For carefully selected patients with low-lying rectal cancers, ultralow anterior resection (ULAR) can be an effective alternative to abdominal perineal resection, and together with neoadjuvant radiochemotherapy can provide the opportunity for sphincter preservation. However, ULAR is not without potential postoperative complications, particularly anastomotic dehiscence which increases in likelihood after receiving radiation therapy. While surveillance imaging is not indicated three years beyond initial surgical resection, changes in chronic symptoms refractory to conservative management may warrant further investigation. In this case report, we present an interesting case of late-onset stenosis and anastomotic breakdown following neoadjuvant radiochemotherapy, ULAR, and coloanal anastomosis for a low-lying rectal adenocarcinoma. Effective patient education, reliable symptom assessment, and multidisciplinary collaboration are essential to assessing for long-term treatment-related complications and providing appropriate treatment in a timely manner. The goals of surgery for rectal cancer are cure, maintenance of function, and optimization of quality of life. For carefully selected patients with low-lying rectal cancers, ultralow anterior resection (ULAR) has become an alternative technique to abdominal perineal resection as it provides a chance for organ preservation . When coUnfortunately, ULAR is associated with potential postoperative complications, the rates of which likely increase with the addition of radiation therapy . PatientWe report an interesting case of late-onset stenosis, anastomotic breakdown, and abscess formation following neoadjuvant radiation and chemotherapy, ULAR, and CAA for a low-lying rectal adenocarcinoma.A 67-year-old man with no comorbidities and no history of smoking was diagnosed with a cT2 N2 M0 rectal adenocarcinoma and was treated in 2008 with neoadjuvant pelvic radiotherapy (50.4 Gy/28 fractions), concurrent 5-fluorouracil-based chemotherapy, laparoscopic ULAR and CAA, and subsequent adjuvant chemotherapy with folinic acid, fluorouracil, and oxaliplatin. Radiotherapy consisted of a then standard four-field pelvic plan delivering 45 Gy/25 fractions to the primary tumor and mesorectal-, presacral-, and internal iliac lymph node regions followed by a sequential four-field boost plan to the primary tumor. Postoperative pathologic analysis revealed a well-differentiated rectal adenocarcinoma without evidence of perforation, perineural invasion, or lymphovascular invasion. The closest surgical margin was 1.5 cm from the distal edge, and the final pathologic stage was ypT2 ypN0.The patient did well in follow-up over the next five years, without evidence of recurrent disease based on clinical assessments, colonoscopy, CT imaging, and carcinoembryonic antigen level monitoring. Unfortunately, he later developed fibrosis and stenosis within the anal canal, causing pain with defecation and pencil-thin stools. The anal canal and low rectum were appreciably narrowed and firm with marked restriction at the point of anastomosis 4 cm from the anal verge. His pain slowly increased in later years, presumed to be from worsening fibrosis. He eventually developed rectal frequency (upwards of 10 bowel movements per day), urgency, and occasional fecal incontinence. Initial medical intervention included oxycocet, proctosedyl suppositories, and topical creams containing 5% ketamine, 5% lidocaine, 6% gabapentin, and 2% baclofen. Despite these treatments, the patient continued to have severe pain peaking at 10/10 in intensity and bowel movements that had a significant impact on his quality of life. Dietary adjustments provided no improvement.A repeat CT revealed the presence of new soft tissue and extraluminal air proximal to the posterolateral aspect of the anastomosis Figure . This reThe present case was an instance of low rectal cancer treated with multimodality therapy including ULAR and late-onset anastomotic dehiscence and abscess formation.Preoperative radiotherapy in the treatment of rectal carcinomas can be beneficial among patients with a planned resection for locally advanced disease . When usAnastomotic dehiscence is a major cause of postoperative morbidity in patients with rectal cancer, with reported leakage rates from 3% to 20% after sphincter-saving resection . The likRadiotherapy has been identified as a risk factor for anastomotic leakage. Dehiscence of anastomoses was reported in 10% of patients following preoperative radiotherapy, with an increased risk in those receiving a short-course rather than long-course regimen . This maWhile surgical technique and neoadjuvant therapies play a role in anastomotic breakdown, patient-related factors such as age greater than 60 years, male gender, preoperative medical disease, obesity, bowel obstruction, smoking, and alcohol abuse can significantly influence the probability of leakage ,13. PostCancer Care Ontario guidelines recommend surveillance imaging annually for three years following surgical resection for a colorectal malignancy . Beyond The present report serves as a reminder that maintaining an open line of communication between the patient and health care team is essential to identify possible recurrences or complications. The surgeon, radiation/medical oncologist, family physician, and pain and symptom specialists can all work together to devise an individualized treatment and surveillance plan.


