptomatic.A CT scan was done revealing a lowering mass in the antrum showing radiolucent radiopacities , and a pThe recurrent lesion found after 11 months, showed on grossing, a predominantly solid tumor mass . On clinical examination, the tooth was partially erupted and malaligned. No complaint of mobility or swelling was seen. On radiographic examination, a well-defined lesion was seen distal to 36. In between the roots of 36 and 37, a well-demarcated radiolucency with multiple specks of radiopacities was noted approximately 1 × 1.5 cm in size . The groHistopathological assessment of the H&E-stained sections revealed an encapsulated lesional cystic mass with epithelium showing ameloblastic changes and large ameloblastic follicles with entrapped ghost cells. Large areas of dentinoid induction and dentin with predentin-like areas in connective tissue were also evident Figures . A histoCalcifying odontogenic cyst (COC) is one of the mixed odontogenic lesions, which has traversed through a series of nomenclature and variations in pathology. In 1971, the World Health Organization (WHO) categorized COC as a “nonneoplastic cystic lesion” which ended in the emergence of a monistic and dualistic concept. The result culminated in 1992 with WHO naming the entity as neoplastic, irrespective of its cystic nature. In 2006, dentinogenic ghost cell tumor (DGCT) was the name given to solid variants as suggested by Praetorius et al. .Thus, DGCT itself is considered a solid variant of COC. Though the tumor manifests either as a solid or a cystic lesion, it can present as a central tumor in the bone as an intraosseous type or can be a more contained tumor with a peripheral presence in the soft tissue .rd to 8th decade of life, frequently in males [Most commonly, DGCT presents as a central lesion in the 3in males . It is ain males , 6, 7, 9Radiologically, DGCT appears as a unilocular or multilocular radiolucent and radiopaque lesion which is well demarcated , 9, 10. The surgical grossing revealed that the lesion was removed in toto in case 1 and was a cystic lesion with a hard odontome-like mass in close association. The case recurred as a completely solid tumor Figures  and 6. CThe histopathology in the cases of DGCT shows the presence of an odontogenic epithelium in the form of cystic epithelium, islands, and nests. The odontogenic epithelium may exhibit tall columnar cells with reversal of polarity along with the presence of ghost cells. The mesenchymal component shows induction of dentinoid, and dentin-like tissue with ghost cells being a significant part of the tumor . In the The ghost cells are said to be odontogenic epithelial cells with aberrant keratinization or coagulation necrosis. These cells are also seen in pilomatrixoma, odontoma, ameloblastic fibroma, and craniopharyngioma. In instances, they undergo calcifications as in our case. Dentin expressed can be a tubular structure similar to normal dentin or have an appearance of an eosinophilic irregular mass . Our casIn case 1, the hard mass showed dentin in normal and dysplastic form, cementum-like areas, and enamel-like areas all arranged haphazardly along with ghost cells. The lesional mass resembled a composite complex odontome. In the same case, the recurrent tumor was completely solid and looked similar to the primary tumor.In case 2, the tumor was well encapsulated and had a cystic element. The histopathology of which resembled case 1.The variation in histopathology in our cases was the presence of ghost cells in close vicinity of the surrounding bone. A large number of ghost cell clusters were seen trapped in between the trabeculae of the bone.Both the lesions were treated conservatively, but the first case recurred after 11 months. From the observations seen in the two cases, we cannot underestimate the behaviour of DGCT. The presentation though might be well contained or restricted. It can behave subtly, or actively as an aggressive tumor with high recurrence , 11. ThiDGCT is a rare tumor that may behave aggressively with a recurrence rate of 33-73% , 10, 11


Hemangioblastomas (HMGs) originating from the cerebellopontine angle (CPA) are extremely uncommon. Nevertheless, the cystic-solid form of this lesion at the above location is even rarer.We present a 31-years old male with a right ear hearing loss of 3 months duration. He did not experience earache or discharge before the hearing loss. He; however, experienced visual acuity and dizziness. General physical examination did not yield much.Computed tomography and magnetic resonance imaging revealed a cystic-solid mass at right CPA. We initial misdiagnosed the lesion as an acoustic neuroma with cystic changes. Immunohistochemistry studies revealed HMG.The lesion was total surgical resection via the retro-sigmoid approach.The patient's symptomatology resolved after the surgery. Two years follow-up show no recurrence of the lesion and the patient is well.Identification of feeding arteries and electro-coagulating them during the operation minimized intraoperative bleeding. The tumor should usually be dissected out whole and not piece meal fashion. Pre-operative CTA is very useful in outlining the vasculature of the tumor. The cystic forms of HMBs are often made up of a tiny vascularized nidus and a very large cystic portion.,4 The solid forms are often uniformly solid while the cystic-solid forms compose of a large cystic portion as well as a large solid portion.,6 HMBs are usually hypervascular and the solid cerebellar types often originates adjacent the brain stem.,8Hemangioblastomas (HMBs) originating from the cerebellopontine angle (CPA) are extremely uncommon.,4,5,9 They often appear as cystic lesions with a nodule of extreme contrast enhancement or a solid lesion.,9 Open surgery is the gold-standard treatment modality for HMBs. Radiosurgery has also proven to be a secure alternative treatment option. The solid forms of HMBs at the CPA often poses major surgical challenge, because very large ones often present with profuse bleeding intraoperatively. The complex neurovascular structures at the CPA often make navigation more difficult. Our case is a cystic-solid variety of HMB at the CPA which we operated on with no major complications as well as neurological deficits.Computed tomography (CT) and magnetic resonance imaging (MRI) are often the gold-standard radiological imaging modalities used in identifying HMBs although they are often mistaken for other CPA neoplasms.2We present a 31-years old male with a right ear hearing loss of 3 months duration. He did not experience earache or discharge before the hearing loss. He; however, experienced visual acuity and dizziness. General physical examination did not yield much. Cranial nerve examinations were unremarkable. We did not observe any cerebellar signs and symptoms. We did not observe any discharges from the ear and otoscopic examination did not reveal same. Rinne and Weber tests demonstrated sensorineural hearing loss. Fundoscopic examination did not yield much. Routine laboratory investigations were within normal rangers.CT revealed a cystic-solid mass at right CPA measuring about 5.3 × 4.3 cm with slightly dense regions. We noticed right auditory canal expansion Fig. A. EnhancWe totally resected the lesion via the retro-sigmoid approach. Intraoperatively, we saw a cystic-solid tumor which was red in color and composed of vascular tissue. We saw a multitude of thick feeding arteries and veins all around the solid portion. The solid part was tough in consistency with clear boundaries. It was bleeding profusely on touch. The solid portion was adhered to the trigeminal nerve, acoustic nerve, facial nerve as well as the brain stem. The tumor originated from the cerebellar pial vessels and took an intra-axial route. We first and foremost identified the feeding arteries and electro-coagulated them to minimize intraoperative bleeding therefore blood loss was minimal (<200 mls). We then carefully resected of the tumor by separating it from the above vital structures. We utilized electromyographic (EMG) and auditory brainstem responses (ABRs) to monitor the cranial nerves above. All cranial nerves functions were intact after the operation.On histochemical staining, we observed intracytoplasmic glycogen within the foamy cytoplasm Fig. A and B. 3,10,11 These lesions are often seen in patients in their 30's or 40's. Nevertheless, the sexual predisposition of CPA HGMs has not been reported. HGMs often exist primarily as cystic with a tiny mural, solid, or cystic-solid. Our case was the cystic-solid type which mislead us in the diagnosis. HGMs usually have a vascularized nidus which is made up of stromal cells encased by abundant capillaries.,5HGMs constitutes of about 1.5% to 2.5% of all neoplasms arising intracranially and 7% to 12% of all posterior fossa neoplasms. as we also observed in our case. Nevertheless, the neoplasm developed at the CPA, an extra-axial site, instead of the cerebellar parenchyme. HMBs arises mostly in the cerebellum, brainstem, as well as the spinal cord.,13 At the CPA, Hemangioblastoma (HMGs) are often misdiagnosed due to the peculiarity of the site, clinical presentation as well as imaging characteristics. The unusual cystic-solid nature of the lesion couples with the symptomatology initially make us misdiagnose the lesion as an acoustic neuroma with cystic changes.Byung et al are of the view that, their lesions in their patient originate from the cerebellar pial vessels and took an intra-axial route In most cases, this tumor is often misdiagnosed because these signs and symptom are typical presentation of most CPA tumors. CT and MRI are very useful in the definitive diagnoses of this tumor. On both CT and MRI, HMGs are frequently seen as a cystic mass with a small hypervascular mural nodule. The cysts typically have smooth boundaries as well as homogeneous cerebrospinal fluid (CSF)-like signal intensity.Hearing loss, tinnitus, ataxia, as well as vertigo are the usual symptomatology in patients with HMGs.,15 Our case