Bevacizumab has shown good efficacy in radiation necrosis (RN) following gamma knife radiosurgery (GKRS) and associated peritumoral edema. However, few studies have reported bevacizumab failure. Moreover, the pathologic transformation of benign meningioma following GKRS has never been reported.A 41-year-old man was admitted with focal seizure on the right arm.Magnetic resonance imaging (MRI) demonstrated a 4.7 cm-sized convexity meningioma involving left motor cortex.Subtotally resected tumor was confirmed as a meningothelial meningioma and subsequently treated by GKRS. During 4-year follow-up after GKRS, seizure and hemiparesis had persisted with progressively worsened peritumoral edema regardless of steroid and bevacizumab treatment. Radical debulking of tumor was achieved and immunohistopathological examination revealed angiomatous meningioma with necrotic core presenting scanty VEGF expression.A follow-up MRI at 4 months after debulking surgery showed a marked reduction of peritumoral edema with improvement of symptoms.This is the first report of pathologically confirmed angiomatous transformation following GKRS. Although the pathogenesis is not fully understood, this rare pathologic transformation may be closely related to RN. Also, if bevacizumab is resistant, debulking surgery for reducing tumor burden could be an effective treatment option to control the RN. The consensus is that GKRS is an effective treatment modality irrespective of whether primary or adjuvant treatment, with long-term tumor control rate of >80%. It is often accompanied by peritumoral edema resulting in progressive neurologic deficits. Vascular endothelial growth factor (VEGF) has been generally accepted as a key factor for RN.–7 VEFG, a known regulator of angiogenesis and vascular permeability, is expressed in both necrotic core and peritumoral brain tissue. Bevacizumab, an anti-VEGF antibody, has shown definite efficacy to RN.,10 It is more specific to RN than other medical treatments with evidence of radiological and clinical improvement. However, most studies have only focused on bevacizumab efficacy during short follow-up period without pathologic confirmation and no studies have reported bevacizumab resistance.Radiation necrosis (RN) is one of the most common complications following GKRS accounting for 10% of patients. Thus, it is not clear whether angiomatous lesion occurred after GKRS.More interestingly, pathologic transformation of benign meningioma has never been reported. Only one case of angiomatous lesion was reported, but it lacked evidence because pathologic evaluation was not performed before GKRS.In this report, we present the case of a patient with bevacizumab-refractory RN following adjuvant GKRS for benign meningioma and discuss the possible relation with this refractory RN and pathological angiomatous transformation.2Written informed consent was obtained from the patient for publication of this case report and accompanying images2.1A 41-year-old man presented with focal seizure on the right arm. Contrast-enhanced magnetic resonance imaging (MRI) revealed an ∼4.7 cm sized, well defined, and heterogenously enhanced mass with minimal edema in the left motor cortex, consistent with a convexity meningioma. A left frontoparietal craniotomy was performed and the tumor was subtotally resected because significant cortical adhesion with rich cortical veins around tumor was observed. There was no decrease or change on intraoperative neurophysiologic monitoring. Microscopically, the tumor was confirmed as a meningothelial meningioma (WHO grade I) without necrosis. Regrowth of the remnant tumor was observed at 10 months after surgery, so GKRS was performed with marginal dose of 13 Gy at 50% isodose line . The response and efficacy of the bevacizumab was determined by both radiologic improvement (decrease edema in T2-weighted MRI) and clinical improvement. After administration of 8 cycles of bevacizumab, frequency of seizure decreased and follow-up MRI indicated slight reduction of peritumoral edema. During bevacizumab treatment, he suffered only transient general weakness without grade 2 side effects or above. However, his neurologic conditions had gradually worsened again. Even though additional 4 cycles of bevacizumab were given, peritumoral edema persisted , which was previously confirmed as a meningothelial meningioma in the first operation. At 4 months after the second operation, follow-up MRI showed marked reduction of peritumoral edema  until symptoms and imaging improved.,15 In our case, the patient was treated for RN with bevacizumab at 5 mg/kg, q4weks, for total 12 cycles. The patient initially showed slight improvement of peritumoral edema in imaging after 8 cycles of bevacizumab. So, additional 4 cycles were used, however, the peritumoral edema was worsened again suggesting treatment failure. This failure may be related to excessive vessel reduction around RN by bevacizumab, aggravating the ischemia and hypoxia which result in paradoxically exacerbating the necrosis. Due to the bevacizumab only targets blood vessel around RN, not the necrosis itself, it can rather aggravate further necrosis. So, in bevacizumab-refractory cases, additional surgery is needed for accurate diagnosis and removal of necrotic core which is irreversible. In our case, the patient underwent debulking surgery for pathologic diagnosis and reducing tumor burden. The specimen showed coexistence of viable tumor tissue and partially necrotic tissue, considered as RN. A follow-up MRI at 4 months after surgery demonstrated definite reduction of peritumoral edema.Many studies, including a randomized double-blind placebo-controlled trial, reported that bevacizumab, a monoclonal antibody for VEGF, achieved reduction in the volume of necrosis on T2 Flair and T1-weighted gadolinium-contrast MRI with improvement in clinical symptoms. In addition, rich collateral veins were observed around the tumor in our case. Radiation injury causing blood vessel necrosis might result in another local hypoxia with neovascularization contributing to angiomatous transformation.More interestingly, initial pathology demonstrated benign meningothelial meningioma, but it was transformed to benign angiomatous meningioma at 4 years after GKRS. Although, the pathogenesis is unknown, it is possible that this phenomenon is closely linked to etiology of RN. RN characterized by disruption of blood–brain barrier and vasculopathy and subsequent tissue hypoxia may trigger pathologic neovascularization resulting in angiomatous transformation. So, we initially expected that extensive VEGF expression triggered by RN and angiomatous transformation may contribute to severe peritumoral edema. The specimen of 2nd surgery showed coexistence of viable tumor tissue and partially necrotic tissue, considered as RN, however, immunohistochemical staining for VEGF showed less positive reactivity than expected. This may also be related to anti-VEGF effect of bevacizumab as mentioned above.By comparing the non-angiomatous meningiomas, it is agreed that angiomatous meningiomas had higher VEGF expression and subsequently produce larger peritumoral edemas.4This is the first report of angiomatous transformation following GKRS with pathologic confirmation. Although the pathogenesis is not clear, this rare pathologic transformation may have a close relation to RN.Although bevacizumab has shown a good efficacy on RN, however, if bevacizumab is resistant, there are no available alternatives to treat RN. So, we suggest that debulking surgery could be an alternative treatment option to control the bevacizumab-refractory RN. Also, VEGF may not be an only contributing factor for peritumoral edema in RN.Conceptualization: Shin JungData curation: You-Sub Kim, Woo-Youl Jang, Tae-Young JungSupervisions: Shin Jung, Kyung-Sub MoonPathology: Kyung-Hwa LeeWriting: You-Sub Kim


Schwannomas are slow-growing, benign tumours normally originating from the schwann cells of the nerve sheath. Intraosseous schwannomas account for 0.175% of primary bone tumours and are extremely rare, especially outside the axial skeleton. Monoclonal gammopathy has been associated with soft tissue schwannomas but never with the intraosseous variety.A 55-year-old woman with a background of monoclonal gammopathy of undetermined significance (MGUS) presented with a 2-year history of right thigh pain. CT scan showed a well defined, lytic lesion with a thin peripheral rim of sclerosis in the midshaft of the femur. MRI displayed a hyperintense, well marginated and homogenous lesion. Definitive diagnosis was made based on the classical histopathological appearance of schwannoma. We managed our patient with local curettage and prophylactic cephalomedullary nailing based on her high mirel score.Intraosseous schwannomas are poorly understood but most commonly reported in middle-aged women. Radiologically, their differential diagnosis includes malignant bone tumours, solitary bone cysts, aneurysmal bone cysts and giant cell tumours. As a result, they are usually diagnosed incidentally on histology. Although malignant transformation is possible in soft tissue schwannomas, all intraosseous schwannomas reported to date have been benign.This case demonstrates the importance of suspecting intraosseous schwannoma as a differential diagnosis for lytic bone lesions to avoid the overtreatment of patients. We also highlight monoclonal gammopathy of undetermined significance as a potential risk factor for a poorly understood disease and make recommendations about the appropriate management of these lesions. As a reThe case report satisfies the SCARE criteria of reporting .29/L) following a viral illness. Further immunological assay testing measured high IgG levels and subsequent serum protein electrophoresis revealed two clonal bands, band 1 IgG 15.5 g/l and band 2 IgA kappa 5.2 g/l (including beta 2 band). These paraprotein levels have remained stable over 12 months. Her 24-hr urine light chain assay was normal. Assessment of plasma cell infiltration of the bone marrow by biopsy was deemed unnecessary at this time as our patient was diagnosed with IgG type monoclonal gammopathy of undetermined significance (MGUS) with a stable paraprotein level. However, CT skeletal survey was ordered as part of the work-up for her thigh pain and showed the lesion in the midshaft of the right femur  was also normal at this time.Her initial laboratory investigations showed: Total WCC 2.7, Neutrophils 0.9–1.2 × 10CT demonstrated evidence of a lytic lesion with surrounding sclerosis eroding the cortex of the femoral diaphysis . SubsequShe underwent simultaneous excision biopsy of the lesion and prophylactic cephalo-medullary nailing based on her Mirel score of 8. Her operation was successfully performed under consultant supervision and she was discharged day 4 post-operatively after receiving care from the inpatient physiotherapy department .Fig. 3 . Soft tiOur case demonstrates the classical radiologic findings in intraosseous schwannoma including a well-defined osteolytic lesion with sclerotic borders; a lobulated or trabeculated contour; cortical erosions; and absence of central calcification. It is important to note that this appearance can be difficult to differentiate from severe diagnosis such as malignancy or osteomyelitis . FurtherAs these tumours are cliniradiologically indistinguisable from their differential diagnoses and have an extremely low incidence they are typically diagnosed histopathologically. Microscopically, they have similar appearances to soft tissue schwannoma and demonstrate two types of cell arrangements, Antoni A and Antoni B. Type A tissue is composed of densely packed, spindle-shaped cells arranged in bundles and cords surrounded by an eosinophilic cytoplasm. The nuclei of these cells are often arranged in palisading rows, forming Verocay bodies. Type B tissue has loosely scattered, pleomorphic schwann cells with predominantly myxoid cytoplasm. They have thick-walled and hyalinised blood vessels. Microcystic areas with haemorrhage are common. Diffuse immunoreactivity for S100 protein is indicative of schwann cell origin. Negative staining for smooth muscle markers such as desmin and smooth muscle actin (SMA) are used to rule out histological differential diagnoses ,16. The Although malignant transformation is possible in soft tissue schwannomas, all intraosseous schwannomas reported to date have been benign . There a4Intraosseous schwannoma is an extremely rare diagnosis, especially in the long bones. It can be difficult to differentiate from other radiographically benign-looking, lytic bone lesions but is an important differential to consider in patients with chronic, insidious thigh pain. MGUS may represent a potential risk factor for intraosseous schwannoma. Finally, although malignant transformation has been reported in schwannomas of soft tissues, all intraosseous schwannomas reported to date have been benign, such that curettage or marginal excision remains the therapy of choice.This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.Ethical approval was not required and patient-identifying information was not presented in this report.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Timothy McAleese: Conceptualization, Data curation, Methodology, Project administration, Software, Writing - original draft.Kevin Clesham: Conceptualization and design, Project administration, Writing - review & editing.Darren Moloney: Conceptualization, Formal analysis, Investigation, Writing - review & editing.Andrew Hughes: Conceptualization, Administration, Validation, Visualization, Writing - review & editing.Nazia Faheem: Conceptualization, Administration, Resources, Software, Formal analysis, Writing - review & editing.Khalid Merghani: Supervision, Conceptualization, Validation, Writing - review and editing.All authors read and approved the final manuscript.This is not applicable due to the nature of this case report.Mr Khalid Merghani is a consultant orthopaedic surgeon and the senior author in this paper. As guarantor he is responsible for the work and/or the conduct of the study, had access to the data and controlled the decision to publish.Not commissioned, externally peer-reviewed.The authors have no conflicts of interest to declare.