Ganglioneuroma (GN) is ranked by the International Neuroblastoma Pathology Classification as a benign tumor. It can occur anywhere along the sympathetic nerve chain and surgical excision is the treatment of choice.An 18-year-old female patient sought medical assistance after 6 months of constant dorsal and back pain radiating from the thoracic region to the right abdominal flank. Magnetic resonance imaging revealed a solid nodular lesion with heterogeneous post-contrast enhancement and lobulated contours, centered on the right foramina of D12–L1, with a projection to the intracanal space, which compressed and laterally displaced the dural sac and had a right paravertebral extension between the vertebral bodies of D11 and superior aspect of L2. Ganglioneuroma was diagnosed using immunohistochemical analysis. It was decided to use a surgical approach in two stages: robot assisted for the anterior/retroperitoneal mass and a posterior hemilaminectomy/microsurgical approach to attempt total resection, avoiding the traditional anterior thoracoabdominal surgical incision and optimizing the patient’s postoperative outcomes. No postoperative adverse events were noted, and the patient was discharged on postoperative day 5.This retroperitoneal GN presentation was peculiar because it originated at the D12 nerve root, which extended to the retroperitoneal space and inside the spinal canal. We hope that our case report can assist future decisions in similar circumstances.The online version contains supplementary material available at 10.1186/s12893-021-01146-x. Ganglioneuroma (GN) is the most mature form of a peripheral neuroblastic tumor , 2, i.e.GNs can occur anywhere along the sympathetic nerve chain and commonly at the mediastinum, retroperitoneum, and adrenal glands . SurgicaHere, we describe the case of an 18-year-old female patient with retroperitoneal GN, which was surgically removed. The time elapsed between first consult and surgical intervention was four months. In addition to the tumor’s rarity, this case is of special interest because of the associated pathologic presentation of a retroperitoneal mass with spinal invasion and the innovative combination of robot-assisted surgery with a traditional neurosurgical approach.An 18-year-old female patient, with uneventful past medical history and no relevant family history, reported constant dorsal and back pain radiating from the thoracic region to the right abdominal flank for 6 months. Pain worsened at physical exertion. The pain was initially treated with anti-inflammatory drugs and analgesics, with a good response for 2 weeks, after which, it became progressively resistant to medication. The patient provided informed written consent that was approved by the IPSPAC Institutional Review Board.Patients neurologic evaluation revealed decreased touch and pain perception between the T7 and T11 dermatomes. Reflexes, muscle strength, and laboratory results were normal.SRY (sex determining region Y)-box 10) (SOX-10) antigens] could suggest other diagnoses, we performed a lamina revision in a second pathology laboratory, which confirmed a diagnosis of GN.Gadolinium-enhanced spine magnetic resonance imaging (MRI) revealed a solid nodular lesion with heterogeneous post-contrast enhancement and lobulated contours centered on the right foramina of D12–L1, with a projection to the intracanal space, which compressed and laterally displaced the dural sac having a right paravertebral extension between the vertebral bodies of D11 and superior aspect of L2. It measured approximately 5.0 × 3.0 × 9.5 cm  . Preoperative preparation, patient positioning, and port-site access followed the lateral transabdominal laparoscopic adrenalectomy protocol. The patient was placed in the left lateral decubitus position and five trocars were inserted: an 8-mm camera port was inserted midway between the umbilicus and the right costal margin 20 cm away from the target; two robotic instrument ports, both 8 mm, were inserted along a line two fingerbreadths 8 cm away from each other over the umbilicus; one robotic instrument 8-mm port was inserted along the same line under the umbilicus at the right iliac pit away from the 8-cm camera port; a 5-mm liver retraction port was inserted in the midline in the epigastrium, and an accessory 10/12 mm auxiliary port was inserted between the camera port and right iliac pit port 7 cm away from each other. The four-step technique of robotic right adrenalectomy was applied : (1) comAfterward the anterior step was performed, the patient was positioned in ventral decubitus position and prepared for a traditional laminectomy. After a dorsolumbar medial incision, a regular bilateral paraspinal muscular dissection was performed. The D11, D12, and L1 lamina, facets, and transverse process were exposed. After performing a D11–L1 hemilaminectomy, the tumor’s origin was identified from the D12 nerve root. The tumor extended from the retroperitoneal space to the spinal canal, entering from the D12–L1 right foramina and compressing the dura mater. Following the reclamation of the D11–D12 zygapophyseal facet and D12 transverse process, the posterior intracanal and paravertebral elements of the mass were removed under microscope visualization. The D12 nerve root had to be severed for total tumor removal; afterward, the retroperitoneal space could be recognized. Postoperative pathologic examination confirmed that it was a GN. Figure Postoperatively, the patient recovered well. On postoperative day 1, the patient experienced episodes of muscle pain in the abdominal wall and laminectomy region, controlled with analgesics and no further investigation was needed. Patient mobilization occurred on postoperative day 2, when the patient could walk with assistance. The patient was discharged on postoperative day 5. After 1-year of follow-up, the patient had