is the cystic-solid variant which was seen on both CT and MRI as a large cyst portion and a large solid portion. In the cystic type, the nodules are often seen as hypointense images on T1-weighted and hyperintense images on T2-weighted on MRI. The lesion enhances intensely on contrast. In most cases, high-flow vessels can be seen as flow voids at the periphery of the mass due to the hypervascularity of the tumor. Edema is mostly insignificant or nonexistent around lesion in all the tumor variants.The solid variant is usually seen as a solid mass without any cystic constituent.,10 It is advocated that as much as possible, solid HMGs should be dissected and excised whole instead of piecemeal manner because of associated life threating hemorrhage. Total resection is achievable with wide field surgical exposure as well as circumferential dissection and devascularization. Nevertheless, restricted access via the retro-sigmoid surgical exposure as well as the complex neurovascular anatomy of the area of the CPA usually make total excision problematic. We achieved total resection of the tumor via the retro-sigmoid approach. Radiosurgery is a secure alternative treatment for residual HMGs after surgical resection. This treatment modality is usually an adjuvant therapy for residual tumor after surgical resection. Radiosurgery is appropriate during tumor recurrence as well as unresectable or deep-rooted tumors at the CPA. We did not adapt the radiosurgery treatment because we achieved total resection and no recurrence of the lesion.Surgery via the middle fossa, retro-sigmoid or transtemporal approach is still the most preferred treatment modality for CPA HMGs. They indicated that radiosurgery may diminish the possibility of extreme intraoperative bleeding during surgical resection. Nevertheless, there are hypothetical life threating complications accompanying preoperative embolization that cannot be overlooked. Corneliusc et al observed poor prognosis, acute tumor hemorrhage as well as death as cardinal complication associated with preoperative embolization for HMGs. Furthermore, tumor engorgement, as well as vessel occlusion resulting in acute infarction, has been associated with preoperative embolization for HMGs.,19Kamitani et al established that preoperative radiosurgery as well as embolization of the feeding vessels are very advantageous in the treatment of solid HMGs with hypervascularity. Chemotherapy is possible because of the similarity of histogenesis of HMGs. Selective intra-arterial chemotherapy may be effective in the treatment of HMGs because of their ability to regulate the proliferation of endothelial cells, smooth muscle cells, as well as fibroblasts. Cheng et al observed facial paresis, lower cranial nerves deficits, abducens nerve paralysis, facial hypesthesia, cerebellar hemorrhage, CSF leakage, as well as pseudo-meningocele as the postoperative complication in patients who were surgical treated. We did not observe any such complication after surgical resection of the lesions in our patient. We utilized EMG and ABRs to monitor the cranial nerves. After our operation, we observe the cranial nerves functions and found them intact. Though HMGs are considered as histologically benign tumors, recurrence of the lesions have been reported after total resection.,21 We did not observe any recurrence of the tumor 2 years after surgical resection. Nevertheless, recurrence rate of HMGs is about 10% to 27%.Another very promising treatment modality for CPA HMGs is endovascular selective chemotherapy.,11 On the other hand, protein inhibin-α, a member of the transforming growth factor β family is usually strongly positive in most HMBs on immunostaining. Also, epidermal growth factor receptor and platelet-derived growth factor are strongly positive on immunostaining. Furthermore, focal areas of immunoreactivity with S-100 protein, NSE as well as GFAP have also been reported. We observer positivity for NSE, Inhibin- α, CAIX, podoplanin (D2–40), as well as BRG on immunostaining which confirmed the diagnosis of HMG.Histochemically, on periodic acid–Schiff staining, characteristic intracytoplasmic glycogen within the foamy cytoplasm are often observed.4Cystic-solid form of HMG at the CPA is very rare. The symptomology as well as radiological findings of HMGs at the CPA often makes these lesions misdiagnosed for other CPA tumors. CTA is very helpful in outlining the vasculature of the lesions during preoperative assessment. The tumor should be dissected out whole as much as possible to avoid life threating bleeding. Feeding arteries should be identifies and electro-coagulated during the operation to minimize intraoperative bleeding.Conceptualization: Zhigang Lan, Seidu A. Richard, Yuekang Zhang.Data curation: Zhigang Lan, Seidu A. Richard, Yuekang Zhang.Funding acquisition: Yuekang Zhang.Methodology: Seidu A. Richard.Writing – original draft: Seidu A. Richard.Writing – review and editing: Zhigang Lan, Seidu A. Richard, Yuekang Zhang.


A 65-year-old man was evaluated for a difficult-to-characterize pancreatic head mass in the setting of idiopathic chronic pancreatitis. He had no other relevant medical history and was not taking any anticoagulant or antiplatelet treatment. Endoscopic ultrasonography fine-needle aspiration (EUS-FNA) failed to reveal neoplasm cells. A linear array echoendoscope  was advanced up to the duodenal bulb, from which the head of the pancreas was visualized. After ensuring a vessel-free access to the pancreatic parenchyma, the FNA was performed using a 22G needle  with a total of 3 passes , and there were no imaging traces of active hemorrhage nor luminal bleeding. In addition, because endoscopic retrograde cholangiopancreatography was not feasible owing to inaccessible duodenal papillae, he was conservatively managed with success, as currently proposed.1 The rational for a nonsurgical approach in stable patients is related to an expected spontaneous reabsorption of the hematoma because of the abundant irrigation of the area. This report points out an exceptional case of a simple EUS-FNA diagnostic procedure, complicated by a voluminous IDH, presenting with gastric outlet obstruction, upper gastrointestinal bleeding related to Mallory-Weiss tears, and important cholestasis.IDH caused by mechanisms other than blunt abdominal trauma or nonaccidental injury to children, namely concerning child abuse, are rare.Author contributions: J. Roseira wrote the manuscript. M. Cunha and J. Rachadell managed the patient. HT de Sousa and J. Brito revised the manuscript. J. Brito is the article guarantor.Financial disclosure: None to report.Informed consent was obtained for this case report.Previous presentation: This case was presented at Portuguese Digestive Disease Week, May 9–June 1, 2019; Algarve, Portugal.


Post-transplant lymphoproliferative disease (PTLD) is a series of proliferative diseases of the lymphatic system. Among patients receiving hematopoietic stem cell transplantation (HSCT), PTLD is a prevalent complication that severely affects rates of survival. Ultrasound plays an essential role in the early diagnosis of PTLD. Contrast-enhanced ultrasonography (CEUS) and CEUS-guided biopsy are critical procedures for tumor diagnosis.Herein, we report the case of a 40-year-old male patient with acute lymphoblastic leukemia who received HSCT more than 1 year ago. Sonography revealed a small hypoechoic nodule in the liver four months after HSCT. Eight months after HSCT, larger and more nodules were observed via ultrasound; CT was used to identify the lesions.CEUS and CEUS-guided biopsy were performed, and the pathological diagnosis was PTLD.The final clinical diagnosis was PTLD, and cyclophosphamide, epirubicin, and dexamethasone were administered as chemotherapy.The patient was discharged after his condition improved.Ultrasound can be used to effectively detect lesions of PTLD early after HSCT. Furthermore, CEUS and CEUS-guided biopsy were effective for early confirmatory diagnoses of PTLD after HSCT. However, position emission tomography/computed tomography is radioactive and therefore, inappropriate when multiple examinations are indicated. The use of CE-CT and magnetic resonance imaging is allergic and nephrotoxic potential of the employed contrast materials. In addition, patients with leukemia usually have renal impairment due to chemotherapeutic drugs. Meanwhile, ultrasound is the preferred surveillance strategy, as it is an accessible and inexpensive imaging method for routine monitoring of post-transplant patients. In addition, contrast-enhanced ultrasonography (CEUS) is less invasive, has no associated nephrotoxicity, allows for real-time observation, and has fewer side effects. It can also be used for the accurate assessment of tumor vascularity, tumor location, and other defining features that aid in subsequent puncture operations. Therefore, ultrasound has gradually become the preferred inspection method for patients following transplantation. Herein, we report a case involving a 40-year old man with hypoechoic nodules in the liver associated with PTLD. We provide detailed information and characteristics of CEUS of the tumor and describe the process of ultrasound-guided biopsy. There have been no noted previous reports on CEUS presentation of adult intrahepatic PTLD after HSCT.Post-transplant lymphoproliferative disease (PTLD) is characterized by abnormal lymphoid proliferation, from benign to malignant, caused by immunosuppression following solid organ or hematopoietic stem cell transplantation (HSCT).2A 40-year-old male patient was admitted to our hospital with recurrent gingival bleeding and was diagnosed with acute lymphoblastic leukemia 15 months before the study. The patient underwent allogeneic HSCT 7 months after diagnosis. During this time, the patient was noted to be in good health. Chemotherapy and supportive treatment were maintained after the transplant. Four months after transplantation, hypoechoic nodules were detected in the liver and spleen via ultrasound