Ameloblastoma is a rare tumor which develops from odontogenic epithelium and its remnants and it occurs in the jaws. Peripheral ameloblastomas are rare and benign extraooseous ameloblastomas which effects soft tissues. This case report declares a peripheral ameloblastoma which is a rare type of ameloblastoma.34 year old female patient referred with a complaint of a gingival growth at right lower premolar area. A firm and granular surfaced gingival growth with the color of pink and red and having 1.5x1 cm sizes was observed at the mentioned area. With an incision from lower right second incisor tooth to lower right second molar tooth a flap from bone was made and lesion was excised. After then specimen was submitted to histopathologic examination. After clinical, radiological and pathological examinations lesion was described as peripheral ameloblastoma.At the control examination after three months of excision there was no recurrence and patieant has no complaint.Although reccurens rate of peripheral ameloblastomas are low, long-term follow-ups are suggested Patient was informed about the importance of regular controls for early diagnosis of possible reccurenses and regular controls were made during one year after excision. Key words:Peripheral ameloblastoma, gingiva, gingival hyperplasia, gingival lesion, alveolar mucosa, extraosseous. Ameloblastoma is a rare tumor which develops from odontogenic epithelium and its remnants and it occurs in the jaws ,2. AccorPeripheral ameloblastomas are rare cases which have %1.3-10 ratio among all ameloblastomas  basoloid cell islands was observed at the loose connective tissue which shows proliferation to beneath of ceratinized stratified flat epithelium. And at the micro magnification (HEx200) basoloid cell islands which shows reverse palizades at their periphery were observed and pathologic diagnosiz was made as ameloblastoma Fig. . After cet al. reported only one case that located at two diffrent locations at the same time.7,8Peripheral ameloblastomas are generally common at one location. Only Hernandez  Peripheral ameloblastomas usually occur at lower jaw premolar area, followed by lower anterior and maxillar tuber areas. These cases can be seen at the ages raging from 9 to 92 and mean age rewiewed is 52.1. Peripheral ameloblastomas are more common for male patients then female patients (%65) ( Peripheral ameloblastomas can be drawn with lots of clinical situations and they can be noticed incidentally at routine dental examination . In this Periphreal ameloblastomas are painless, sessile, firm and granular or pebbly like surfaced exophytic growths and their clinical appearance can be interefered with many different clinical situations like pyogenic granuloma, giant cell granuloma, inflammatory fibrouse hyperplasia related to prosthesis and basal cell carsinoma ,9,12. FoGenerally Peripheral ameloblastomas do not penetrate to bone structure and lesions do not affect the cortical bone which they have been located on . In our  Impacted tooth relation is a common situation for unicystic ameloblastomas according to literature but only one case that reporting peripheral ameloblastoma with an impacted tooth was found at the literature search ,7. In th


Dear Editor,A 12-year-old girl, who had no co-morbidities, sought dermatological care due to the emergence of brownish spots that looked like “dirt”, which were spread through the body and had progressively evolved in the previous two years. This condition caused great social impact to the patient, given that she suffered from discrimination at school. She had used ketoconazole cream as prescribed by a physician of the Basic Health Unit; however, there was no clinical response. The patient noticed that the lesions became discreetly clearer after intense rubbing with gauze, but without improvement after washing with soap and water.The dermatological examination indicated that the patient had hyperkeratotic brownish plaques with a dotted pattern, affecting the neck, back, abdomen, and, in a less evident manner, the lower limbs . After s“Terra firma-forme” is a Latin expression, which means “solid earth”, also known as Duncan's dirty dermatosis. It is a benign and idiopathic skin disorder that was described in 1987.3The pathogenesis is uncertain; however, it is attributed to disturbance in the maturation of keratinocytes, leading to the compaction of these cells, associated with melanin, sebum, and microorganisms in the epidermis.This dermatosis is characterized by slightly elevated, brownish or blackened, hyperkeratotic, and asymptomatic plaques and papules. The lesions are typically located in the neck, face, and trunk, bilaterally or unilaterally. It affects patients with adequate hygiene habits, and the lesions are not removed by washing with soap and water.2When the presence of the condition is suspected, dermatoscopy helps by detecting brownish polygonal plates arranged in a mosaic pattern.Differential diagnoses are made with hyperpigmented lesions, including confluent and reticulated papillomatosis of Gougerot and Carteaud, which has some similar characteristics and is considered a superficial variant of that dermatosis. It is possible to differentiate them by removing the lesions with alcohol in terra firma-forme dermatosis, in addition to considering other clinical findings, such as for example, acanthosis nigricans, which is a skin disorder usually associated with metabolic disorders and dermatitis neglecta, easily washed with soap and water,2The treatment consists of the application of 70% isopropyl alcohol; however, the lesions may recur.The patients and families should be advised to clean the skin at home,It is important to recognize this dermatosis in order to avoid incompatible diagnostic and therapeutic procedures, as well as to reassure adolescents and family members, thus reducing social impact on patients’ lives.None declared.Bruna Anjos Badaró: Conception and planning of the study; elaboration and writing of the manuscript; obtaining, analysis, and interpretation of the data; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.Lucia Martins Diniz: Approval of the final version of the manuscript; conception and planning of the study; effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature; critical review of the manuscript.Paulo Sergio Emerich Nogueira: Intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the literature.None declared.