improved muscle strength but still experienced episodes of myofascial pain in the dorsum and on the right lateral abdominal flank, again controlled with analgesics. However, she did not adhere to her physical rehabilitation protocol.GNs are rare benign neurogenic (ganglion cell) tumors, commonly affecting adolescents and youth adults , 8. AlthRetroperitoneal GN commonly presents local mass-effect symptoms, leading to an often casual diagnosis , 11, 13.GN is also distinguished by the increased production of catecholamines, with Verner–Morrison syndrome as the recurrent example of this endocrine dysregulation., 14, 15 Cai et al.  reportedPreoperative diagnosis predominantly relies on imaging techniques. Reported imaging findings , 8, 17 iConsidering the indefinite diagnosis suggested by the imaging examination, a biopsy and/or excision for histopathological examination is frequently performed. Besides the level of cellular maturity, distinct between malignant neuroblastic (neuroblastoma and ganglioneuroblastoma) tumors and GNs is the presence of hemorrhage, metastasis, and necrosis in malignant presentations .The International Neuroblastoma Pathology Committee  standardAlthough partial or no resection can be accepted as a GN treatment , 25, theNevertheless, to our knowledge, no other case treatment was as innovative as the association of these two surgical techniques. The traditional option for removing a retroperitoneal mass would involve an extensive thoracoabdominal surgical incision, such as in adrenalectomy procedures. In such cases, open surgery can result in higher doses of analgesics –31, greaAdditional file 1: Video S1. Video that demonstrates liver retraction.mp4.Additional file 2: Video S2. Video that demonstrates tumor dissection.mp4Additional file 3: Video S3. Video that demonstrates laparoscopic suturing of the diaphragm.mp4.


Robot-assisted thoracoscopic surgery (RATS) is useful for surgery in the apical region of the chest cavity, as it narrows towards the head. Here, we describe a nonfunctional, rib-invasive paraganglioma arising in the posterior mediastinum that was successfully removed using RATS combined with chest wall resection.A 31-year-old woman presented with a posterior mediastinal mass on chest computed tomography (CT) scan during a medical check-up 2 years prior. Positron emission tomography/computed tomography scan with F-18 fluorodeoxyglucose revealed a mass associated with standardized uptake maximum value of 2.69. With a preoperative diagnosis of neurogenic tumor by CT-guided percutaneous fine-needle aspiration biopsy, we performed robot-assisted tumor resection combined with chest wall resection. The wristed instruments of the robotic surgical system have increased range of motion and enabled the tumor resection without organ injury in the thoracic cavity. Histopathology examination revealed a non-functional paraganglioma with rib invasion.RATS is a useful technique, enabling safer and easier resection of a mediastinal tumor adjacent to surrounding organs. Mediastinal paragangliomas are rare tumors that arise from extra-adrenal paraganglionic cells of the sympathetic nerve system; they are commonly known as pheochromocytomas. Paragangliomas are classified as functional or nonfunctional based on their ability to synthesize and release catecholamines.Robot-assisted thoracoscopic surgery (RATS) has been used to overcome the limitations of video-assisted thoracoscopic surgery with favorable results for mediastinal tumors; the number of RATS procedures has increased rapidly in Japan , 2. HereA 31-year-old woman presented with a posterior mediastinal mass on chest computed tomography (CT) scan during a medical check-up two years prior. She had left shoulder pain presentation to our hospital. Contrast-enhanced CT revealed a solitary and non-homogeneous 4-cm mass in the posterior mediastinum invading the left 2nd rib (Fig. Under general anesthesia, the robot was positioned at the head of the operating table. The patient was intubated with a nerve integrity monitor electromyogram endotracheal tube for intraoperative neuromonitoring and place.Robotic 8-mm ports were introduced Fig. a. The daGrossly, the resected tumor was a tan red-colored, irregular-surfaced and ovoid-shaped mass measuring 4.5 × 3.3 × 2.2 cm (Fig. Various approaches have been described for surgical treatment of neurogenic mediastinal tumors. These include thoracotomy , 5, neckIn the present case, we performed tumor resection using RATS combined with chest wall resection. Although the mediastinum is tightly packed with intertwined organs and conduits, the wristed instruments of the robotic surgical system have increased range of motion and enabled the tumor resection without organ injury in the thoracic cavity. In addition, some authors reported the postoperative course of RATS. In comparison with conventional median sternotomy and VATS approach, RATS revealed lower amount of postoperative drainage, shorter duration of chest drainage and length postoperative hospital stay . These rParagangliomas develop from chromaffin cells of the sympathetic nervous system distributing over the body . Most meIn conclusion, RATS is a useful procedure, enabling safe and minimally invasive resection of mediastinal tumors invading the surrounding organs. Further applications in thoracic surgery are expected in the future.Additional file 1. Intrathoracic view during the surgery. The parietal pleura surrounding the tumor was resected with the robotic electrocautery, the edge was dissected from the lower edge of the first rib to the upper edge of the fourth rib. The first and second intercostal muscles were dissected to secure the surgical margin. After that the second rib was cut down through the posterior incision, and the tumor was removed in its entirety, including the chest wall, from the thoracic cavity.