Fig. A–F. NoduUS was performed using the LOGIQ E9  equipped with a convex transducer (frequency range 1–6 MHZ). Two-dimensional ultrasound showed multiple low-echo nodules in the liver with clear boundaries, uneven internal echoes, and surrounding hypoechoic halos. Color Doppler ultrasound showed no obvious blood flow signal in the nodules Fig. B,E. We p3,10–14 PTLD usually occurs within 6 to 12 months after HSCT, before the reconstruction of EBV-specific cytotoxic T-cell immunity.,11,15,16 In allogeneic HSCT, previous studies demonstrated that the incidence rate of PTLD following allogeneic HSCT varied greatly from 1.0% to 17%. It also varied with the stem cell source, degree of HLA mismatch, and patient characteristics. Conditioning eigenfactors can contribute to the development of PTLD, including the degree of human leukocyte antigen mismatch along with aggressive T-cell depletion methods. Prompt diagnosis is crucial to prevent the development of malignant lymphoma.PTLD is a serious complication of high heterogeneity following solid organ and HSCT, and it has high rates of morbidity and mortality.Multiple imaging diagnostic methods are essential tools for early diagnosis and staging of lesions, and ultrasonic examination is a common surveillance strategy for patients after transplantation. Second, CEUS can more efficiently identify tumor characteristics, vascular distribution, and dynamic relationships with surrounding tissues in real time. It is also safer for patients with abnormal liver and kidney function. Third, there were necrotic areas within the partial nodules in our case. CEUS can be used to distinguish between enhanced activity areas and nonenhanced necrosis. Ultrasound-guided puncture can reduce the acquisition of necrotic tissue and obtain greater value pathological tissue. Furthermore, a study showed that, as a radiation-free and widely used imaging method, US is reliable for the detection and exclusion of abdominal or soft tissue lymphoma in children with PTLD, while CT may be reserved for supplementary imaging in cases with incomplete or equivocal findings on US. In addition, false-negative results of PET/CT mainly occur in areas with high physiological background activity and early PTLD lesions. Therefore, US and CEUS examinations have significant practical value in this case.In our case, the intrahepatic lesion was detected early by ultrasound, and CT was not possible. Differences between US and CT may be attributed to the patient receiving plain CT instead of enhanced CT. Plain CT is best for medium density tissues and cannot differentiate between normal and diseased tissues. Because of the patient's abnormal liver and renal function, enhanced CT was not the preferred method of examination. Furthermore, the tumor was small and located in segment VI of the right posterior lobe of the liver in the low position; hence, plain CT might have omitted it. Although the patient did not undergo enhanced CT, we considered CEUS to be the more suitable modality for routine examination and diagnosis of the tumor. First, two-dimensional ultrasound showed no obvious blood flow signal in the lesion. Ultrasound contrast media flow of blood has a better correlation with tumor microvascular density. Therefore, CEUS is more sensitive than CE-CT for detecting low vascular lesions. Thus, we suggest that the intrasplenic nodule was not of the same nature as the intrahepatic nodule and might be related to a manifestation of graft-vs-host disease. Thus, changes in drugs administered may be responsible for the disappearance of the nodule. We hope that future investigations will elucidate these causes.It is worth noting the disappearance of the splenic nodule Fig. A,B. Beca CEUS of PTLD after renal transplantation was similar in appearance, with peripheral to central enhancement followed by low enhancement. Because of the lack of similar cases, we will continue to collect cases and analyze the CEUS of intrahepatic PTLD after HSCT to provide valuable information for clinical diagnosis and treatment. The limitations of this case include the lack of a subsequent ultrasound after the condition of the patient improved and the unknown influence of drug intervention on the condition. Future research should focus on the clinical application and follow-up of therapeutic efficacy.To the best of our knowledge, the use of US and CEUS for the detection of intrahepatic PTLD after HSCT has not been systematically reported. In our case, two-dimensional ultrasound revealed multiple hypoechoic nodules in the liver with clear boundaries, neat contours, uneven internal echoes, and no obvious blood flow signals. CEUS showed that the foci with necrotic areas presented rapid annular enhancement in the arterial phase; while in the initial primary foci with nonnecrotic areas, there was rapid global enhancement. Low enhancement was observed in both the venous and parenchymal phases in all nodules. In addition, PTLD was reported in other cases, several of which were characterized by the presence of PTLD after renal transplantation. However, none implemented CEUS to characterize intrahepatic lesions after HSCT.Small lesions of PTLD can be detected early via ultrasound. CEUS has fewer side effects, facilitates real-time observation, is less invasive, and can assist in evaluating tumor properties. Thus, US and CEUS are of great significance in the detection and diagnosis of PTLD after HSCT.www.editage.com) for English language editing. My deepest gratitude goes to the reviewers for their serious work and pertinent advice.I thank the other authors for their help with CEUS, and I want to express my appreciation to the other authors for their suggestions and guidance on scientific thinking. I would like to thank Editage (Conceptualization: Jianchun Li, Li Wang, Ligang Wang.Investigation: Weinan Chen, Ailin Cui, Yang Liu.Supervision: Jianchun Li, Xiaoming Fan, Yanming Zhang.Writing – original draft: Weinan Chen.Writing – review & editing: Weinan Chen, Yang Liu.


Popliteal venous aneurysms are rare vascular disorders associated with a high risk of pulmonary embolism. We present the case of a 56-year-old woman hospitalized for a third episode of unprovoked pulmonary embolism. Venous ultrasonography identified a popliteal aneurysm, repeatedly missed by two-point compression venous ultrasonography, which was eventually confirmed by a magnetic resonance examination. Because of its highly symptomatic nature despite optimal anticoagulant treatment, the decision was made to undergo surgery, consisting of aneurysmectomy followed by patch angioplasty. The goal of this paper is to report a rare case of popliteal venous aneurysm and its treatment strategies and postoperative evolution. Popliteal venous aneurysms (PVAs) are rare pathologic vascular disorders. They may have various aetiologies including congenital, trauma, varicose veins, localized degenerative changes, and inflammation. Moreover, they usually remain silent until a thromboembolic event is developed. Given the rarity and potential fatal complications of this condition, without an established consensus of its management we report a case of primary popliteal venous aneurysm and discuss the therapeutic approach.A 56-year-old female patient presented to the emergency room with a recent syncope followed by dyspnoea of acute onset. The medical history was significant for a left Baker’s cyst, Hashimoto thyroiditis, and recurrent episodes of unprovoked pulmonary embolism . During previous hospitalizations, Doppler venous ultrasonography showed no signs of deep vein thrombosis. Furthermore, no predisposing factors were identified, and the patient underwent screening tests for malignancy and blood tests for acquired or inherited coagulation disorders, all of which had been negative.Since the hospital discharge after the first diagnosis of pulmonary embolism, the patient was commenced on long-term treatment with direct oral anticoagulant which she has been following up until the current hospital admission.Physical examination revealed a hemodynamically stable patient with an oxygen saturation of 96% without additional oxygen, blood pressure of 110/70 mmHg, heart rate of 76 bpm, respiratory frequency (RF) of 18 r.p.m., and a temperature of 36.8 °C. Heart sounds were regular, and no cardiac murmurs were detected. Lung sounds were normal, with no dry or moist rales. Examination of the lower extremities was unremarkable—no palpable masses and no signs of deep vein thrombosis were identified. By using the recommended pulmonary embolism (PE) prediction rules, the revised Geneva and the Wells score, the patient was classified in the moderate-probability category, thus having a 20–30% risk of PE. The pulmonary embolism severity score (PESI) was 56, corresponding to a low-risk.Blood work showed elevated plasma D-dimer levels (2125 ng/mL) and normal cardiac biomarkers.ECG showed signs of RV strain, with inverted T waves in leads V1–V4, and incomplete right bundle-branch block.Echocardiogram revealed no signs of RV pressure overload or dysfunction.A computed tomographic pulmonary angiography was performed that revealed filling defects located in the segmental arterial branches of the right inferior lobe, confirming the diagnosis of pulmonary embolism. Two-point compression ultrasonography showed no signs of deep vein thrombosis. However, a complete ultrasonography of the lower extremity venous system in real-time B-mode revealed a 6 × 5 × 4 cm anechoic mass located in the cranial extremity of the left popliteal fossa, contiguous to the popliteal vein, with sluggish, swirling blood flow, but no signs of thrombosis . Doppler spectral analysis revealed low velocity blood flow with normal phasic variation corresponding to a venous waveform. The ultrasonography also identified the Baker’s cyst as a fluid-filled “speech bubble” structure at the postero-medial knee with a “neck” at its deepest extent.Knee MRI was performed to better describe the extension and anatomy of the aneurysm. It described an aneurysmal