The gastric volvulus is a rare condition in which the stomach, or part of it, rotates on its axis, for over 180°, constituting a surgical emergency. Even more rare is gastro-gastric intussusception. A delay in their diagnosis and treatment can have fatal consequencesAn 82-year-old woman was admitted to the Surgery Unit with a two-day history of abdominal pain associated at first with coffee vomiting and, subsequently, with unproductive retching and oligoanuria. Physical examination showed severe dehydration, fever, at the abdominal level, palpation caused a marked tenderness of all quadrants, with signs of peritonism.Laboratory test showed showed neutrophilic hyperleukocytosis and high C reactive protein level. Abdominal computed tomography revealed an acute intrathoracic gastric volvulus and a gastrogastric intussuception. The patient was submitted to exploratory laparotomy, subtotal gastrectomy with Roux en Y anastomosis and simple plastic of the esophageal hiatus. At the end of the surgery, however, the patient died of your septic shock.The traditional treatment for a patient with acute gastric volvulus is an immediate surgical intervention to derotate the stomach and prevent vascular insufficiency.In the presence of necrosis or gastric perforation, resection should be performed.The few cases of gastrogastric intussusception described in the literature have been treated with sub-total gastrectomy and gastro-jejunal anastomosis. Any delay in diagnosis and treatment can prove fatal.Intrathoracic Gastric Volvulus and, even more, retrograde gastrointestinal intussusception are very rare pathologies, difficult to diagnose. Temperature: 37.8 °C. BP: 90/50 mmHG. HR: 140 bpm. GCS: 8. Marked tenderness at palpation of the all abdominal quadrants, with signs of peritonism. Anuric patient.Blood tests showed neutrophilic hyperleukocytosis , an increase in creatinine (1.47 mg/dl), CRP (139.7 mg/l) and LDH (504 IU/l) values.CT of the thorax and abdomen with contrast showed a voluminous sliding hiatal hernia with herniation of nearly the entire stomach in the retrocardiac seat. A possible diagnosis of acute gastric volvulus was made. The presence of retrograde gastro-gastric invagination at the level of the antrum was also reported.Considering severe sepsis and rapid declin of the general conditions, after rapid resuscitation with infusion of liquids, electrolytes and broad-spectrum antibiotic therapy, two hours after admission to the emergency department the patient was subjected to exploratory laparotomy which revealed an abundant intra-abdominal bilious effusion and voluminous hiatal hernia with complete herniation in the thorax of the stomach, twisted on its long axis and with ischemic necrosis of the antrum , linked to the laxity of the perigastric ligaments, and secondary (more common) to gastric or diaphragmatic anomalies.Volvulus can be classified as Conventionally, gastric volvulus is imagined as an intra-abdominal condition. However, although uncommon, an intrathoracic variant is observed and is linked to a herniation of the stomach in the chest through a diaphragmatic defect  .The Upside-down stomach (UDS) is the rarest form of hiatal hernia (<5%), characterized by the herniation of the entire stomach, or most of it, in the posterior mediastinum and would predispose to intrathoracic gastric volvulus .As well as hiatal hernias, UDS can give a wide variety of symptoms such as retrosternal pain, heartburn, post-prandial fullness, dysphagia, nausea, vomiting, anemia and mass-effect symptoms ,23.organoaxial (59%), when the stomach rotates around the pylorus and the gastroesophageal junction and mesenteroaxial (29%), when the stomach rotates along the longitudinal line parallel to the small gastric curve, or mixed (12%) [According to Singleton, gastric volvulus can be classified as or mixed 2% [1,19,ed (12%) .The gastric volvulus, clinically, can present itself as an acute abdomen or as a chronic intermittent recurrent pathology.Acute gastric volvulus presents itself with a sudden and violent pain in the upper left quadrant or at the base of the left hemithorax. Other symptoms may be wheezing, unproductive retching, dehydration and prerenal insufficiency ,22.The most common complications of acute gastric volvulus are incarceration, strangulation and perforation.The mortality rate varies between 30% and 50% and it increases to 60% if strangulation and ischemia occur .Because of its rarity, a doctor with no deep experience, could relate this pathology to other non-surgical abdominal diseases or even to an acute coronary syndrome.Hence an accurate anamnesis, a detailed clinical examination and a careful interpretation of the radiological images are important to face a patient who has vomit and high abdominal pain .In 1904, Borchardt described the triad of acute epigastric pain, unproductive retching and the difficulty or impossibility of positioning a nasogastric tube.This triad is found in 70% of patients with acute organoaxial volvulus.Chest x-ray highlights the presence of abdominal viscera that have risen in the chest ,23.Other tests, often not performed in acute, are the barium contrast studium and digestive endoscopy .The Chest and Abdomen CT allows to have an immediate diagnosis, to know the extent of the herniation, to put the right surgical indication, facilitating so preoperative planning ,3,4,23.The traditional treatment is an immediate surgical intervention to derotate the stomach and to prevent vascular insufficiency.In the presence of necrosis or gastric perforation, resection should be performed.At the same time, a reduction of the hernia and repair of the diaphragmatic defect should be made.The stomach is then fixed to the anterior abdominal wall by simple suturing or by placing a gastrostomy tube.Open or laparoscopic surgery can be superimposed in terms of results. However, in urgency, the open treatment is often preferred.In elderly patients and high surgical risk ones, an attempt at medical management may be helpful.The simple positioning of a nasogastric tube or, an endoscopic decompression with PEG positioning can be decisive .A much rarer clinical condition is gastrogastric intussusception.In adults, it only occurs 5% of cases and less than 10% of these cases affect the gastro-duodenal region .Patients with this condition often have nonspecific symptoms, typically characterized by epigastric pain and vomiting .If untreated, intussusception can cause ischemia of the invaginated bowel wall and consequent perforation with peritonitis.The typical tomographic sign of intussusception is the target-shaped image .The evidence from previous studies also indicates that a soft tissue growth, malignant or benign, is a typical concurrent finding and lead point. However, cases of gastrogastric intussusception with alternative pathophysiological mechanisms have been reported .The presence of hiatal hernia, the laxity of the gastric ligaments, the increased intra-abdominal pressure and previous diaphragmatic surgery are considered predisposing factors .In 2017, Behrooz, described the first case of gastrogastric intussusception from vascular congestion, assuring that portal hypertension, ascites, and the presence of a hiatal hernia triggered the invagination in the absence of an underlying neoplasm .Hiatal hernia, in the absence of a tumor that acts as an invaginating head, is a well-known risk factor of esophagogastric intussusceptions as reported by Ghahremani and El-Hajj  Table 1Table 1.TThis pathophysiological mechanism is what we postulate may have occurred in the patient of the clinical case reported by us, i.e. the vascular congestion of the gastric wall, linked to the gastric volvulus, in association with the hiatal hernia and the laxity of the ligaments, behaved as a trigger intussusception.The treatment of adult invaginations is generally surgical and, given the high incidence of underlying malignancies, consists in the resection of the intestinal segment involved and anastomosis. The few cases of gastrogastric intussusception described in the literature, have been treated with sub-total gastrectomy and gastro-jejunal anastomosis.In the reported case, the patient had an intrathoracic organoaxial gastric volvulus associated with retrograde gastrogatric intussusception with ischemic necrosis and perforation at the level of the antrum. The chronicity and the intermittence of the symptomatology have never prompted the patient to undergo a previous diagnostic investigations. Only in the face of the presence of violent epigastric and thoracic pain, unproductive retching, dehydration and decay of the general status, she resorted, with considerable delay, to medical treatment, but the gastric volvulus and invagination, which occurred in the hours prior to hospitalization, contributed to the necrosis and the consequent perforation with peritonitis, mediastinitis and a septic shock resulting fatal for her. Hence the importance, in the face of a patient with violent abdominal pain, with unproductive vomiting, not to neglect the diagnostic hypothesis of an acute gastric volvulus, because any delay in diagnosis and treatment can prove fatal.4Intrathoracic Gastric Volvulus and, even more, retrograde gastrointestinal intussusception are very rare pathologies. A delay in their diagnosis and treatment can have fatal consequences such as gastric ischemia and perforation. In consideration of these severe complications, we underline the importance of a correct diagnostic framework with a detailed anamnesis, a meticulous physical examination and a careful analysis of the radiological tests when you are faced with a patient who presents unproductive vomiting and sudden epigastric pain.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.Giovambattista Caruso: Operated on the patient, drafting the manuscript.Sebastiano Caramma: Operated on patient.Domenico Zerbo: Literature search.Angelo Zappalà: Literature search and revising the manuscript.Giuseppe Evola: Revising the manuscript.Carlo Reina: Drafting the manuscript.Giuseppe Angelo Reina: Clinical supervision and consultation.This case report does not require registration as a research study.The guarantor for this case report is Giovambattista Caruso.Not commissioned, externally peer-reviewed.


Chronic intradiploic organizing hematoma of the skull is a rare lesion that usually presents as a progressively growing mass after head trauma, thus making it difficult to diagnose. To date, only nine cases that have been histopathologically confirmed as organizing hematoma of the skull have been reported in the literature. Herein, we describe a case of a chronic organizing hematoma involving the right parietal bone, presenting as a slowly growing mass in a 54-year-old man. The lesion was also visualized on magnetic resonance imaging (MRI) with a zero echo time sequence. In this case report, we emphasize that chronic intradiploic organizing hematoma should be considered in the differential diagnosis of a palpable scalp mass. We also highlight the importance of meticulous radiological review in the context of appropriate clinical suspicion and the usefulness of the zero TE sequence in evaluating calvarial lesions. Chronic intradiploic organizing hematoma (CIOH) of the skull is a rare condition that usually presents as a progressively growing scalp mass ,2. Altho3; receiver bandwidth, ±31.25 kHz; and, total number of scans, 3. MRI demonstrated an expansile mass with inhomogeneous signal intensity, showing predominantly hypointense on T1-weighted images and hyperintense on T2-weighted images. The lesion showed multifocal serpentine enhancing foci on contrast enhanced T1-weighted images. However, there was no combined abnormality in adjacent brain parenchyma , which uses a nonselective radiofrequency excitation pulse and a three-dimensional (3D) radial center-out k-space trajectory to separate bone from soft tissues and achieve selective bone images . It geneThe differential diagnosis for CIOH should be considered for other calvarial lesions, as follows: 1) benign neoplastic and nonneoplastic lesions; aneurysmal bone cyst, giant cell tumor, giant cell reparative granuloma, fibrous dysplasia, eosinophilic granuloma, intradiploic epidermoid and dermoid cyst, cavernous hemangioma, circumscribed osteomyelitis, tuberculous granulomas, and hemophilic pseudotumor  benign n,16, and An accurate diagnosis can be established by a histopathological examination, because it is difficult to differentiate CIOH from other diseases, such as giant cell tumors, aneurysmal bone cysts, giant cell repetitive granulomas, cavernous hemangioma, or fibrous dysplasia, which can be radiologically or histologically similar to CIOH ,11,15. CHerein, we report a case of CIOH in the right parietal bone presenting as a slowly growing mass with clinical, radiological, and histopathological findings. The diagnosis of CIOH can be challenging in clinical practice because of its rarity and nonspecific clinical and radiological features. Therefore, clinical suspicion and awareness are important for accurate diagnosis and management for patients with space-occupying lesions of the skull vault. In addition, a ZTE sequence is useful for evaluating calvarial lesions in a daily practice, because it can easily identify the bony lesions as similar to CT images with its inherent benefit of not generating radiation.