Non-functional, extra-adrenal, retroperitoneal paraganglioma is a rare, neuroendocrine, and potentially malignant tumor. Its diagnosis and treatment may be challenging. A 69-year-old female patient was admitted because of a left para-aortic, solid, 4.4-cm mass, incidentally discovered during abdominal ultrasonography for screening purposes. Her clinical examination was unremarkable. Preoperative differential diagnosis based on cross-sectional imaging included tumor of neuroendocrine or mesenchymal origin. Hormonal investigation with 24-hour urinary catecholamines and metanephrines and plasma-fractionated metanephrines was in the normal range. Following consultation with the endocrinologist and anesthesiologist, the tumor was removed by using the three-dimensional (3D) laparoscopic transperitoneal surgical approach. The perioperative course was uneventful and the patient was discharged on the third postoperative day. Histopathologic findings were consistent with the diagnosis of retroperitoneal extra-adrenal paraganglioma of 5 cm in maximum diameter. Paragangliomas are rare neuroendocrine tumors originating from chromaffin cells along the sympathetic and parasympathetic chains . ParasymIn June 2019, a 69-year-old female patient was admitted to the Department of Urology of Korgialenio-Benakio Hellenic Red Cross Hospital, Athens, Greece, due to a tumor within the left para-aortic space. Her medical history included depression. She was given citalopram hydrobromide 10 mg per os on a daily basis. In addition, she was placed under endocrinologist’s surveillance due to the presence of thyroid nodules. Clinical examination was unremarkable.The mass had been incidentally discovered during abdominal ultrasonography for screening purposes three months ago. Further investigation with an abdominal CT scan demonstrated the presence of a well-defined, left para-aortic, solid, 4.4-cm mass, located at the height of the fourth lumbar vertebra. The lesion presented heterogeneous enhancement following intravenous administration of contrast agent. The CT scan could not differentiate between the mesenteric mesenchymal lesion and the retroperitoneal tumor Figure . PreoperAfter obtaining informed written consent, the patient underwent surgical removal of the mass under general anesthesia, by using three-dimensional 3D) laparoscopic transperitoneal, 4-trocars lateral approach with the lesion side up and the table half-flexed. A 3D high-definition camera and a 30° laparoscope were used, while pneumoperitoneum was performed by using an open Hasson technique and was steadily maintained at 12 mmHg. Following the tumor’s exposure, the mass was manipulated gently and the dissection was performed by using monopolar hook diathermy and a harmonic scalpel  and for synaptophysin  and negative for epithelial AE1/3 cytokeratin. Sustentacular cells stained with S100 protein were identified at the periphery of the nests. Histopathologic findings were consistent with the diagnosis of paraganglioma. The cellular proliferation marker Ki67 was low (1-2%) and the tumor’s score was 5 according to the Grading System for Adrenal Pheochromocytoma and Paraganglioma (GAPP), corresponding to paraganglioma of intermediate differentiation Figure . Tumor’s123I-metaiodobenzylguanidine (MIBG) scintigraphy, abdominal CT scan, and hormonal investigation were negative six months postoperatively, and a rigorous follow-up scheme including outpatient consultations with oncologist and endocrinologist was subsequently scheduled.Three months postoperatively, the patient underwent thyroidectomy, and histology revealed papillary thyroid cancer. Postoperative genetic screening was negative. Retroperitoneal extra-adrenal paragangliomas are rare tumors originating from chromaffin cells of the primitive neural crest during their migration to form the paraspinal sympathetic paraganglia. They have an estimated incidence of 2-8 per million people in the general population ,7-9. TheThey usually present as a single unilateral tumor; however, in 10-50% of cases, they are part of hereditary syndromes like multiple endocrine neoplasia (MEN) types 2A and 2B, Von Hippel Lindau disease, neurofibromatosis type 1, Carney-Stratakis dyad, and familial paraganglioma associated with mutations in genes encoding succinate dehydrogenases B, C, and D ,10. The Clinical presentation and findings contribute to the diagnosis, although there are asymptomatic forms . The triBoth preoperative biochemical investigation and imaging modalities play a vital role in the diagnosis of paraganglioma and in subsequent treatment planning . Elevate123I MIBG scintigraphy, 68Ga-labeled 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid-octreotate (DOTATATE) positron emission tomography (PET)/CT, or 18F-labeled L-dihydroxyphenylalanine (L-DOPA) PET/CT provide a whole-body investigation and are able to differentiate between functional and non-functional paraganglioma and to detect synchronous tumors or metastases, especially if CT scan and MRI are negative and if there is a strong suspicion of catecholamine secreting tumor [CT scanning and MRI are used in order to locate the tumor . MRI is ng tumor ,12. Diagng tumor . It is wng tumor .Following diagnosis, an