dilatation of the distal popliteal vein measuring 64 × 19 × 31 mm, with turbulent blood flow which exerted a mass effect on the adjacent muscle groups .After a Heart Team session, and in accordance to the patient’s wishes, the decision was made to proceed with surgical repair of the aneurysm.Access to the popliteal fossa was obtained via a posterior approach.Intraoperative findings confirmed the presence of 6/4 saccular aneurysm located along the distal popliteal vein .The popliteal vein and the aneurysm were isolated, followed by longitudinal incision and drainage of the aneurysm, which revealed no thrombotic material. Next, the surgical procedure consisted of tangential aneurysmectomy followed by patch angioplasty with bovine pericardium .Microscopic examination with tricrom masson staining protocol showed continuous thinned venous layer with fragmentation of elastin fibres (blue arrow) and few smooth muscle fibers (white arrow) confirming the diagnosis of popliteal venous aneurysm .Early post-surgery, the patient developed a hematoma that required surgical haemostasis and drainage. Otherwise, the recovery was uneventful. Post-discharge medication consisted of oral anticoagulant and aspirin.The patient remained symptom-free at 2 months after the surgery. The antiplatelet treatment was discontinued after 3 months. After consulting with the patient, the decision has been made to maintain the anticoagulant treatment.Venous aneurysms are rare findings, having different aetiologies and locations throughout the body ,2. They Also, a higher prevalence in women was reported. A possible explanation could be the oestrogen-related effects on angiogenesis .Ultrasound, CT, or magnetic resonance (MR) imaging are used as non-invasive diagnostic tools for this condition. We believe that the tool of choice remains the color duplex ultrasound, which besides describing the morphology of the aneurysm can predict the risk of thrombus formation by detecting the turbulent flow in the aneurysmal segments. But, when an intervention is warranted, computed tomography venography or magnetic resonance venography may be required for preoperative planning. Diagnosing PVAs venous thromboembolic complications should follow the guideline recommended diagnostic workup . In our Currently, there are no criteria with regard to the venous size in defining venous aneurysms. Isolated dilatation of one and a half, two or three times more than the native vein have been described in the existing literature ,2,4. MorEven though there is controversial data regarding the therapeutic management of PVAs, expectant monitoring, medical management with anticoagulation, and surgical repair are the possible therapeutic plans . AnticoaThe operative technique must be selected on a case-by-case basis. Posterior access is the most common, followed by resection of the aneurysm sac and lateral venorrhaphy to reconstruct the vein. Primary venorrhaphy can be done if the aneurysm wall appears to be of good integrity. In our case, intraoperative findings of a severely diseased venous wall dictated the use of a patch. The procedure usually has few complications, including transient common peroneal nerve palsy and hematoma formation, as in our case .Whereas endovascular techniques have become a mainstay of vascular surgery, they currently have no defined role in the treatment of venous aneurysms .There are cases of recurrent popliteal venous aneurysms after surgical patchplasty that require surgical reintervention. The role and duration of anticoagulation in postoperative asymptomatic patients is unclear. Several reports suggest oral anticoagulation between 3–12 months, the use of compression stocking, or lifelong aspirin therapy [The small number of existing reported cases make definitive recommendations hardly to be made. Even though current guidelines for the diagnosis and management of acute pulmonary embolism  do not m


At the follow-up, he was healthy, with normal renal function, without dilatation of the collecting system or vesicoureteral reflux.Primary obstructive megaureter (POM) is an uncommon disease in adults, it is defined as an intrinsic congenital abnormality at the lower segment of the ureter in the ureterovesical junction. It is generally unilateral, but may be seen bilaterally in 15% to 25% of cases and occurs more often in males. Symptoms includes flank pain, recurrent urinary tract infection, hematuria and acute renal colic. Chronic ureteral obstruction may lead to serious complications such as infection, stone formation and renal failure. Primary symptomatic or complicated obstructive megaureter requires surgical management, using different surgical techniques, consisting of excision of the adynamic ureteral segment and reimplantation into the bladder. We present a case of 19-year-old young male, without past medical history, presented to the emergency department with intermittent recurrent left flank pain for the last three years, without lower urinary tract symptoms (LUTS), fever or haematuria. On clinical examination, his abdomen was soft to palpate, with no tenderness. Routine laboratory tests were normal. Urinary ultrasonography (US) revealed a giant left hydroureteronephrosis, with reduction in cortical thickness. Abdominal and pelvic computed tomography (CT) showed a left giant hydroureteronephrosis  and huge dilatation of left ureter with tortuosity down to the urinary bladder (B and C). These findings allowed us to retain the diagnosis of primary obstructed megaureter. The patient underwent an excision of the terminal narrow adynamic ureteral segment, with laparoscopic transperitoneal ureteral reimplantation according to the Lich-Gregoir technique. The postoperative course was completely uneventful. The patient was discharged from the hospital at the 5


Laryngeal rhabdomyosarcomas (RMSs) mainly occurred in children, while were extremely rare in adults. Consequently, less information was available to guide clinicians to manage adult RMSs in larynx.A 42-year-old man presented with a 2-year history of gradually worsening hoarseness. Then, he underwent a surgery with suspension laryngoscope with initially being diagnosed as vocal cord cyst. Unexpectedly, the lesion was proved to be embryonal rhabdomyosarcoma (ERMS), pathologically. Next, he underwent chemoradiotherapy, while the tumor relapsed 18 months after the last treatment. Subsequently, a vertical hemilaryngectomy and a right selective neck dissection was performed, and the chemotherapy according to the anticancer drug sensitivity in vitro was arranged. Until the last check-up 18 months after chemotherapy, the patient did not display clinical or radiological signs of local recurrence and metastases.Misdiagnosis and missed diagnosis of laryngeal RMSs might appear when tumors presented as smooth protuberance. We reported the first case of laryngeal RMSs in an adult with the multidisciplinary strategy based on the chemosensitivity assay in vitro. Furthermore, a systematic review of the literature was also discussed, highlighting the initial diagnostic pitfalls and subsequent management problems that may occur with this uncommon tumor. As a result, less information was available to guide clinicians to manage adult RMSs in larynx. We reported the first case of laryngeal RMSs in an adult with the multidisciplinary strategy based on the chemosensitivity assay in vitro.Primary laryngeal sarcomas constitute less than 1% of all malignant laryngeal tumors . RMSs usA 42-year-old man presented with a 2-year history of gradually worsening hoarseness without pain, dyspnoea or dysphagia. He had no history of alcohol or tobacco use. Flexible laryngoscopy in January 2016 showed a reddish, smooth and submucosal mass with protrusion medially in the anterior third of the right vocal cord, and glottic closure was incomplete posteriorly with normal mobility  glottic ERMS. Then the experiment of anticancer drug sensitivity of this tumor to eight chemotherapeutic regimens was determined in vitro, revealing that DCF regimen  was the most efficient . The clinical features of the case in this study were similar with previous cases.The first case of laryngeal RMS was reported by Glick in 1944 . SubsequTable . The cliThe clinical features and macroscopic appearances do not differ substantially from the other laryngeal tumors, therefore, the initial diagnosis of laryngeal RMS in adults may be difficult . The mos(Table ). The case described here was an adult with the type of ERMS, which did not accord with this general pattern concerning the preponderance of age and pathological type. ERMS of the larynx must be differentiated from other common histological types in that it differs significantly with respect to its management [Therefore, the definite diagnosis is based on optical microscope and immunohistochemistry . PositivTable . The casnagement .The management of RMSs has evolved from radical surgery to less morbid regimen, which is now typically limited to organ-sparing procedures supplemented with chemoradiotherapy. This paradigm shift can be attributed to the multimodality protocols initiated by the Intergroup Rhabdomyosarcoma Study Group (IRSG) over several decades , 26, 27.Although the survival rate of RMSs has been improved from 25% in 1970 to approximately 75% today, local tumor recurrence and metastasis remained challenging. Some authors noted that micro-metastasis is presumed leading to the high rate of failure with surgery alone . PrognosThis was an unusual occurrence of an ERMS in the larynx of a middle-aged man who was a non-smoker. Misdiagnosis and missed diagnosis of laryngeal RMSs might appear when tumors presented as smooth protuberance. Therefore, it is important for otolaryngologists to remain vigilant and to suspect, confirm, and localize these tumors. Presently, multidisciplinary treatment based on the anticancer drug sensitivity remains the mainstay of the management, and offers best results to the patients. This is ideal, but not represent the reality around the word.