Triplicate ureter is a rare congenital anomaly of the upper urinary tract.In this case we present a triplicate ureter with ipsilateral ureterocele in a 9 years old boy. After literature review we found out this presentation very rare.Ureteral triplication classified by Smith into 4 subtypes, and usually associated with other congenital abnormality, most common one is contralateral ureteric duplication. Usually this anomaly is asymptomatic.We present a type 3 ureteral triplication.Although triplicate ureter is a rare congenital anomaly, we must keep it in our differential diagnosis of unexplained urinary symptoms. Physical examination showed stable vital signs, body weight of 29 kg, and no abdominal masses.Urine analysis and kidney function test were unremarkable.Radiological investigations started with Ultrasonography for the urinary tract  which shDue to the above investigations, cystoscopy was done and showed normal urinary bladder apart from large ureterocele, which was large enough to be managed endoscopically. Then decision was made to perform left partial nephroureterectomy for the upper moiety  and to excise the ureterocele. After the operation, the patient did well and was discharged home after 15 days without any complications. Histopathology came back as left ureter excision with chronic inflammation and left partial nephrectomy with acute and chronic pyelonephritis changes.A Follow-up IVU one-month post operation showed normal nephrogram bilaterally with incomplete duplication of left kidney without obstruction .Fig. 4PoWritten and oral consent for use of the data and photos for research project and educational purposes was obtained from the parents.3Ureteral triplication is a rare congenital anomaly of upper urinary tract . UreteraTriple ureters may be responsible for recurrent urinary tract infections, incontinence, enuresis or pain caused by ureterocele but most of the time this anomaly is asymptomatic and detectedincidentally during the investigation of other conditions, and this can explain its late presentation . in chilMost cases of ureteral duplication conform to the Weigert-Meyer law , which s4Ureteral triplication is a very rare congenital anomaly, and because of the paucity of presenting symptoms, this can delay its diagnosis. Our recommendation is to keep this anomaly in the differential diagnosis of recurrent UTI, incontinence and recurrent urolithiasis.No conflicts of interest.No sources of funding for my research.This research did not need ethical approval.Fully informed written consent was signed from the parents of the patient.MOHAMMAD. AL-ZUBI: study design, data collection, data analysis and writing the paper.ANAS AL FAQIEH: data collection, and writing the paper.OROUB ALTAMIMI: data analysis and writing the paper.SOHA ALBEITAWI: study design, data collection.NA.MOHAMMAD. AL-ZUBI.Not commissioned, externally peer-reviewed.


Post-herpetic abdominal pseudohernia is a neurologic complication of herpes zoster resulting from paresis of the abdominal wall muscles ipsilateral to the eruption. This poorly known condition may raise suspicion for true abdominal wall hernia or other concerning etiologies, resulting in extensive work-up and imaging. Post-herpetic abdominal pseudohernia is a relatively benign condition, which resolves spontaneously in the majority of cases. Therefore, it is important for the clinician to be aware of this complication in order to avoid unnecessary imaging or excessive management, which may increase the cost of care and burden to the patient. Herpes zoster (HZ) is a clinical syndrome resulting from reactivation of the varicella-zoster virus (VZV). Initial infection with VZV generally occurs in childhood, resulting in varicella infection. Following resolution of the primary infection, VZV remains dormant in the dorsal root ganglia, autonomic ganglia, and cranial nerve ganglia. Host-specific immunity against VZV may wane with aging, stress, or state of immunosuppression, resulting in HZ, which is a spontaneous reactivation of the VZV. HZ is generally characterized by a vesicular rash distributed in a unilateral dermatomal fashion ,2.Segmental zoster paresis, or muscle zoster, is a neurologic complication of HZ, which results in a lower motor neuron weakness of the associated dermatome -3. AboutA 57-year-old man with history of diabetes mellitus and hypertension was diagnosed with HZ by his primary care provider. The patient was treated with valacyclovir. After one month, the initial dermatologic condition had resolved. However, the patient reported pain, skin hyperpigmentation, and an area of outpouching on the right abdomen and flank in the region of prior HZ eruption Figure . His priPHAP is a rare complication of HZ associated with segmental zoster paresis. Patients with this condition may exhibit bulging asymmetry of the abdomen, a reducible mass of the abdomen, or diminished or absent abdominal reflexes in the context of recent HZ -6.PHAPs are more commonly found in the T10-T12 dermatome, in males, and those in their seventh decade. It usually presents within the first three to four weeks following HZ eruption but may present up to two months following diagnosis. On rare occasions the pseudohernia may present prior to eruption of HZ or in the absence of any HZ eruption [Diagnosis of PHAP may be made clinically by assessment of presentation and history ,5. Some Unlike true abdominal hernias, PHAPs do not require surgery. In fact, up to 80% of all patients with PHAP will show spontaneous recovery by one year, with a median recovery time of about five months . For theWe report the uncommon case of an adult man presenting with a painful abdominal outpouching. Although rare, clinicians should consider the diagnosis of PHAP in a patient with this presentation because of its noninvasive work-up and benign disease course. Evaluation with history and physical exam are sufficient to make a clinical diagnosis of PHAP. Extensive imaging and testing are unnecessary, resulting in increased health-care costs and increased treatment burden on the patient.


Endovascular procedures with liquid embolic agents such as ethylene-vinyl alcohol (EVOH) copolymers are indicated before surgical treatment of cervical paraganglioma. Consequently, these agents are now available as low viscosity formulations, one of which is Squid 12, which are demonstrating superior vascular penetration. Cases of facial paralysis secondary to embolization of cervical vascular lesions with classic embolic agents have been reported in the English literature, however, this complication has not been described with new generation options such as Squid 12.We describe the case of a 43-year-old patient with a left neck carotid paraganglioma. Embolization was performed under general anaesthesia before surgical excision. In the immediate postoperative period, the patient developed total left facial palsy. Since the imaging tests (Computed Tomography (CT) and Magnetic Resonance Imaging (MRI)) and neurological examination showed no involvement of additional cranial nerves (CN), we hypothesise that the main cause of this complication is ischemia of the vasa nervorum of CN VII secondary to embolization. Almost six months later, the patient continues to present total facial paralysis , and palsy of the left CN X and XII as a complication secondary to surgical resection of the paraganglioma.This case is relevant since it is the first clinical case of permanent facial paralysis secondary to embolization with Squid 12. The choice of embolic agent for embolization of vascular head and neck tumours is challenging since the vascular patterns of tumours that are optimal candidates for this agent have not yet been defined . HoweverSquid®  is a copolymer of EVOH dissolved in a DMSO (dimethyl sulfoxide) solution with suspended micronized Tantalum powder for radio-opacity. While Squid 18 has similar properties to Onyx®, Squid 12 is innovative in terms of viscosity. A less viscous product, Squid 12 is more fluid than other agents. Studies show that this property enhances penetration of the tumoral parenchyma and diffusion of the embolic agent in parts of the tumour further from the puncture site –5. AlthoWe describe the case of a 43-year-old woman diagnosed with cervical paraganglioma as a casual finding in computed tomography. Her medical history included sinusopathy complicated by sinus thrombosis and bilateral optic neuropathy with no clinical signs of effect on the facial or other cranial nerves. A laryngeal examination by the otolaryngologist two months earlier was normal, revealing no abnormal findings. The CT image shows a 32x26x41mm carotid paraganglioma  with normal sensitivity. No other loss of neurological function was detected.The stroke code was activated in response to the clinical examination and vascular procedure performed. Computed tomography(CT)perfusion, and an MR angiography of the supra-aortic vessels Figs.  and 3 shThe surgeon completed the resection of the paraganglioma three days after the embolization, but could not avoid damaging the vagus nerve (CN X), resulting in recurrent left laryngeal nerve paralysis. The surgery also affected CN IX, XI, and XII, eliminated the gag reflex, caused hypoesthesia of the left side of the face and pharynx, velopalatine asymmetry and motor dysfunction of the tongue. Five days after the operation, the patient still presented Grade V left-side facial paralysis and the left side of the vocal chords were paralysed with signs of acute laryngeal compensation. However, the motor tone of the tongue had recovered completely. Episodes of aspiration were observed in the volume-viscosity swallow test (V-VST). Rehabilitation and inserting a nasogastric tube as an initial measure, although finally, because of the deteriorating situation, it was decided that our Dysphagia Unit should perform a percutaneous gastrostomy.The patient continues to suffer from facial paralysis, but to a lesser degree on the House-Brackmann scale (IV): lack of lip seal, facial asymmetry at rest and poor facial movements, without synkinesis.MRI performed in the immediate postoperative period Fig. , showed Facial palsy is a rare complication in patients undergoing arterial embolization. There is a theoretical estimated 6% risk of harbouring an anatomical vascular pattern that may expose the facial nerve to greater risk during embolisation , 3–5. FuSudden onset facial palsy subsequent to an endovascular procedure should be treated as a major complication. All patients should be apprised of this risk before undergoing this type of procedure because of its psychological and social impact. Squid 12 is considered a safe liquid embolic agent for this type of procedure.We have described an initial case of permanent facial paralysis after the use of Squid 12 when embolising vascular tumours of the head and neck. We believe that due to the complex vascular anatomy of the area, it is likely that small vasa nervorum were occluded when diffusing the embolizing agent, since there were no difficulties during or after the technique. This vasa nervorum occlusion could have happened on the one hand, in the intrapetrosal course from the Fallopian canal, where the facial nerve is supplied by the petrous branch of the middle meningeal artery and by the stylomastoid branch of the occipital artery, which sometimes originates directly from the external carotid; and on the other hand, in the extrapetrosal portion of the facial nerve, that first receives irrigation from branches of the occipital and posterior auricular arteries at the base of the skull, and subsequently at the level of the parotid gland and outside of it, where vessels come from the superficial temporal for the temporofacial trunk and from the facial for the cervicofacial trunk .If we compare the Squid 12 with the Onyx, are very similar in terms of composition. However, that lower viscosity described in the Squid 12 literature, can favor penetration in all vascular pedicles and their more distal branches, affecting irrigation vascular of the facial nerve in this case. The persistence of the nerve injury could also be favored by postoperative changes on MRI Fig. .This case shows that cranial nerve palsy is a possible complication of the procedure even with the new embolic agents such as Squid 12, therefore an accurate risk-benefit assessment is always necessary before the treatment. In our opinion, further studies are required to assess the risk-benefit ratio of preoperative embolization with new liquid embolic agents in asymptomatic vascular head and neck tumours.