α-blocker is administered at a dose progressively up-titrated for at least one to two weeks preoperatively, accompanied by high sodium and fluid intake ,11. The 131I-MIBG for tumors that exhibit uptake on diagnostic scan) are viable treatment options for metastatic disease [Surgical resection represents the standard of care and can be performed traditionally by open surgery, while there has been a trend towards minimally invasive procedures in recent years ,4. Furth disease .Histopathologic findings can usually confirm the diagnosis of a retroperitoneal paraganglioma, as the tumor microscopically shows the classic zellballen pattern, formed by well-developed, polygonal chromaffin cells surrounded by an intervening fibrovascular stroma and peripheral sustentacular cells . ImmunocThe prognosis of retroperitoneal paraganglioma is good in the case of a completely resected benign lesion and poor in the case of a malignant one . AccordiNon-functional, extra-adrenal, retroperitoneal paraganglioma poses a real diagnostic and surgical challenge. Growing evidence indicates that minimally invasive surgical procedures are effective and safe in the treatment of such lesions. A multidisciplinary medical approach and treatment planning are of paramount importance to achieve optimum outcomes for the patients, both oncologically and with regard to perioperative complications.


Sialolipoma is a rare tumour which may arise from both major and minor salivary glands and has recently been described as a variant of salivary gland lipomatous lesions.We report a 54-year-old male who presented with a 7-year history of large right anterior neck swelling. He was clinically euthyroid and had no compressive or infiltrative symptoms. He sought medical attention due to the discomfort exerted by the weight of the mass and was keen for excision. The swelling appeared like a goitre but physical examination proved otherwise. Imaging was suggestive of a benign tumour arising from the right parapharyngeal fossa. The mass was surgically excised and was noted to be adherent to part of the submandibular gland. Histopathological examination revealed a new variant of benign adipocytic tumour of salivary gland or sialolipoma arising from the submandibular gland. Besides being the largest sialolipoma to be reported, there are also no reports of giant submandibular sialolipomas masquerading as a huge goitre in appearance.Submandibular sialolipomas can present in really large sizes and appear as a giant goitre. It is important to differentiate between benign lipomas from liposarcomas and tailor the management accordingly. Surgical enucleation is the preferred choice of treatment for these benign tumours with low recurrence rates. A huge anterior neck swelling is almost always associated with goitre by a commoner especially from the endemic region. However, that is not always the case. History, clinical examination and imaging allows us to ascertain the origin of the mass. Large anterior neck swellings theoretically can arise from the skin, subcutaneous tissue, muscle and various other structures in the region. Common causes that we usually encounter are thyroglossal cyst, lymphangioma, branchial cyst, cystic hygroma, lymphadenopathy and goitres. Lipomatous lesions of the head and neck region are generally uncommon and may arise from any structure that contains fat tissue. Sialolipoma is a rare variant of lipomatous tumour occurring within the salivary gland with less than 100 cases reported globally. Histologically, it is composed of predominantly mature adipocytes intermingled with various benign salivary gland parenchyma . We repoA 54-year-old gentleman presented with a 7-year history of right neck swelling which had progressively increased in size without compressive or infiltrative symptoms. He complained of a pulling discomfort exerted by the weight of the mass, accentuated by gravity in the erect position. He denied history of malignancy in the family or exposure to radiation. On examination, he was clinically euthyroid. The 10 × 10 cm swelling was located at the right anterior triangle of the neck extending from the submandibular region to the level of the cricoid cartilage and medially not crossing the midline Fig. . A fine A multidisciplinary team discussion between general surgery, radiology and otorhinolaryngology team was undertaken to discuss the MRI images and possible approaches to excise this lesion. The patient subsequently underwent excision of the lesion with the otorhinolaryngology team on standby for radical surgery if findings were suggestive of malignancy. Intraoperatively, a single, lobulated, lipomatous tumour measuring 10 × 12 cm in size with multiple engorged vessels on its surface was found to be located at the right parapharyngeal fossa Fig. a - c. ItHistopathological examination revealed a fairly circumscribed lesion composed of lobules of various sizes and mature univacuolated adipocytes separated by fibrous septae Fig. . It measSialolipoma is classified as a new histological variant of lipoma instantiated by a benign salivary gland including salivary ducts and serous acini admixed with mature adipose tissues. It was first introduced by Nagao et al. before the term