Appendiceal mucinous neoplasms are rare findings defined by an accumulation of mucus within the vermiform appendix, and can be caused by a variety of conditions. Appendiceal mucinous neoplasms are important to consider because they can develop into pseudomyxoma peritonei as a consequence of perforation. We report a case of a 55-year-old man who initially presented with increasing abdominal girth, constipation, anorexia, and unintentional weight loss. Computed tomography examination of the abdomen and pelvis demonstrated a huge thin-walled cystic mass causing significant displacement of the surrounding abdominal and pelvic structures. The mass was amenable to resection and removed without perforation. Gross pathologic examination demonstrated a 44.0 × 40.0 × 23.0 cm unilocular cystic mass with a section of attached bowel. Microscopic examination revealed high-grade appendiceal mucinous neoplasm arising in a background of low-grade appendiceal mucinous neoplasm. This case report provides an evidence to include appendiceal mucinous neoplasms in the differential diagnosis of large abdominal cystic masses. Appendiceal mucinous neoplasm (AMN) is one of the causes of appendiceal mucocele, which is marked by dilation of the appendix from mucus accumulation In this report, we describe a case of a 44.0 cm mucocele caused by a high-grade appendiceal mucinous neoplasm (HAMN), which is significantly larger than any other previously reported case. In addition, we present the histological features that led to the diagnosis, discuss the classification of appendiceal mucinous neoplasms, as well as treatment options and prognosis.A 55 year-old man with medical history significant for hypertension presented to his primary care physician (PCP) with complaints of progressively increasing abdominal girth of 1 year duration, accompanied by unintentional weight loss, constipation, and poor appetite. On physical exam, the patient's height was 166.8 cm and weight was 86.2 kg. The patient's abdomen was distended, diffusely tender and firm, and measured 49 inches in circumference. Abdominopelvic ultrasound and contrast-enhanced computed tomography (CT) scan were performed. Ultrasound examination showed a huge mixed echogenic mass measuring 36.5 cm within the central portions of the abdomen and pelvis . No signAt surgery, the mass was well-circumscribed, with adhesions to the abdominal wall and the bowels. It strongly adhered to the lower right colon, near the cecum. There was a palpable otomy between the cecum and the mass. The mass was resected entirely intact while maintaining the ileocecal valve. No visible vermiform appendix was seen during the operation. The mass was sent to pathology for intraoperative consult. It was received as an intact, unoriented pink-red unilocular cystic mass with a small portion of attached bowel. The specimen weighed 18.6 kg, and cystic mass measured 44.0 × 40.0 × 23.0 cm . The masAdditional sections of the mass were taken for permanent sections. The wall of the mass showed diffuse hyalinization, with loss of muscularis mucosa and submucosa A. The maFollowing the surgery, the patient did well. He initially required 2 units of packed red blood cells for anemia and experienced postoperative ileus. After some time, the patient gradually advanced to a regular diet and was able to ambulate in the hallways without trouble. On the thirteenth postoperative day, the patient tolerated a regular diet and exhibited bowel movements. He was subsequently discharged and is still doing well to date.Appendiceal mucinous neoplasms are a heterogeneous group of neoplasms ranging from simple mucoceles to complex pseudomyxoma peritonei. Mucoceles are subset that consists of gradual cystic dilation of the vermiform appendix due to slow accumulation of mucoid contents. It is uncommonly seen, occurring in between 0.2% and 0.4% of appendectomies and 0.3 %and 0.7% of all appendiceal pathology The differential considerations of an abdominopelvic cystic lesion are wide and include ruptured appendicitis, mucinous cystadenoma, lymphoma, cecal carcinoma, ovarian and fallopian tube pathology that may mimic smaller appendiceal mucinous neoplasms. Characteristic findings for ruptured appendicitis on CT scan include significant periappendiceal inflammatory changes, focal defect in the enhancing appendiceal wall, extraluminal gas, or extraluminal leak of enteric contrast The classification of mucinous neoplasms of the appendix is controversial and different terminologies have been used to describe these lesions. Recent efforts to build a consensus naming system have led to the development of a classification system that includes LAMN, HAMN, and mucinous adenocarcinomas ,9,22. ThClinically, LAMN presents most often as acute appendicitis, and is more common in females, and in the sixth decade of life. Appendices that have LAMN can either be dilated by mucin, or can appear unremarkable HAMN is a relatively new term, resulting from a consensus of the Peritoneal Surface Oncology Group International in 2015. Previously, this entity was referred to as “noninvasive mucinous adenocarcinoma” or “cystadenocarcinoma,” which was deemed to be unclear and inconsistent with other terminology for appendiceal neoplasms A literature search for LAMN, HAMN, and appendiceal mucocele showed that the largest reported case of appendiceal mucinous cystic neoplasm measured approximately 17 cm in greatest dimension In conclusion, we report a case of HAMN, which is the larger than any other previously reported. HAMN and other mucinous neoplasms of the appendix must be included in the differential diagnosis for all cystic abdominal masses because rupture of such lesions can lead to PMP. Special efforts must be made during surgery to avoid rupture of cystic masses, especially appendiceal mucinous neoplasms. Further research and trials will have to be performed to outline standardized procedures in treating AMN including performing debulking surgery, chemotherapy use, and medical management for patients who are not surgical candidates. In addition, for patients with large neoplasms, standard of therapy is not outlined and may require physician judgment on anatomic structures that will have to be removed. Pathologists and surgeons will need to continue gathering data to identify a standardized diagnostic and treatment plan for AMNs in the future.Patient was notified and consented for the publication of this report.


Intracranial solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare spindle cell tumors of mesenchymal origin that include benign and malignant neoplasms.We present a 66-year-old male with a 5-year history of headache and dizziness, with left progressive sensorineural hearing loss over 1 month.WHO grade II SFT/HPC originating from the internal auditory canal in the left cerebellopontine angle.surgical resection.No local recurrence or metastases were observed in the follow-up 3 months after the surgery.Intracranial SFTs/HPCs are rare mesenchymal neoplasms that are challenging to manage. If the imaging characteristics of tumor are not typical, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis. The first SFT/HPC involving CNS was reported in 1996 by Carneiro et al CNS SFTs/HPCs account for only 0.09% of all meningeal tumors, while intracranial SFTs/HPCs are extremely rare. Intracranial SFTs/HPCs are extra-axially located and develop from the meninges. Intracranial SFTs/HPCs in the cerebellopontine angle (CPA) are frequently misdiagnosed as meningiomas or schwannomas. We report a case of a benign SFT/HPC in the CPA mimicking vestibular schwannoma (VS). Furthermore, we discuss the differential diagnosis of intracranial masses in the CPA and how to distinguish them based on imaging characteristics.Solitary fibrous tumors (SFTs) and hemangiopericytomas (HPCs) are rare spindle cell tumors of mesenchymal origin that account for less than 2% of all soft tissue masses.2A 66-year-old male presented to neurology outpatient department with a 5-year history of headache and dizziness, and 1 month ago he had left progressive sensorineural hearing loss. His vestibular testing was positive. Computed tomography (CT) of the head revealed a 3.0 × 2.7 cm well-defined slightly hyperdense mass without peritumoral edema in the left CPA. On enhanced CT, the mass was homogeneously enhancing, extending into the enlarged internal auditory canal (IAC). Given the CT characteristics, a provisional diagnosis of a VS was made , and Vimentin. Cells were negative for CK, EMA, S-100, GFAP, SMA, olig-2. With these results, a diagnosis of WHO grade II SFT/HPC was made (Fig. 3 various extra-pleural occurrences of these tumors have been reported. Intracranial SFTs/HPCs are rare mesenchymal neoplasms and constitute a heterogeneous group of rare spindle cell tumors that include benign and malignant neoplasms. Intracranial SFTs/HPCs usually occur in the fifth decade, but without significant gender predilection. When the tumors become large enough or infringe into the important functional areas, patients will show clinical symptoms including episodic headaches, dizziness, gait imbalance, sensory disturbance, hemiplegic paralysis, and epileptic seizure.,12Since the first SFT/HPC described by Klemperer et al in 1931 as a pleural tumor, T2-hypointense areas represent fibrosis components that show intense contrast enhancement, while T2 iso- or hyperintense areas represent hypercellular components that show moderate heterogeneous enhancement. The combination of these 2 components gives rise to the so-called yin yang sign, which is associated with intracranial SFTs/HPCs. For WHO grade II and III, the tumors usually show intermediate-high signal intensity in T2WI. Tortuous flow-empty vascular shadow is often seen inside or on the surface of these 2 grades tumors, which is essential to differentiate WHO grade I with WHO grade II and III. Besides, the incidence of peritumoral edema in WHO grade II and III is higher than WHO grade I. But in this case, there is no peritumoral edema. SFTs /HPCs in the CPA are frequently misdiagnosed as meningiomas or schwannomas. Radiologically, Meningiomas show dural tail sign and calcification more frequently than SFTs/HPCs; in contrast, SFTs/HPCs show necrosis, cystoid degeneration, and flow-empty actions more frequently than meningiomas. The adjacent bone may help to differentiate meningioma from SFT/HPC. The adjacent bone is often thickened in meningiomas; SFT/HPC, in contrast, can erode the adjacent bone. Schwannoma originates from Schwann cells of the nerve sheath and grows along the nerve sheath. The patient with schwannoma usually has corresponding neurological symptoms. Cystoid degeneration is often seen in these lesions, so MRI usually shows long T1 and long T2 signals. VS is the most common cranial nerve schwannoma. VS is also the most frequent tumor of the CPA and IAC, and arises from Schwann cells around the vestibular nerve and ganglia. The gold standard imaging modality for VS diagnosis is T1WI MR imaging of the IAC with and without contrast. Without contrast, VSs are hyperintense relative to cerebrospinal fluid and are isointense to hypointense relative to gray matter on T1-weighted sequences. VSs obviously enhance with contrast. Cystic VSs can also occur with frequency of 11% to 48%. The expansion of IAC can be used as an important basis for evaluating VS. In this case, CT and MRI both showed that the tumor extended into the enlarged IAC and closely connected to the vestibulocochlear nerve. Besides, the patient had left progressive sensorineural hearing loss. So given the imaging and clinical features of this patient, the tumor is misdiagnosed as VS in this case. If the imaging characteristics of tumor are not typical, an accurate diagnosis is difficult to made. Then, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis. Immunohistochemically, SFTs/HPCs usually show reversed 12q13 and the NAB2–STAT6 fusion gene, while meningiomas are typically reactive for EMA.Radiologic features can be helpful in predicting tumor pathology, while pathological examination remains the gold standard in its diagnosis and pathological grading. However, no imaging modality correlates perfectly with any diagnosis. The signal intensity in T2WI of SFTs/HPCs’ solid portions can be helpful to evaluate the pathological grade. For WHO grade I, a majority of the cases show intermediate-low signal intensity in T2WI. A minority of the cases are made of 2 obviously different signal intensity areas in T2WI. Melone et al found that SFTs/HPCs invading the cerebral venous sinuses significantly recurred earlier. Invasive skull base SFTs/HPCs or those infiltrating deeply into a venous sinus, usually cannot be entirely removed without high risks of postoperative complication. Therefore, the extent of resection is the most powerful prognostic factor for recurrence of SFTs/HPCs. Preoperative embolization, preoperative chemotherapy, and postoperative adjuvant radiotherapy have been performed. Radiotherapy after surgical resection of SFTs/HPCs continues to be controversial. Most neurosurgeons and neuro-oncologists would advocate adjuvant radiotherapy especially if the tumor was not entirely excised. No conclusion can currently be drawn regarding the effectiveness of these measures. In the CNS, the clinical behavior of SFTs/HPCs is particularly aggressive, with recurrence rates of 61% to 76% and metastasis rates of 23% to 64%. Reported 5-year overall survival probabilities vary between 67% and 96% with median overall survival between 7 and 16.2 years.Surgery is recommended as the criterion standard of treatment. Complete excision of the mass is clearly superior to incomplete excision, and sub-totally removed SFTs/HPCs may recur or continue to grow.In conclusion, intracranial SFTs/HPCs are rare mesenchymal neoplasms that are challenging to manage. For their rarity and resemblance to other more common brain tumors, they are often poorly recognized. In other words, if the imaging characteristics of tumor is not typical, clinicians should depend on tissue biopsy and immunohistochemistry to make a definitive diagnosis. SFTs/HPCs are associated with a significant risk of recurrence that may reduce overall survival. Even if no researches have showed the effectiveness of these measures, most clinicians advocate that tailored maximal tumor resection upon initial surgery is beneficial and that postoperative adjuvant radiotherapy is useful for SFTs/HPCs displaying grade II or III, even in case of complete resection.Data curation: Jie Huang, Ya-qi Zhu.Supervision: Yong Du.Writing – original draft: Xi Yue.