Angioma serpiginosum (AS) is an unusual vascular nevoid disorder that is more common in women. It begins in childhood and stabilizes in adulthood. Dermoscopy of angioma serpiginosum reveals typical small, sharply demarcated, round-to-oval red lagoons, corresponding histopathologically to dilated, thin-walled capillaries found in the superficial dermis.A 29-year-old man presented with a 10-year history of asymptomatic red lesions over the left side of abdomen. There was no associated history of itching or trauma. General and systemic examination was normal. Cutaneous examination revealed multiple minute, pinpoint, grouped, bright red, nonblanchable macules and irregular patches distributed in a patchy blaschkoid pattern over the left side of abdomen .Dermoscopy of the erythematous lesions using a 3Gen DermLite II Hybrid M dermatoscope, polarized mode, ×10 magnification, revealed multiple oval-to-round, well-demarcated, red-colored lagoons on a slightly erythematous background . LesionsA punch biopsy of 4 mm taken from erythematous lesions revealed the presence of mild hyalinization and increased proliferating small capillaries with a normal endothelial lining in the papillary and superficial reticular dermis . There wAS is an uncommon benign, vascular nevoid disorder first described by Hutchinson in 1889 .AS typically begins in childhood and has preponderance in women. It is characterized by multiple small, asymptomatic, nonpalpable, deep red punctate macules organized in serpiginous and gyrate patterns. The lesions are predominantly distributed on the lower extremities and buttocks and usually appear unilateral, but bilateral asymmetric involvement or linear morphology has been reported, though rarely. Segmental patterns of lesions, as seen in our case, may reflect cutaneous mosaicism.Differential diagnosis includes pigmented purpuric dermatosis, unilateral nevoid telangiectasia, port-wine stain, and angiokeratoma corporis diffusum. Apart from the clinical presentation, confirmation of diagnosis is made by histopathology. A characteristic histopathological feature of AS is the vascular proliferation located at the papillary dermis, which is composed of dilated capillaries . The lacClinical findings alone or a noncontributory biopsy report can be misleading, so dermoscopic features help to distinguish AS from other vascular-related diseases. The characteristic dermoscopic findings reported in the literature include erythematous, well-demarcated, round-to-oval dots and lagoons ,2. The rAS is a benign disease and commonly asymptomatic requiring no treatment, although it raises cosmetic issues. There are several reports describing intense pulsed light or pulsed dye laser as treatment, with a clearance rate ranging from 25% to 75% [


Cryptorchidism is defined as the absence of one or both testicles from the scrotum, which is considered one of the most common birth defects of male genitalia. Mostly the undescended testes are in the inguinal region, while the intra-abdominal location accounts for only 10 % of the cases. The potential risk of malignant transformation in an undescended testis is approximately 2.5–8 times higher than in scrotal position. Torsion of the cryptorchid testis is a very rare cause of acute abdominal pain with few cases published in literature.A 44-year-old male patient presented to the emergency room with acute abdominal pain. General examination revealed relevant tachycardia. Upon abdominal examination there was tenderness and rebound tenderness all over the abdomen, more evident in the lower abdomen. Interestingly, routine examination of the inguino-scrotal region revealed empty right hemi-scrotum despite the patient not being aware. Routine blood tests showed marked anemia Hb: 7.4 g/dl. Urgent pelvi-abdominal U/S showed moderate free intra-abdominal collection in addition to a pelvic mass. CT of the abdomen showed a well-defined heterogenous hypodense mass measuring about 8.5 × 5.5 cm in the pelvis. Urgent exploratory laparotomy was done, and a mass was found to be torted and ruptured. Excision was done and histo-pathological examination revealed testicular mixed germ cell tumor.Undescended testis is associated with an increased risk of infertility, testicular cancer and torsion. In any male with undescended testis and intra-abdominal mass; the risk of intra-abdominal testicular tumor should be considered. The estThe potential risk of malignant transformation in an undescended testis is approximately 2.5–8 times higher than in normal population . MoreoveHere we present a case of intra-abdominal testicular mass complicated by torsion, rupture and internal hemorrhage mimicking acute abdomen. This work has been reported in line with the SCARE criteria .2A 44-year-old male patient who is not known to be diabetic or hypertensive was presented to the emergency room with sudden diffuse abdominal pain for 8 h with no history of trauma. He experienced intermittent attacks of vague lower abdominal pain lasting for few hours with spontaneous relief over the last 2 days. The patient underwent open appendectomy 2 years ago; the patient is married with 3 offspring’s, the youngest of which is 12 years old.General examination revealed relevant tachycardia (pulse116/min), normal blood pressure was 118/76 mmHg and the respiratory rate was 14 breaths / min. Oxygen saturation on room air was 99 %, and he was afebrile.Upon abdominal examination there was tenderness and rebound tenderness all over the abdomen, more evident in the lower abdomen. Interestingly, routine examination of the inguino-scrotal region revealed empty right hemi-scrotum despite the patient not being aware about this. Routine blood tests showed high white blood cells count = 20,000 10*3 /mm and marked anemia Hb: 7.4 g/dl.Urgent pelvi-abdominal U/S showed moderate free intra-abdominal collection in addition to a pelvic mass. Computed tomography of the abdomen  showed aA decision was made to proceed for urgent exploratory laparotomy owing to the drop of hemoglobin level. Initial resuscitation was performed and through a midline incision we found a rounded gangrenous perforated mass in the pelvis, attached to the lateral pelvic wall on the right side by a narrow band containing blood vessels and cord-like structure, as shown in  & 3 .FigThis mass was found to be torted around its band about 180 degrees counterclockwise. The gangrenous mass was excised through ligation of its vascularized pedicle flush with the lateral pelvic wall, formal abdominal exploration was done, and no evidence of suspicious lymph nodes or other masses was found. The specimen was sent for histopathological examination.Pathological and Histological examination confirmed that the mass was a necrotic hemorrhagic testicular tissue with wide areas of infarctions. Unfortunately, the pathology revealed testicular mixed germ cell tumor .Fig. 4MiThe patient had an uneventful postoperative course and was discharged on the fifth hospital day to complete his course of treatment with the oncology department in our institute.3Malignant transformation in an undescended testis usually peaks at the third or fourth decade of life, where pure seminoma represents more than 90 % of the histopathological features of undescended testicular tumors in adulthood . Sub-ferThe Nordic group made a consensus that orchidopexy must be performed prior to one year of age for maximum preservation of potential future fertility. If the condition is diagnosed later in life, surgery should be performed as early as possible . HoweverThere are many studies linking cryptorchidism to cancer. However, there are no long series of intra-abdominal testicular tumors, and most of the cases are in the form of case reports.Abu-Zaid et al. reported a 25-year-old male with left undescended testicle presenting with a rapidly growing mass in the left inguinal region. Tru-cut biopsy revealed mixed germ cell tumor . Sert etIn our case, the right testis was intra-abdominal; the patient was married with 3 children and did not seek medical advice before, about having only one testicle. Our decision to go for urgent exploration was due to the vital instability of the patient owing to the ongoing internal bleeding, which is a very rare presentation.Regarding the pathology in our case, it is also rare to encounter mixed germ cell testicular tumor as most cases reported in literature were pure seminoma. To the best of our knowledge this is the third reported case of intra-abdominal mixed germ cell tumor on top of a neglected undescended testis.4Undescended testis is associated with an increased risk of infertility, testicular cancer and torsion; therefore, it is mandatory to increase awareness of this condition among the primary care physicians. The scrotal examination is an integral part of the abdominal examination.In any male with undescended testis and intra-abdominal mass; the risk of intra-abdominal testicular tumor should be considered.None.No Funding.Ethical approval obtained from Cairo University Ethical Committee.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.Ahmed Ghobashy: Main operator – reviewing the manuscript.Doaa Hasan: Data curation.Ahmed AbdElsalam: Conceptualization - Critical revision of the manuscript.Aboubakr Ahmed: Data curation - Drafting the manuscript.Ahmed Arafat: Drafting manuscript.Mahmoud Tarek: Data collection.Moutaz Ragab: Drafting the manuscript.Not applicable.Moutaz Ragab.Not commissioned, externally peer-reviewed.