sialolipoma was accepted by the World Health Organization Classification of Head and Neck Tumours in 2005 . It is dThe aetiology of anterior neck swellings can be divided by onset or duration, namely, congenital, acute, subacute and chronic. In this case, a 7-year history of a progressively growing neck swelling is suggestive of its chronicity. The initial primary diagnosis for these swellings can be of a thyroid pathology, laryngocele, thyroglossal duct cyst, lipoma, liposarcoma or even parathyroid carcinoma . HoweverImaging modalities in lipomatous tumours provide a preoperative diagnosis and allows the surgical team to plan the operation besides looking for features to suggest malignancy. Simple ultrasonography can be useful but its findings vary depending on the expertise of the operator. In good hands, it can predict the tumour origin as in our case. Computed tomography (CT) and MRI are more accurate in depicting the tumour texture, location and compressive or infiltrative features compared to ultrasonography. Additionally, MRI allows us to differentiate between lipoma and liposarcoma by looking at the intensity of the fat signal and is superior to the conventional CT in this aspect. MRI findings suggestive of liposarcoma are namely thickened or nodular septa , its association with non-adipose masses and prominent foci of high T2 signal and prominent areas of enhancement . Fine neThe treatment of choice in managing sialolipomas is surgical excision of the involved salivary gland. Guided by MRI, access to the tumour can be decided and this is a very crucial step in planning the surgery. In certain cases, the lipoma may be intramuscular hence posing a greater challenge to achieve complete tumour excision. Bleeding from the muscle is a common occurrence intraoperatively and meticulous haemostasis is important in such cases. Other possible risks and complications pertaining to this surgery include injury to the surrounding vessels and nerves, namely the facial artery, marginal mandibular nerve , lingual nerve and hypoglossal nerve. Post-operative salivary fistula and sialocoele are complications that need to be avoided. Nevertheless, proper planning and complete surgical excision provides good surgical outcomes as the risk of recurrence is zero .In conclusion, submandibular sialolipomas can present in really large sizes and appear as a giant goitre. The attending surgeon should be able to differentiate between benign lipomatous tumours and liposarcomas through history, physical examination and imaging. Complete surgical enucleation with the involved salivary gland is the mainstay of treatment with low recurrence rates.


Malrotation is a congenital anomaly during the development of the embryonic intestine. Although it is generally considered a pediatric surgical condition, it can have significant implications for adult surgery in terms of reconstruction.The patient was an 85-year-old man with pancreatic cancer and intestinal malrotation. He underwent pancreaticoduodenectomy with modified Child’s reconstruction. Because the ascending colon and efferent loop twisted easily, we fixed the ascending colon to the abdominal wall. Thereafter, right twist and stenosis of the efferent loop occurred. On the 22nd day after the initial surgery, detorsion and Braun anastomosis were performed for efferent loop fixation. Postoperative oral intake was good, and the patient was discharged from our hospital on the 24th day after the reoperation.This is a rare case of pancreaticoduodenectomy with malrotation following reoperation due to a complication after Child’s reconstruction. In similar cases of intestinal malrotation, it is important to consider avoiding coaxial positioning of intestinal parts and an upper abdominal space while selecting a reconstruction method. Intestinal malrotation is a developmental anomaly of the embryonic intestine, which is frequently observed in neonates. Although it occurs rarely in adults, it influences the condition of the intestines and has significant implications for reconstruction surgery. Here, we present the case of a patient who underwent pancreaticoduodenectomy and had intestinal malrotation following reoperation due to torsion of the efferent loop.The patient was an 85-year-old man who presented with appetite loss and choluria. His past medical history included hypertension, tuberculosis, and ulcerative colitis. Laboratory examination revealed elevated hepatobiliary enzyme and serum tumor marker levels: total bilirubin, 21.2  mg/dL; aspartate aminotransferase, 83  IU/L; alanine aminotransferase, 103  IU/L; alkaline phosphatase, 1699  IU/L; γ-glutamyl transpeptidase, 926  IU/L, carcinoembryonic antigen, 5.9  ng/mL; carbohydrate antigen 19–9, 1966  ng/mL, and s-pancreas-1 antigen, 371.5  U/mL. Computed tomography revealed a low-density pancreatic head mass (diameter: 25 mm) and dilation of the common bile duct and pancreatic duct. The superior mesenteric vein (SMV) was located to the left of the superior mesenteric artery, and this phenomenon is known as the SMV rotation sign  series showed that contrast medium easily flowed through the intestines Fig. a, and thIntestinal malrotation is a congenital anomaly, with 80% of cases