Oral metastatic tumors due to malignancies at distant locations are very rare. There are only few cases in the literature reporting about mandible/cervical metastases. Sometimes the oral finding is the manifestation of the disease.We report on a 50-year-old patient who was diagnosed with a pathological mandibular fracture and cervical lymph node metastases due to an adenocarcinoma of the lung without other peripheral metastases. Following the interdisciplinary tumor board decision, resection and ipsilateral neck dissection including a mandible reconstruction using a microvascular fibula transplant was performed. The patient received an adjuvant chemotherapy with carboplatin/pemetrexed.The combination of cervical metastases and a pathological mandibular fracture has not been reported before. Distinction between oligometastatic and polymetastatic disease may be difficult. Bone lesions and a high number of metastases are associated with a negative outcome. In cases of limited oligometastatic diseases, metastasectomy might sometimes be indicated. Due to the absence of other peripheral metastases decision of metastasectomy was made.Due to rare occurrence of cervical metastases special attention should be paid to the incidence of possible metastases of distant malignancies at uncommon regions. With a In this report, we describe the case of pulmonal adenocarcinoma metastases to the mandible bone and cervical lymph node (level I and II) as the first sign of a metastatic tumor. The patient was referred to our department of oral and maxillofacial surgery by his dentist. This work has been reported in line with the SCARE criteria .2A 50-year-old male was referred to the emergency department by his dentist with a perimandibular swelling. In the physical examination there was an indolent, fluid-filled swelling intraorally in the left mandible region with signs of infection. In his medical history an adenocarcinoma of the lung (pT3 pN2b L1 V1 Pn1 R1 M0) was treated operatively with the resection of the right superior lobe and radiotherapy 16 months ago. The patient is a former smoker, not taking regular medication. The common radiological diagnostics, including computed tomography (CT), showed a mandible fracture  and abno3Dentists and surgeons are often confronted with unspecific symptoms like swelling, paresthesia, bleeding or tooth mobility . The attHere, metachronous oligometastases of the primary adenocarcinoma of the lung were histologically and immunohistochemically identified in the mandible as well as cervical lymph nodes. One has to distinguish between oligometastastic and polymetastatic disease, understanding oligometastases as a state where local cancer treatment can still be curative . The defIn this case, mediastinal lymph node involvement or other peripheral metastases could be excluded. The isolated cervical lymph node metastases of level I and II and the pathological mandibular fracture may be classified as extrathoracic metachronous oligometastases . To the 4Besides the rare occurrence of mandible metastases, this case proves that simultaneous cervical metastases are also possible. In cases with primary malignancies at a non-oral distant location, dentists and surgeons have to give special attention to the incidence of possible metastases of these malignancies.The authors have no conflicts of interest.There was no funding of the research.No ethical approval needed.Written consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.CS: Data collection, writing manuscript.CD: patient’s surgeon, data collection.JOV: patient’s surgeon, data collection, case discussion.NT: radiological assessment, visualization, recommendation in tumor conference, proof reading.RW: patient’s surgeon, recommendation in tumor conference, proof reading manuscript, visualization.MH: Conceptualization, patient’s surgeon, recommendation in tumor conference, proof reading manuscript.BBB: Conceptualization, writing, recommendation in tumor conference, supervision.None.Dr. Claudius Steffen.Not commissioned, externally peer-reviewed.


Plexiform schwannoma (PS), variant of schwannoma, often involves multiple fascicles as plexiform neurofibroma, and is usually located superficially on the dermis and subcutaneous layers. Spinal PS is extremely rare, and there is insufficient information on its natural course and treatment strategy. We describe the clinical features and treatment of giant intradural PS at the lumbosacral spine.A 66-year-old man presented with leg pain, paresthesia, and weakness for 2 years. Magnetic resonance imaging demonstrated a large mass lesion involving a continuous multi-lobulated bead-like mass and a cystic portion from L1 to S3. The lesion was iso-intense on T2-weighted images (WI), iso- to slightly low-intense on T1-WI, and heterogeneous enhancement on contrast-enhanced T1-WI. The large mass lesion had three portions, including a cystic mass at L1, continuous multi-lobulated bead-like mass with a cystic portion from L2 to S1, and multi-lobulated mass from S2 to S3, which were identified with severe adhesions with cauda equina on operative assessment. Grossly total extirpation was achieved at the lumbar spine, and remained three round shaped small masses at the lumbar area and a multi-lobulated round masses from S2 to S3 involving nerves related with motor function of the lower extremities and anal sphincter, respectively. Histological examination revealed multinodular or plexiform growth pattern composed of spindle-shaped tumor cells, which were diffusely and strongly positive for S100 protein with KI67 < 1%. There were no recurrence of preoperative symptoms and changes of the remained masses over a 2-year follow-up period.Subtotal extirpation to minimize neural deficits and close observation can be considered an appropriate treatment strategy for a giant spinal PS considering its benign prognosis and histological features, with a high risk of neurological damage during surgery. Schwannoma is a slow-growing benign tumor arising from Schwann cells of the nerve sheaths of peripheral nerves and believed to originate from embryonic neural crest cells . GeneralWe report the experience of surgical treatment and 2-year follow-up for the spinal giant intradural PS located extensive lumbosacral area.A 66-year-old man presented with both leg pain and paresthesia for 2 years. The symptoms aggravated in recent 6-month. The patient had a history of alcoholic liver cirrhosis and cured stomach cancer after subtotal gastrectomy 15 years ago with hematological abnormalities including thrombocytopenia (94 K) and anemia (7.6). Neurological examination showed grade 4 motor weakness of the lower extremities and neurogenic claudication with no bowel or bladder dysfunction. Magnetic resonance imaging demonstrated a large multi-lobulated bead-like mass lesion with a cystic portion from L1 to S3. The lesion was iso-intense on T2-weighted images (WI) and iso- to slightly low-intense on T1-WI with heterogeneous enhancement (Fig. The extensive multi-lobulated mass lesions were located intraspinal canal and attached with neural components, which is judged to be difficult to access for a biopsy. We decided on surgical treatment to confirm a histological finding and improve the symptoms with removal of the mass lesion. The large mass lesion had three portions, including a cystic mass at L1, continuous multi-lobulated bead-like mass with cystic portion from L2 to S1, and multi-lobulated round masses from S2 to S3, which were identified under dural opening after total laminectomy of L1-S2. There were severe adhesions and tangles between the large mass lesion and the cauda equina with thickened arachnoid membrane and septum. The mass lesion from L1 to S1 was gross totally removed. We retained several small masses at the lumbar spine and multi-lobulated round masses from S2 to S3 involving nerves related with motor function of lower extremities and anal sphincter, respectively, under intraoperative monitoring.The patient experienced voiding difficulty with no other additional neurologic deficits after surgery. Histological examination revealed multinodular or plexiform growth pattern composed of spindle-shaped tumor cells. The tumor cells had elongated wavy nuclei and arranged in fascicular pattern with focal nuclear palisading, which were diffusely and strongly positive for S100 protein with KI67 < 1% Fig. . A benigPS shows histologic features similar to those of conventional schwannoma, except for a more Antoni A pattern. However, PS shows complex growth and often involves multiple fascicles, and this differs from the single fascicle involvement of conventional schwannoma. Based on these features, it is difficult to achieve complete excision of PS without damaging multiple fascicles and neurological deficits . DiffereThree cases of spinal giant intradural PS have been reported in the literature, two were treated with grossly total extirpation and one was treated with subtotal extirpation Table . The gia