Orbitocranial penetrating injury (OPI) with multiple vascular invasions is a rare occurrence. To our knowledge, experience with its clinical treatment is rather limited, especially for infants. This case report describes an infant who fell from a 0.5 m high bed and landed on a toy with a keen-edged plastic rod. The fractured end of the rod was noted at the medial aspect of the left eyelid, and she was experiencing impaired consciousness. Computed tomography showed that the foreign body penetrated the cavernous sinus with internal carotid artery involvement, and compressed the transverse sinus through the cerebellum. Emergency surgery was performed with temporal occlusion of the left common carotid artery. The rod was removed from the orbital side, and bleeding from cavernous sinus region was effectively controlled under direct inspection via a sub-temporal approach. The patient was successfully treated and recovered consciousness after 17 days. This is the first report of successful management of OPI combined with multiple vascular injury in an infant. Herein, we highlight the anatomical imaging features of the injuries and also the individualized strategy concerning vascular invasion. Orbitocranial penetrating injury (OPI) refers to trans-orbital penetrating brain injury (PBI). PBI is significantly less prevalent than closed traumatic brain injury (TBI), with high mortality and morbidity rates –4. The cA 1-year 3-month-old girl was admitted to our hospital on August 29, 2019, 3 h after sustaining an OPI where a plastic rod pierced her orbital cavity via the lower left eyelid and penetrated the brain. The patient had no previous medical, family, or neurological disease history. Physical examination revealed that the patient was experiencing impaired consciousness, with a Glasgow Coma Scale score of 9. There was neither bleeding in the nasal cavity nor in the auditory canal. While the right pupil was 2 mm and sensitive to light, the left pupil was dilated and did not react to light. Movement in the right eye was normal; however, the left eye did not move. The patient demonstrated soft neck, and negative Kernig's and Brudzinski's signs. The right limbs were unresponsive to pain and the left limbs exhibited an avoidance reaction.X-ray revealed an intracranial FB of ~130 mm in length. Fortunately, there was no sign of fracture. Computed tomography (CT) indicated that the FB had penetrated the medial, inferior portion of the eyeball; superior orbital fissure; parasellar region; circular cistern; and left cerebellar hemisphere. A hematoma was located in the cerebellar hemisphere and the cerebellar vermis, with edematous surrounding brain tissue and high density of the tentorium cerebelli. Three-dimensional CT reconstruction of the skull confirmed that the orbital wall was intact, with no sign of fracture or bone destruction. CT arteriography (CTA) revealed that the FB was close to the inner wall of the cavernous sinus segment of the left ICA. CT venography (CTV) showed that the tip of the FB was compressing the left transverse sinus . DigitalThe patient received emergency surgery on August 9, 2019. We first implanted an intracranial pressure (ICP) monitoring probe in the frontal horn of the right ventricle . A straight incision was then made in the neck to fully expose the left common carotid artery (CCA) for temporary occlusion. Subsequently, the left cavernous sinus and part of the FB were exposed via a subtemporal approach. The FB was determined to have perforated the left cavernous sinus but had only damaged the ICA in a limited manner. To be cautious, we temporarily clipped the left CCA and then removed the rod from the side of the orbital socket. Because the ICA had not ruptured, bleeding from the cavernous sinus was soon staunched via electrocoagulation and compression. Fourteen hours after the first surgery, the ICP fluctuated around 25 mmHg, and a further CT scan revealed increased hemorrhage in the cerebellum. Therefore, we conducted a second surgery for cerebellar hematoma evacuation and removed ~5 mL of epidural hematoma from the left occipital region and ~14 mL of hematoma from the left cerebellar hemisphere. Postoperative CT demonstrated changes following hematoma evacuation and complete FB removal. The ICP probe maintained proper drainage and was correctly positioned in the lateral ventricle .The FB was completely removed . PostopeOPI is a subset of PBI, which is the most life-threatening form of TBI and can be classified into two types; high-energy and low-energy , 8, 9. IImaging plays a significant role in the choice of treatment strategy for OPI. X-ray is typically used for preliminary assessment and localization of intracranial FB. However, CT examination is the gold standard for the diagnosis of TBI and can be applied in OPI of different FB materials . MRI is There is currently no standardized OPI guideline. However, treatment principles coincide with other PBI subtypes, including complete FB removal, local debridement, neurovascular decompression, hemostasis, and dural repair . Early cOPI outcome is generally good in absence of direct injury to the brainstem or laceration of major vessels . Quite aOPI poses tough challenges to neurosurgeons worldwide due to the anatomical complexity of the trans-orbital pathway. For diagnosis, clinicians have to simultaneously consider FBs, and injury to the eyes, orbit, CNs, vessels, FBs, and brain tissues. This implies a greater requirement of diagnostic techniques, including high-resolution CT, MRI, and DSA , 32. ForIn this report, we highlight the importance of assessment based on CT reconstruction, CT angiography, and DSA, which are crucial for reference in OPI complicated with vascular injuries. The key part of an OPI surgery is decompression and removal of FBs, while preserving the normal tissue as much as possible. As an individualized strategy, temporary vessel occlusion is a choice when there is the possibility of lethal vascular damage. Infant cases put forward significant demand for perioperative management, particularly the control of complications. These treatment processes require close cooperation among multidisciplinary teams, involving neurotrauma, neuroimaging, ophthalmology, and neurocritical care.The original contributions presented in the study are included in the article/Ethical review and approval was not required for the study on human participants in accordance with the local legislation and institutional requirements. Written informed consent to participate in this study was provided by the participants' legal guardian/next of kin. Written informed consent was obtained from the individual(s), and minor(s)' legal guardian/next of kin, for the publication of any potentially identifiable images or data included in this article.YW: contribution to the conception, draft and revision of the work, analysis, acquisition, and interpretation of data. TC: contribution to the acquisition of data and revision of the work. MY, JM, ZL, and YC: contribution to the revision of the work and interpretation of data. XL: contribution to the conception and revision of the work. FC: contribution to the enlightenment of treatment strategy. JL: conception, revision of the work and analysis, acquisition, and interpretation of data. YW, TC, MY, JM, ZL, YC, XL, FC, and JL: approval of the final manuscript as submitted, and agreement to be accountable for all aspects of the work. All authors contributed to the article and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


In 1977, Carney et al. first described an association of gastric epithelioid leiomyosarcoma or gastrointestinal stromal tumor (GIST), pulmonary chondroma, and extra-adrenal paraganglioma. This previously unrecognized disorder came to be known as Carney’s triad. We describe a case of a 27-year-old female with metastatic GIST, diagnosed with Carney’s triad following pulmonary wedge resection, and highlight the surgical implication of this rare disease association. Carney triad is a rare syndrome characterized by the association of gastrointestinal stromal tumor (GIST), paraganglioma, and pulmonary chondroma. The etiology of the disorder is unknown. Diagnosis relies on a high index of suspicion from the clinician and management is surgical. A 27-year-old female presented with a history of abdominal pain. CT imaging of the abdomen revealed multiple gastric masses, hepatic lesions, bilateral adrenal adenomas, and a well-circumscribed pulmonary mass in the right lower lobe. Positron emission tomography revealed hypermetabolic activity in stomach, adrenal gland, retroperitoneal nodes, and in the right lung. CT-guided percutaneous lymph node biopsy of the gastric mass revealed a GIST . The patient was diagnosed with metastatic GIST and started on a highly selective tyrosine kinase inhibitor. The patient’s disease status remained stable for three years when she developed enlargement of one of her gastric masses. Upper endoscopy evaluation revealed an ulcerated bleeding gastric mass and evidence of gastric outlet obstruction. She underwent a distal gastrectomy with Billroth II reconstruction. Pathology was consistent with a GIST. The patient then developed progressive enlargement of her right lower lobe mass two years following gastrectomy. CT chest at time of evaluation revealed a 4.9 x 4.2 cm right lower lobe cystic lesion Figure .Following appropriate preoperative evaluation, she underwent an uncomplicated robotic-assisted right lower lobe wedge resection with negative margins. Pathology demonstrated a pulmonary chondroma with three reactive level 9R lymph nodes Figures , 3. A diThe disorder of synchronous or metachronous gastric GIST, pulmonary chondroma, and extra-adrenal paraganglioma was first described by Carney et al. in 1977, and thereafter came to be known as the Carney’s triad .GIST is the most commonly observed (99%) tumor in the triad, and gastrointestinal bleed or abdominal pain is often the presenting symptom . GISTs mPulmonary chondromas are rare benign lung tumors, present in 76% of cases of Carney’s triad, and GIST plus pulmonary chondroma was the most common combination of tumors 53%) . The pul% . The pExtra-adrenal gangliomas were present in 47% of Carney’s case series . In a reEach of the characteristic tumors in Carney’s triad is rare; however patients who develop these lesions in clusters should undergo thorough and ongoing surveillance, particularly if the patient is female, younger than 40 years, and has multifocal tumors. Surgical resection remains the mainstay of therapy and has curative potential. Long tumor-free intervals have been reported prior to recurrence or development of new sites of disease. Lifelong surveillance is recommended. Carney’s triad should be considered in a patient with metastatic GIST and lung tumor.


Paratesticular soft tissue sarcomas are very rare malignant mesenchymal tumors. With only few cases reported in the literature, data regarding diagnostic and management of these tumors are limited. We reported a case of primary paratesticular leiomyosarcoma in a 72-year-old man complaining of a progressively growing painless right scrotal mass. The patient underwent radical inguinal right orchiectomy and adjuvant 3D conformal radiotherapy to the tumor bed including the surgical scar. The prescription dose was 54 Gy, and no pelvic irradiation was performed. He remained free of recurrence for the last 16 months. Soft tissue sarcomas of the genitourinary tract are rare malignant tumors accounting for less than 2% of all urological tumors , 2. MoreA 72-year-old man with past medical history of hypertension and atrial fibrillation presented to the hospital with a progressively growing painless swelling in his right hemiscrotum as a chief complaint. No lower urinary tract symptoms were reported. Physical examination revealed a 3 cm firm-to-hard mass, isolated from the epididymis. Right epididymis and testis were normal. Scrotal ultrasound revealed a 4 × 3 right extra testicular heterogeneous lesion with peripheral vascularity. The patient underwent radical right inguinal orchiectomy. Grossly, the resected specimen measured 9 cm, showing a 3 cm testis covered by intact tunicae and a distant white surface mass measuring 2 cm. Four satellite nodules were associated, ranging from 2 to 5 mm. Combined histological examination and immunohistochemical study revealed a primary paratesticular grade 1 (score3) leiomyosarcoma arising from the spermatic cord Figures . He was th and 7th decades and survival rates ranging from 50% to 80% [Paratesticular leiomyosarcoma are unfrequently encountered malignant soft tissue tumors that may arise either from the epididymis, the scrotum, or the spermatic cord , 6. Few % to 80% . Authors% to 80% , 10.Typically, paratesticular leiomyosarcoma presents as a firm nontender scrotal painless mass, independent from the testis on physical examination. Scrotal ultrasound is the primary imaging modality for the evaluation of a scrotal mass, differentiating extratesticular from intratesticular lesions and dismissing several differential diagnoses such as epididymitis, cysts, and hydrocele . HoweverDue to the rarity of these malignancies, literature data regarding the optimal therapeutic management are inconclusive. However, most of the authors recommend radical orchiectomy with high ligation of the spermatic cord as the standard surgery . Adjunctp < 0.01) [Adjuvant management of paratesticular leiomyosarcoma remains controversial. The Memorial Sloan-Kettering Cancer Center released one of the largest retrospective studies on urologic sarcomas, including 131 patients, of whom 57 presenting with paratesticular localisation , 17. In  < 0.01) , 17. By  < 0.01) . TherefoWith limited available data, the diagnostic and treatment of paratesticular leiomyosarcoma remains challenging. Although radical inguinal orchiectomy is the mainstay of treatment, adjuvant radiotherapy may improve recurrence-free survival and should be considered, particularly for high-grade tumors. Multi-institutional collaboration and a centralized case management approach are needed to gain experience and better define the optimal management of these tumors.