occurring in neonates . The remWang et al.  classifiWhen performing pancreaticoduodenectomy in patients with malrotation, factors such as intestine position, vascular anomalies, and reconstruction procedures should be considered. The Ladd procedure has traditionally been performed to treat malrotation, and it entails division of Ladd’s bands, detorsion of the volvulus, and reorientation of the small bowel in the right intestine and the colon in the left intestine . In caseKawano et al.  reportedThere were two complications of child’s reconstruction in our patient. First, there was a risk of detorsion of the volvulus given that the ascending colon was adjacent and positioned coaxially to the efferent loop. We prevented its torsion by additionally fixing the ascending colon to the abdominal wall. However, reconstruction may best be performed using the Cattell’s approach or Imanaga’s method, particularly in cases such as our case, because the ascending colon cannot be positioned coaxially to the efferent loop in these methods. Second, there was a space in the right upper abdomen that the efferent loop easily entered. We usually do not perform Braun anastomosis along with pancreaticoduodenectomy because we believe that Braun anastomosis is not necessary in all cases. In the present case, although Braun anastomosis was useful in the stabilization of the efferent loop, the large space in the upper abdomen could have been avoided if reconstruction was performed using Cattell’s approach or Imanaga’s method. These methods might be useful in the cases of nonrotation-type intestinal malrotation or some cases of reversed rotation-type intestinal malrotation; however, a definite recommendation cannot be given considering the different types of anomalies. In addition, Braun anastomosis or intestinal fixation should be considered.We presented a rare case of pancreaticoduodenectomy with malrotation following reoperation due to a complication of a surgical method. Coaxial positioning of intestinal parts and an upper abdominal space should be avoided in patients with intestinal malrotation who undergo gastrointestinal tract reconstruction.


We present a multisystemic approach involving diverse specialists of a rare disease. Bringing into the perspective the importance of multidisciplinary work and complete patient knowledge in order to an adequate clinical practice and patient outcome. We present a multisystemic approach involving diverse specialists of a rare disease. Bringing into the perspective the importance of multidisciplinary work and complete patient knowledge in order to an adequate clinical practice and patient outcome. We report a multisystemic approach combining study methods and collaboration with a radiologist for a 16‐year‐old male patient, diagnosed with a JNA measuring 2 × 3 × 5 cm with retarded diagnosis who received endoscopic surgery management.Juvenile nasopharyngeal angiofibroma (JNA) was first described in 1897 by Chelius as a polyp that took place during the adolescence and early adulthood stage. This type of tumor occurs only in 0.05% of all head and neck tumors, it is benign, and as the name implies, it is installed in the nasopharynx, more specifically, in the sphenoid process of the palatine bone with the horizontal wing of vomer and on the roof of the pterygoid process of the sphenoid bone. Histologically, it is formed by a cylindrical and pavement epithelium with the characteristics of the epithelium found in the nasopharynx, below is a loose cellular tissue, and the neoformation itself is constituted by fibrous and highly vascularized tissue. JNA is almost exclusive to men, so it is believed that there is a certain hormonal relationship with the etiology that causes it, but still has not been proven. Despite the histological benignity, its clinical behavior can be malignant due to the growth along natural holes and fissures related to its site of origin, causing bone and vascular destruction, accompanied by large hemorrhages. The vessels lack a muscular layer, which leads to excessive bleeding with minimal manipulation or trauma of the tumor in question.Its symptomatology is somewhat unspecific and generalized, characterized by a progressive nasal obstructive syndrome, usually unilateral, which ends up obstructing the choana. It is accompanied by night snoring, sleep apnea, headache, daytime oral breathing, and mild epistaxis. Eustachian tube dysfunction may occur if there is a nasopharyngeal component by the tumor. Symptoms usually become present 6 months to 1 year prior to diagnosis.On physical examination with anterior rhinoscopy, it usually reveals a reddish lobed mass located in the back of the nasal cavity and the cavum; preoperative diagnosis is based on clinical and imaging characteristics; incisional biopsy can lead to massive bleeding and is not recommended routinely.Within the diagnostic approach, computed tomography of the skull and sinuses is useful to show the extent of bone erosion due to the tumor, while magnetic resonance imaging is useful to delimit the margins of the tumor, especially if there is any intracranial extension.Once the diagnosis is made, the JNA can be staged with Fisch and Radkowski classification.Fisch