Our patient was a female indigenous preterm infant born to a mother on a high dose of methadone. On admission at the age of 12 h, she was found to have significant gastric distension. Initial abdominal X-ray showed a large gastric bubble with little evidence of rectal gas. Malrotation was suspected and surgical intervention was discussed. However, repeat abdominal X-ray, ultrasound and upper Gastrointestinal series were found to be normal and without acute findings. Thus, surgery was avoided. The gastric distension resolved spontaneously. She never required opiate therapy for neonatal abstinence syndrome. Given the pattern of gas seen on the initial abdominal X-ray and its spontaneous resolution after removal of maternal methadone, we suspect this baby had neonatal narcotic bowel syndrome. This has never been reported in the literature and is a unique finding. Given the lack of current reports, further observations for this syndrome should be conducted.Narcotic bowel syndrome is defined as worsening abdominal bloating and cramping with chronic opiate use, leading to paralytic ileus. This syndrome is common yet underreported in adults. However, there is no current evidence of such conditions in the newborn after exposure  In the given case report, we present a neonate with physical signs and radiologic evidence of ileus secondary to maternal methadone ingestion.Opioids are potent analgesic agents used to treat severe pain that is both acute and chronic in nature . Common This baby girl was born via home spontaneous vaginal delivery at approximately 32 weeks to a mother with no prenatal care or ultrasounds. She was HIV negative, and the remainder of serology results and GBS status were unknown. Subsequent postnatal screening was negative. The mother had a remote history of illicit drug use and was on maintenance therapy with methadone 100 mg/day throughout pregnancy. She was taking it as prescribed, which was confirmed by home visits. She had no other illicit substance use. She did intermittently smoke cigarettes and marijuana during the first trimester of pregnancy. She did not specify any alcohol use. Baby was born at home by spontaneous precipitous delivery and brought to a peripheral hospital by ambulance for further care. Her birth weight was 1392 g. Secondary to worsening respiratory distress, she was intubated and transferred to our tertiary care center.On admission, she was screened for infection and started on empiric antibiotics. Blood cultures were negative. Baby was clinically stable but was found to have abdominal distension and was nil per os. The initial abdominal X-ray  showed mTherefore, an abdominal ultrasound was conducted to rule out malrotation and assess the gut vasculature. The superior mesenteric artery and vein were found to be in normal orientation. Furthermore, pediatric surgery was promptly consulted after the ultrasound and saw no acute need for exploratory laparotomy. They recommended an upper Gastrointestinal (GI) series after 2 days of gastric decompression. In the meantime, the patient was hemodynamically stable. The upper GI series showed no acute obstruction or other significant findings . The abdFor more than 30 years, methadone has been used as a first-line medication for opioid substitution therapy during pregnancy . DespiteThere is well-documented evidence of delay of feeding onset and attaining full feeds in neonates with NAS or exposed to morphine in the NICU for analgesia , 10. ForInterestingly, there was a case report of an infant in Italy who experienced similar paralytic ileus after treatment with fentanyl for procedural sedation while ventilated . Althougin utero exposure to methadone and was thought to have a malrotation on imaging. After ruling out a surgical cause of obstruction, the only factor that could be associated with such significant ileus was determined to be maternal methadone consumption. Given the pattern of gas seen on the initial abdominal X-ray and its spontaneous resolution after 72 h, we suspect this baby had paralytic ileus secondary to maternal methadone treatment. This has never been reported in the literature and is a unique finding. Hence, this correlation is a reminder to neonatology decision makers that maternal substance use may be a significant considering factor when sending a patient with small bowel obstruction to the operating theater. Given the lack of current reports, further surveillance for this phenomenon should be conducted.In conclusion, the given neonate was born premature after significant None declared.No sources of funding were used.Ethics approval for said case report was waived. Parental consent was gained prior to creating this manuscript.All images and information presented about the given minor were used with informed consent from the individual’s legal guardian. All images were anonymized.Veronica Mugarab Samedi is a guarantor for this publication.


Amyand hernia is a rare disease seen in approximatively 1% of all hernias, complications of it, like acute appendicitis, or perforated appendicitis are even more rare, about 0.1%. Its diagnosis is very difficult in the preoperative period: it is usually an incidental finding.We report an unusual case of perforated gangrenous appendicitis with peri-appendicular abscess occurring in an irreducible Amyand’s hernia. An 80-year-old male, with chronic obstructive disease and pulmonary emphysema, atrial fibrillation, acute myocardial infarction, underwent urgent surgery, under local anesthesia, for right incarcerated inguinoscrotal hernia.He was found to have a perforated gangrenous appendicitis with peri-appendicular abscess within a right indirect inguinal hernia sac. Appendicectomy and Bassini’s hernia repair were performed under local anesthesia without any complications.The treatment of Amyand’s hernia is not standardized. The current generally accepted algorithm for Amyand’s hernia is essentially contingent on the appendix’s condition within the hernia sac.Appendectomy and primary herniorrhaphy, under local anesthesia, for type 3 of Amyand’s hernia, is a safe procedure and easy to perform and, if confirmed by further study, could be part of every surgeon’s knowledge. It is not unusual to find an incarcerated hernia (defined as the inability to reduce the hernia content); typically the hernia content is the omentum or small bowel .In a very low frequency one can find the cecal appendix inside the hernia sac: this condition is called “Amyand’s Hernia” whether it is inflamed or not .In 1735 C. Amyand described the first case of incarcerated inguinal hernia, containing a perforated appendix, in an 11-year-old boy .TablMost of the authors in case of noninflamed appendix do not suggest removal. The transection of a fecal-containing organ in an otherwise clean procedure may increase morbidity and mortality from septic complications and, again, the surgical manipulation, during appendectomy, in the base of the caecum could increase the recurrence rate of the inguinal hernia, due to detachment in the deep inguinal ring ,8.However, some authors propose a prophylactic appendectomy for the risk of future appendicitis .If an acute appendicitis is associated with Amyand’s hernia, the classical recommendation is to proceed with appendectomy and herniorrhaphy. The use of mesh in such a situation remains controversial. According to Losanoff’s classification in every case of appendectomy a mesh should not be used due to the risk of infection and septic complications.The treatment of choice in these cases, also as reported by Sharma H et al. in the series of 18 consecutive cases of Amyand’s hernia, is appendectomy using Bassini or Shouldice repair, along with thorough abdominal and pelvic washouts to reduce septic complications .However, there are documented reports of successful outcomes for hernias, identified as type 2 Amyand’s hernia, performing appendectomy and tension free repair with mesh, as reported by Shaban et al. .Laparoscopic repair of Amyand’s hernia, with and without mesh placement, is increasingly reported in literature .Appendectomy can-be performed under general, regional and local anesthesia but the anesthetic risks are least with the latter. Jebbin NJ, in 2007, reported that appendectomy under local anesthesia is safe and effective .Sharma et al., in a prospective study of 165 patients with appendicitis, reported that appendectomy under local anesthesia is quick, cost-effective and carries little morbidity and can be used for all ages .We report the case of an eighty-year-old man who arrived at the operating table diagnosed with a strangulated right inguinal hernia, who underwent surgery under regional anesthesia as we usually do with elderly patients and with concomitant pathologies that contraindicate general anesthesia.During the operation, a perforated gangrenous appendicitis with inguinoscrotal abscess (Amyand's Type 3) is found when the hernial sac is opened. We proceeded with appendectomy, under local anesthesia, without any discomfort for the patient.Similar to what Sharma H. reported , at the According to Losanoff, prosthetic repair of the hernial defect was avoided by preferring a primary Bassini’s hernioplasty.In 2008, Tisdale and Barwell reported a similar case of appendicitis, with a peri-appendicular abscess, in an irreducible Amyand’s hernia treated with appendectomy and sutured hernia repair performed under local anesthesia .4We provide a case report of a rare entity known as an Amyand’s hernia that presented an incarcerated that was diagnosed intraoperatively with a perforated gangrenous appendicitis, known as type 3, and treated with an appendectomy and primary herniorrhaphy, under local anesthesia, with a successful outcome.This procedure, safe and easy to perform, if confirmed by further study, could be part of every surgeon’s knowledge.All the authors certify that there is no conflict of interest regarding the material discussed in the manuscript.All the authors declare that this research didn’t receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval has been exempted by our institution because this is a case report and no new studies or new techniques were carried out.Written informed consent was obtained from the patient for publication of this case report and accompanying images.Not Applicable.The guarantor for this case report is Giovambattista Caruso.Not commissioned, externally peer-reviewed.Giovambattista Caruso: Conceptualization, Writing - original draft, Writing - review & editing, Data curation. Chiara Toscano: Resources, Data curation. Giuseppe Evola: Methodology. Salvatore Antonio Maria Benfatto: Visualization. Martina Reina: Data curation. Giuseppe Angelo Reina: Supervision.