Angiodysplasia is a common type of lesion characterized by malformed submucosal and mucosal blood vessels. Angiodysplasia of the gallbladder is extremely rare, usually an incidental finding, with only two cases reported. Laparoscopic cholecystectomy is a curative treatment for angiodysplasia of the gallbladder. Our report describes a case of angiodysplasia of the gallbladder in a patient who underwent elective laparoscopic cholecystectomy for biliary colic because of gallstones, and a systematic literature review. We surmise that angiodysplasia of the gallbladder could be a risk factor for gallstones in younger female patients. Vessels are essential, integrative structures of all tissues; therefore, their malformation could be the cause of various pathological conditions. According to the latest WHO classification of tumors of the digestive system, angiodysplasia includes clinically or endoscopically defined entities that are histologically considered to be vascular malformations . This isA 29-year-old woman was referred to the Department of Surgery of the Orašje County Hospital (Bosnia and Herzegovina) with a history of intermittent colicky right upper quadrant abdominal pain. She had no other significant medical history and had no previous history of gastrointestinal bleeding. Abdominal ultrasonography showed multiple gallstones within the gallbladder, with regular intra- and extrahepatic bile ducts, pancreas, and liver. Routine hematological and biochemical test results were normal. The patient underwent elective laparoscopic cholecystectomy. There were no intraoperative or postoperative complications, and the patient was discharged on the third postoperative day. The patient remained healthy at 6-year follow-up.Because of the lack of macroscopic pathological alterations, one sample of the gallbladder wall from the body and another from the neck were subjected to microscopic examination, which is a standard routine procedure for gallbladder grossing in our laboratory. Histologically, microscopic slides from the gallbladder body showed that almost the entire cylindrical epithelium of the mucosa was missing, with focal widening of the mucosal folds due to the accumulation of foamy histiocytes and foreign body-type multinucleated giant cells among numerous cholesterol crystal clefts. There was a moderate mixed inflammatory cell infiltrate in the submucosa, and the muscular layer was slightly hypertrophic, which is consistent with chronic cholecystitis. The most unusual feature of the slide from the body of the gallbladder was the presence of large dilated vessels within the muscular layer extending into the submucosa and mucosa . The appAngiodysplasia is an uncommon lesion that is mainly located in the gastrointestinal tract and may present either no symptoms or gastrointestinal bleeding. This type of lesion occurs at higher frequencies in patients with aortic stenosis, cirrhosis, pulmonary disease, renal failure, and von Willebrand's disease . The mosMost commonly, angiodysplastic lesions are typically seen in elderly patients of both sexes . With reThe pathophysiology of angiodysplasia of the gallbladder  remains unclear, although it is likely to be the result of increased contractility of the gallbladder, causing intermittent obstruction of the vessels that penetrate through the muscular wall. One hypothesis is that the occlusion causes focal dilatation and tortuosity of the overlying mucosal vessels, and another is that congestion of the capillaries and precapillary sphincter failure together lead to the formation of arteriovenous collaterals , 3. In oLaparoscopic cholecystectomy is curative for the treatment of angiodysplasia of the gallbladder, as evident from our case and the case reported previously . AlthougIn conclusion, we emphasize the importance of having examined detailed clinical data prior to grossing and tissue processing, to increase the possibility of obtaining an optimal tissue sample and an accurate diagnosis. Hence, the clinical picture of biliary colic and cholecystolithiasis in female patients younger than 40 years should be the “warning sign” for the pathologist to consider the possibility of an angiodysplasia finding.


Anterior inferior cerebellar artery (AICA) aneurysms are rare, less than 1%-2% of all intracranial aneurysms. Aneurysms of the distal AICA are even less common and can present with hearing loss and facial paralysis because of their relationship with the internal auditory canal (IAC). A 65-year-old male was followed for fluctuating left facial weakness and left-sided hearing loss for over a year. Serial magnetic resonance imaging (MRI) scans showed a mass near the left IAC, thought to be a vestibular schwannoma. Just prior to his next clinic visit, the patient deteriorated suddenly from a subarachnoid hemorrhage. Cerebral angiography revealed a 5.5 mm saccular aneurysm at the distal left AICA, which was clip ligated via a translabyrinthine (TL) approach. The patient had a good functional outcome  after 30 days despite persistent left facial weakness. Stable obliteration of the aneurysm was demonstrated by cerebral angiography postoperatively. Distal AICA aneurysms are rare and can have a similar presentation to tumors in the cerebellar pontine angle. Because of the unique anatomy of the distal AICA, open clip ligation via a TL approach is an effective method to secure these aneurysms. Anterior inferior cerebellar artery (AICA) aneurysms are rare and comprise less than 1%-2% of all intracranial aneurysms . AICA anA 65-year-old male with a past medical history of hypertension and hyperlipidemia presented to the emergency department with a sudden onset of left ear pain, tinnitus, decreased hearing, and facial paralysis after heavy-lifting. A computed topography (CT) scan of the brain was normal. The patient was given a tentative diagnosis of Ramsay Hunt syndrome and discharged home with prednisone and valacyclovir. Magnetic resonance imaging (MRI) of the brain on the following day revealed a 7 mm x 3.8 mm x 3 mm enhancing mass near the left IAC, interpreted as a VS. The patient was referred to the otolaryngology service and followed with serial MRIs of the brain at four-month, eight-month, and 18-month intervals. The last MRI showed an increase of the size to 8 mm x 4 mm x 4 mm with central non-enhancement . A non-contrast CT scan revealed a Fisher Grade 4 SAH Figure . He was On the second hospital day, the patient underwent clip ligation of this aneurysm via a TL approach Figure . The detFollow-up DSA revealed total occlusion of the aneurysm with patent proximal AICA and distal AICA filling retrograde from collaterals Figure . During AICA anatomy and aneurysm locationThe most widely used anatomical classification of AICA divides it into four segments: (1) anterior pontine segment (A1) from AICA origin to olivary prominence, 2) lateral pontine segment (A2) between olivary prominence and flocculus, 3) flocculopeduncular segment (A3) between the flocculus and cerebellopontine fissure, and 4) cortical segment (A4) . Typical flocculo lateral  corticalClinical presentationsAcute SAH is the most common presentation in patients with AICA aneurysms ,6,17. ApDiagnosisThe diagnosis of AICA aneurysm is usually straightforward with DSA or CTA. Some patients who present with headache, hearing loss, or other neurological symptoms may undergo MRI first. Differentiating a VS from an aneurysm can be difficult based on MRI alone. Marchini et al. proposed two features on MR imaging to distinguish AICA aneurysms from VS: absence of IAC enlargement and the “blurry dot sign” . The sigSurgical managementThe most commonly reported surgical intervention for occlusion of an AICA aneurysm is clip ligation via a retrosigmoid (RS) craniotomy. In the literature, there are only two cases of AICA aneurysm which were approached via a TL corridor. Gonzalez et al. used this approach for clip ligation of a proximal AICA aneurysm that was causing brainstem compression . Diaz etEndovascular techniquesMultiple methods have been applied endovascularly for AICA aneurysm embolization, such as coils, n-buytlcyanoacrylate, ethylene vinyl copolymer, and flow diverters ,8,18,19.OutcomePatients with AICA aneurysms often achieve a good clinical outcome even with aneurysm rupture ,4,10,13.Distal AICA aneurysms are rare, can present with hearing loss and facial palsy, and have a similar appearance to VS on MRI. Once recognized, endovascular or open surgical clip ligation can be used to secure the aneurysm. For distal AICA aneurysms in cases without useful hearing, clip ligation via a TL approach is an effective option.