Pilonidal sinus (PNS) is a chronic inflammatory perianal disorder that rarely occurs outside sacrococcygeal region. The aim of this study is to report an extremely rare case of bilateral inframammary PNS with brief literature review.A 25-year-old female presented with a discharging sinuses in both inframammary region for two years. Examination showed multiple discharging sinuses with several centimeters of induration and tenderness. Under general anesthesia, complete excision of the sinuses with primary closure done. Histopathological examinations showed chronic foreign body granuloma surrounding hair shaft pictures consistent with PNS.Inframammary PNS has never been reported in the literature. As with intermammary PNS, in this case also it is associated with obesity and large breasts with tight brassieres. Diagnosis is usually clinical. In contrast to sacrococcygeal PNS, operation under general anesthesia is main treatment modality.Pilonidal sinus of inframammary region is an extremely rare condition. High index of suspicion is required for diagnosis. Excision with primary closure is the definitive therapy. The mosUp to date, 17 cases of intermammary PNS have been reported in the literature, while no inframammary case has been reported . This st1.1A 25-year-old female presented with multiple discharging sinuses in the both inframammary areas that extends into the intermammary region for two years. Past and family histories were clear. She was non-smoker and single.1.2There were multiple discharging sinus extending from left inframammary region, through the intermammary area to the right inframammary sitewith 8 × 9 cm of induration and tenderness .Fig. 1Mu1.3After preparation for general anesthesia, complete excision of the sinus tracts was performed through a butterfly shaped incision . Primary1.4Two weeks later, the drain was removed. After six months, the scar was healthy, there was no sign of recurrence.2PNS is a suppurative disease that occurs due to hair penetration of epidermis, causing a sequelae of foreign body reaction: inflammation, sinus formation and granulation tissue lined tract . PNS mosThe etiology for PNS was not known until recently (Although the etiology is not well known but nowadays the disease has an acquired etiology) . PNS forThe fourth cause is pressure on atypical areas, such as intermammary area by pressure effect of breasts ,16. RepoIn general, the reported risk factors for PNS are hairiness, young age, male gender, prolonged sitting, deep navel and cleft and poor personal hygiene . While tThe differential diagnosis for an atypical PNS is a long list such as hernia, endometriosis, urachal cyst, epidermoid cyst, pyogenic granuloma, dermoid cyst and infected sebaceous cyst .The diagnosis of atypical PNS is not straight forward. Most of intermammary PNS cases can’t be diagnosed preoperatively. Shreef et al. reported 12 cases of intermammary PNS, where only 25% of the cases could be diagnosed preoperatively . In the There is no standard strategy for management of PNS. It varies from extensive resection to conservative therapy. Injection of a mixture (100 g petroleum jelly (Vaseline) +50 g henna powder (Lawsoniainermis powder) +5 g tetracycline, that was stored at 2 °C–8 °C.) to the PNS site had an extraordinary effect on the patients; they returned to work immediately compared with the operative groups, who stayed at home for at least 10 days . ManagemDefinite therapeutic approach of intermammary PNS is excision and primary closure . The curIn conclusion, pilonidal sinus of inframammary area is an extremely rare condition. It is mostly associated with obesity and large breasts with tight brassieres. As intermammary PNS, excision with primary closure is the definitive therapy.No source to be stated.Approval is not necessary for case report in our locality.Consent has been taken from the patient and the family of the patient.Abdulwahid M. Salih: Surgeon performed the operation and follow up.Tomas M. Mikael, Rawand A. Essa, Fahmi H. Kakamad, and Mohammed Q. Mustafa: Writing the manuscript.Shvan H. Mohammed, Diyar A. Omar, Karukh K. Mohammed, Hunar Ali Hassan, Masrur S. Aziz, Drood C. Usf, Kayhan A. Najar: literature review, final approval of the manuscript.Not applicable.Fahmi Hussein Kakamad.Not commissioned, externally peer-reviewed.There is no conflict to be declared.


The submental island flap (SIF) is a reliable option for reconstructing defects in the facial region and offers several advantages when compared to free-flap alternatives. While the reconstructive applications of the SIF have been demonstrated in the lower face, there are limited reports on its utility as a composite flap for reconstructing defects of the upper facial skeleton. To our knowledge, we report the first cases of composite (osteocutaneous) SIFs used for reconstruction of complex facial defects involving the zygoma and lateral orbit respectively.Three consecutive cases are presented. All were performed following resection of skin cancers with invasion of the upper facial skeleton. The first case was a 68-year-old male with a longstanding history of non-melanoma skin cancers who presented with a 7 cm recurrent basal cell carcinoma (BCC) with bicortical invasion of the left zygoma. The second case was an 88-year-old female with several squamous cell carcinomas (SCC), including a dominant 7.1 cm SCC on the right temple with orbital invasion. A third case was a 75-year-old immunosuppressed male with a 6.5 cm SCC of the right cheek with invasion of the orbit and zygoma following prior resection as well as high dose radiotherapy. The operative management of all cases involved harvesting the SIF on its vascular pedicle alongside the inferior portion of the mandible with rigid fixation to address the bony defects. The first case was robust throughout adjuvant radiotherapy with no flap complications after 2 year follow up. The second patient received adjuvant radiation therapy to an area that was previously radiated. Although the flap remained viable for a year, the patient experienced delayed soft tissue loss over the bony segment and eventual devitalization of the distal flap. The third case achieved a satisfactory result with no complications.Our case series outlines a unique application of the composite (osteocutaneous) submental island flap (SIF) for reconstruction of complex facial defects involving the upper facial skeleton. The osteocutaneous SIF should be used with caution in patients receiving adjuvant radiotherapy who have a history of previous radiation to the same or overlapping field. Facial reconstructive surgery is particularly challenging following resection of invasive cancers in the head and neck region. Complex facial defects involving bone often require free flaps from distant donor sites to optimize functional and aesthetic outcomes. Free flaps are chosen in these circumstances to provide robust areas of soft tissue with vascularized bone , 2. HoweThe pedicled submental island flap (SIF) is gaining popularity for its diverse applications in facial reconstructive surgery. The flap was first described by Martin et al. and is supplied by the submental vessels of the facial artery or retrograde flow from the angular artery , 4. The A unique feature of the SIF is the ability to harvest the flap with the inferior portion of the mandible as a source of vascularized bone , 5. ThisIn the present series, we report three consecutive cases of composite (osteocutaneous) SIFs used for reconstruction of the upper facial skeleton. All cases required the resection of aggressive, recurrent skin cancers followed by reconstruction using the composite SIF. To our knowledge, we report the first cases of osteocutaneous SIFs used for reconstruction of complex facial defects involving the lateral orbit and zygoma respectively.A 68-year-old male was referred to our institution for surgical management of a large, 7 cm, recurrent basal cell carcinoma (BCC) involving the upper face. Clinical examination revealed an ulcerative lesion impinging on the left canthus and lateral orbit with extension to within a centimeter of the helical root posteriorly. Computed tomography (CT) confirmed extensive invasion of underlying tissues including bony invasion of the zygomatic arch and posterior body of the zygoma Fig. .Fig. 1aA wide local excision of the primary tumor was performed including the entire temple from the left lateral canthus and orbit to the route of the helix and tragus. The tumor was found to penetrate extensively into the underlying tissue, and thereby required a limited parotidectomy with further dissection of the temporal fossa. Ultimately, the entire lateral canthus, large portion of the left lower eyelid, entire zygomatic bone, left lateral orbit and sections of mid-facial muscles were resected leaving a large facial defect.A composite submental island flap  was harvested with the inferior border of the mandible  Figs.  and 3. AThe patient remains disease-free with no complications on 2-year follow up. The flap was robust throughout adjuvant radiotherapy. The patient required one revision procedure to address lower lid ectropion after radiation, as well as an unrelated BCC. The latter was done with a concurrent mid-brow lift to address a pre-existing brow ptosis and to provide a full thickness skin graft for the lower lid malposition.An 88-year-old female was referred to our institution for surgical management of recurrent squamous cell carcinomas (SCC). Clinical and radiological examination identified a 7.1 cm right temple mass with extensive invasion of underlying tissues including the lateral orbit. The patient experienced diplopia and diminished mobility of the right eye. A head CT revealed a mass continuous with the lacrimal gland, lateral rectus muscle and globe with extension into the lateral orbital wall and roof.The tumor resection resulted in a large orbital and temporal defect. The orbital roof resection unfortunately led to a cerebral spinal fluid leak Fig. . The durThe post-operative pathology confirmed clear resection margins. Although the flap was completely viable, radical adjuvant radiation therapy led to loss of overlying soft tissue over time Fig. . It is iA 75-year-old male with a history of extensive cutaneous malignancies was referred to our institution for surgical management. His malignancies occurred in the context of chronic immunosuppression following kidney transplantation and insulin-dependent diabetes mellitus. Previous treatments had included 3 prior surgeries, 2 before radical radiation and one after as a salvage procedure involving radical parotidectomy and segmental resection of the right zygomatic arch and lateral orbit. Pathology showed an invasive 6.5 cm SCC with close margins and multiple positive regional nodes. A 3.5 cm recurrence was identified in the lower face at 6 month follow up.The patient underwent additional surgery with reconstruction of the SCC defect of the lower face as well as the zygomatic and temporal defect of the upper face Fig. . The ostThe submental island flap (SIF) is an excellent option for reconstructing a variety of defects that extend beyond the lower facial region. Several studies have outlined practical advantages of the SIF, including safe dissection by residents in-training, enhanced outcomes when compared to radial forearm free flaps for similar defects, and excellent cosmetic results at both defect and donor sites , 3, 9–13When compared to free-flap alternatives, the SIF offers several advantages which include reduced operating time, lower health care costs and no requirement of microvascular expertise , 11. TheThe limitations of the SIF include unwanted hair growth on the flap and the much more concerning potential for transfer of malignant upper neck nodes leading to recurrent cancer . HoweverTo date, the reconstructive applications of the SIF have been focused predominantly on the lower face, oral cavity and pharynx. Our demonstration of the composite SIF to reconstruct facial defects involving the zygoma and lateral orbit has not been described previously.The composite (osteocutaneous) submental island flap has unique applications for reconstructing complex defects involving the upper facial skeleton. This flap can be used in patients requiring adjuvant radiation, and in those who have received prior radiotherapy and require salvage surgery. Caution should be used in patients who require more than one course of radiation to the same area. Further research is required to validate the effectiveness of the composite SIF in large patient cohorts.