Plexiform fibromyxoma of the stomach, also known as plexiform angiomyxoid myofibroblastic tumor, is a rare benign gastric mesenchymal tumor, first described in 2007, which usually arises in the gastric antrum and affects adults. Few cases have been reported in children and adolescents. It can present with different clinical manifestations including abdominal pain, dyspepsia, hematemesis, and vomiting. Preoperatively, this tumor is usually diagnosed as gastrointestinal stromal tumor (GIST), and the correct diagnosis is made only after histopathological examination following surgical resection. Most cases were reported from East Asia , North America, and Europe. We report herein a unique case of plexiform fibromyxoma, the first to be reported from the Middle East, arising in the cardia of the stomach in a 16-year-old adolescent male, with a brief review of the literature. Plexiform fibromyxoma (PF) is a rare benign gastric mesenchymal tumor, described for the first time in 2007 by Takahashi et al., who named it plexiform angiomyxoid myofibroblastic tumor of the stomach . It affeA 16-year-old boy presented with a recent history of two episodes of hematemesis, without associated other gastrointestinal symptoms. Six months earlier, he started dieting to lose weight, and he lost around 20 kilograms. Past medical, surgical, and family histories were unremarkable. Initially, he was seen at a local hospital, and a computed tomography (CT) scan was done there. CT scan showed a submucosal gastric mass. The patient then was referred to our hospital for further management.On physical examination, the patient was overweight, looking pale, but not in pain or distress. His vital signs were within normal limits. His abdomen was soft, not tender or distended, with no palpable masses. Laboratory investigations including hepatic and renal function tests, electrolytes, and coagulation profile were all within normal. However, complete blood count (CBC) revealed low hemoglobin values (8.2 grams per dL) which are most likely related to hematemesis.Computed tomography (CT) scan revealed a lobulated submucosal gastric mass at the gastric cardia near the gastroesophageal junction measuring 4.7 × 4.3 × 4 cm. The mass had a predominantly low attenuation component with central gas component which could be due to an associated ulcer. Superiorly, the mass had an exophytic component abutting the left hemidiaphragm and near the inferior aspect of the left hepatic lobe . The remUpper gastrointestinal (GI) endoscopy was done and showed normal esophagus, submucosal mass (5 cm) with deep ulcer at the cardia, and first and second parts of the duodenum were normal . In addiThe patient underwent laparotomy with wedge resection of the mass at the gastroesophageal junction, with primary closure of the stomach. The postoperative course was uneventful.Pathological examination of the specimen, which was labelled as “gastroesophageal junction mass,” revealed a single mass covered by mucosa featuring three ulcerated areas; the largest measured 1.5 × 1 × 0.6 cm and the other small measuring 0.6 and 0.5 cm in diameter. The mass was serially sectioned revealing a single rubbery soft white homogenous well-circumscribed tumor with whorly appearance, measuring 5 × 5 × 4.5 cm. The ulcerated area showed necrotic material inside the cavity of the ulcer. On histopathological examination, sections showed a mesenchymal myxoid multinodular plexiform tumor arising within the muscularis propria that extended to the submucosa and reached into areas of the muscularis mucosae. The overlying gastric mucosa featured areas of ulceration covered by neutrophilic exudate with granulation tissue formation, and mixed inflammatory infiltrate was noted. Lymphoid follicles were seen in the lamina propria.The tumor was composed of nodules of spindly mesenchymal cells with elongated nuclei and conspicuous nucleoli and eosinophilic fibrillary cytoplasm, embedded within loose myxoid stroma with scattered capillaries seen. The tumor nodules were seen dissecting their way or surrounded or traversed by bundles of smooth muscle fibers derived from the muscularis propria resulting in a plexiform pattern Figures . ImmunohOne week postoperatively, upper GI contrast study revealed free flow of contrast along the whole segments of the esophagus and through the gastroesophageal junction down to the stomach with no evidence of contrast leakage. Diet was advanced as the patient tolerated. The patient was discharged home with analgesic, pantoprazole, and follow-up appointment in the clinic.Helicobacter pylori. The patient was started on proton pump inhibitor (PPI) drugs and treatment for Helicobacter pylori.On follow-up visits at the clinic, he was doing well, tolerating diet, and did not have any episodes of hematemesis, abdominal pain, or reflux. Four months following surgery, upper GI endoscopy was done, and it demonstrated gastric esophageal reflux (GERD) with mucosal break > 5 mm above the Z line with sutures on the other site. The endoscopic examination of the stomach showed folded mucosa near the cardia, with normal appearance. A scar of recently healed ulcer was seen in the first part of the duodenum. Biopsy taken during the endoscopy showed chronic active follicular gastritis with Three years following his surgery, he was doing well and tolerating diet. He did not have any episodes of hematemesis, abdominal pain, or reflex. His most recent upper GI endoscopy at 30 months postoperatively showed no tumor recurrence. We planned to follow up the patient every 6 months to ensure the absence of any recurrences.Since its initial description in 2007, this rare gastric neoplasm that was named initially as “plexiform angiomyxoid myofibroblastic tumor” (PAMT)  had beenThe most common location of PF is in the gastric antrum ; in some cases, the duodenal bulb is involved (22%). However, it is less common to be found at the gastric body  and at the gastric fundus  [In addition to the current case, there had been eleven previously reported cases of PF in children below the age of 18 years . Their aPF presented usually as a submucosal mass; however, it could involve any layer from the gastric serosa to the gastric mucosa. Finding of ulcer associated with tumor was not uncommon, and it explained the hematemesis our patient had. The size of the tumor varied from 1.5 cm up to 15 cm , 3.Its diagnosis prior to surgery is difficult and is often confused with GIST based on findings on images and endoscopy. In a recent multicenter study of seven cases, Lai et al. had intraoperative frozen sections and/or EUS-FNA on six cases, and all of them were diagnosed preoperatively or intraoperatively as GIST. EUS-FNA material showed elongated spindle cells with streaming oval or elongated nuclei, features that can be seen in GIST . PF can The role of CT scan in differentiating PF from GIST had been reported by Ikemura et al. They stated that PF showed a heterogeneous tumor in the gastric antrum which was drastically enhanced with contrast medium and consisted of a number of highly small nodules around the tumor rim. These CT findings reflect the characteristic growth pattern of PF and are claimed by the authors to be distinct enough to differentiate it from GIST .Treatment is mainly surgical resection. The kind of surgical resection is usually determined by the size, the depth, and the location of the tumor. Surgical procedures include distal gastrectomy (most commonly reported surgery), partial gastrectomy, wedge resection, antrectomy, subtotal gastrectomy , 3, and Among all the reported cases, PF exhibited a benign biological behavior, with low mitotic activity and no local recurrence or distant metastasis , 10. In Plexiform fibromyxoma, a rare mesenchymal gastric tumor, usually presents with nonspecific upper gastrointestinal symptoms. It is quite uncommon to be encountered in children with only ten cases on record. It is most commonly found in the gastric antrum but can be located anywhere in the stomach. We report the twelfth case in the pediatric age group, with the unusual location of the tumor at the gastric cardia near the gastroesophageal junction. Images and endoscopy can aid in assessing the location and the size of the tumor, which helps to decide the best surgical technique. However, the diagnosis is usually established after histopathological examination of the tumor following surgery. It seems that this tumor has no potential of recurrence or metastasis.


Immunotherapy-based combinations have become standard of care in advanced renal cell carcinoma (RCC). Despite the potential for complete radiographic response, complete pathologic responses have been rarely reported. We present two cases of confirmed complete pathologic response to immunotherapy despite residual radiographic abnormalities. The first case describes a 68-year-old female with metastatic RCC who was treated with upfront pembrolizumab plus axitinib. She underwent nephrectomy after 15 doses of pembrolizumab with pathology revealing no evidence of viable tumor. To our knowledge, this is the first reported case of a complete pathologic response with pembrolizumab in metastatic RCC. The second case describes a 64-year-old female with metastatic RCC who was treated with second-line nivolumab after progression on cabozantinib. After 13 doses of nivolumab, she underwent nephrectomy with pathology revealing no evidence of viable tumor. These cases highlight the potential for scar tissue, fibrosis, and necrosis to persist radiographically after treatment with immunotherapy despite the absence of viable tumor cells. Immunotherapy and immunotherapy-based combinations have revolutionized the treatment of advanced renal cell carcinoma (RCC). Immunotherapy utilizing checkpoint inhibition was FDA approved in RCC after CheckMate025 demonstrated improved overall survival of nivolumab compared with everolimus in the VEGF-refractory setting . UpdatedA 68-year-old female presented in April 2019 with progressive left-sided abdominal pain and a 45-pound weight loss. Subsequent CT scan revealed a 7.7 x 4.2 x 4.9 cm complex left renal mass with left adrenal gland involvement and regional lymphadenopathy Figure 2A 64-year-old female presented in July 2018 with progressive abdominal pain, nausea, 40-pound weight loss, and new cough. She was found to have a palpable right upper quadrant mass with CT scan revealing an 11 x 9.3 cm heterogeneously enhancing mass replacing the majority of the right kidney Figure 4Review of published literature revealed five other reported cases of complete pathologic response to immunotherapy in RCC at time of radical nephrectomy: three cases after treatment with nivolumab monotherapy, one after combination nivolumab plus ipilimumab, and one after combination nivolumab plus cabozantinib –14. To oThe patient from Case 1 underwent coil embolization for refractory hematuria after her first dose of pembrolizumab. While the intent of embolization was not disease control, there have been some studies of locoregional therapy in combination with immunotherapy in hepatocellular carcinoma , less isThese cases provide examples of confirmed complete pathologic responses to immunotherapy. However, without pathologic interrogation of tissue from prospective trials, the true rate of these responses is unknown. There are several ongoing neoadjuvant studies in both the locally advanced and metastatic setting that will help determine the rate of pathologic response Table 2.Interestingly, a post-hoc exploratory analysis of Keynote-426 showed that depth of response (such as >80% tumor reduction but less than complete response) was associated with improvement in overall survival in the pembrolizumab plus axitinib arm, with similar overall survival to the group with complete radiographic response . AdditioWhile the patient in Case 1 discontinued immunotherapy after nephrectomy, the patient in Case 2 elected to resume treatment. Importantly, there are currently no data to confirm that the discontinuation of systemic treatment after nephrectomy, even with a complete pathologic response, is safe in the metastatic setting. Therefore, the decision to resume immunotherapy after nephrectomy remains an individualized decision between patients and their oncology team, taking into account their initial response to therapy and their tolerability of adverse effects.The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding author.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.All authors contributed equally to this case report. All authors contributed to the article and approved the submitted version.KB, research funding to institution: Bristol-Myers Squibb for a young investigator grant; serves on advisory board for MedOnc Live and Exelexis. ND, research funding to institution: Astra-Zeneca, Hoffman-LaRoche, Pfizer, Merck, Incyte, Immunomedics, Mirati Therapeutics, Seattle Genetics, Exelixis, Taris Biomedical, Bristol-Myers Squibb, Jounce Therapeutics; Travel: Calithera Biosciences. BR, research funding to institution: Pfizer, Merck, GNE/Roche, Aveo, Astra-Zeneca, Bristol-Myers Squibb, Exelixis; consulting: BMS, Pfizer, GNE/Roche, Aveo, Synthorx, Compugen, Merck, Corvus, Surface Oncology, 3DMedicines, Arravive, Alkermes, Arrowhead, GSK; stock: PTC therapeutics.The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.