classification:Stage I: Tumors limited to the nasal cavity and nasopharynx without bone destruction.Stage II: Tumors that invade the pterygomaxillary fossa and the sinuses with bone destruction.Stage III: Tumors that invade the infratemporal fossa, the orbit, and/or the parasellar region, but that maintain a lateral location with respect to the cavernous sinus.Stage IV: Tumors that invade the cavernous sinus, the area of the optic chiasma, and/or the pituitary fossa.Radkowski Staging:Type IA—Limited to nose or nasopharynx.Type IB—Extension into at least one paranasal sinus.Type IIA—Minimal extension into sphenopalatine foramen. Includes minimal part of medial pterygomaxillary fossa.Type IIB—Full occupation of pterygomaxillary fossa with Holman‐Miller sign. Lateral or anterior displacement of maxillary artery branches. May have superior extension with orbital bone erosion.Type IIC—Extension through pterygomaxillary fossa into cheek, temporal fossa, or posterior to pterygoids.Type IIIA—Skull base erosion with minimal intracranial extension.Type IIIB—Skull base erosion with extensive intracranial extension ± cavernous sinus.Regarding treatment before surgery, unilateral carotid angiography is necessary to evaluate the vascular supply of the angiofibroma and allow embolization of the main nutritional vessel, the internal maxillary artery. Occlusion of nutritional vessels reduces intraoperative bleeding, a major cause of morbidity, and may reduce tumor size. There are three main methods for embolization: transarterial, embolization with direct injection on the tumor, and a combination of the two. There is no preferred technique to perform an approach. It is customary to perform a more extensive approach such as lateral rhinotomy and degloving of the facial middle third. Open surgical methods are also used for locally advanced tumors, including those with intracranial involvement, optic nerve, or the internal carotid artery. The currently recommended treatment is surgical excision, which may or may not be accompanied by preoperative embolization.Recently, thanks to the advances in endoscopic surgery, this method has been used as a therapeutic option in appropriate cases, since in larger lesions, extrusion of the tumor is preferred.Radiation has been used in tumors that are not resectable or that are already advanced. It is a second option, although it is not needed in a large percentage of cases. Recurrence in this type of tumor does not respond to cell metastasis or dedifferentiation, but rather to the persistence of residual tissue in the surgical site or in sites that, due to their location, are not resectable during the first surgical period. The main sites where residual tissue is found are: pterygoid canal, pterygoid process, pterygopalatine foramen, nasopharynx, pterygopalatine fossa, sphenoid sinus, and infratemporal fossa (to name a few). As mentioned earlier, the presentation of this type of tumor brings a clinical challenge to many medical specialties, making it an entity that should be brought into the picture when making a differential diagnosis with symptoms such as progressive nasal obstructive syndrome, night snoring, sleep apnea, headache, daytime oral breathing, and mild epistaxis.Its clinical behavior makes the accurate diagnosis also as important, making the disease worse while time elapses from the symptoms’ onset to surgical treatment. Thanks to the modern devices to diagnose and treat this disease, it has been proven that the earlier this disease is diagnosed the prognosis is better in the long term.With this case report, we aim to inform and prepare most clinicians into knowing this clinical entity and understanding its presentation, management, and follow‐up and to reduce the time lost from symptom onset to surgical management.2A 16‐year‐old male patient with no important background history. His condition began 3 months ago, after a flu‐like illness, where he perceived a predominantly right nasal obstruction, with greater intensity at night, accompanied by hyposmia, occasional bloody rhinorrhea; as well as retronasal discharge, otic fullness, sometimes accompanied by mild epistaxis; later, right hemicranial headache, facial heaviness, sensation of retro‐ocular pressure is added to the movement; hyponasal voice and nighttime snoring. He reported having a cough and tiredness for a long time already. He also described having a feeling of instability to the movement, accompanied by nausea or sometimes vomiting for a month, and in the end a total nasal obstruction.He went to a general practitioner doctor and a gastroenterologist who only gave him symptomatic management. Afterwards was referred to an otoneurologist who rules out any neurological alteration causing the symptoms. While performing rhinoscopy, obstruction in the right nostril is found. The patient reported occasional mild epistaxis in the left nostril, without reporting it to be uncontrollable, as well as having the flu 1 week prior, which was managed symptomatically. After 6 months, he was referred to us for medical consultation. After physical examination, we arrived at the following findings:Using a 0mm 4mm endoscope support: Left septal deviation. Turbinate hypertrophy. A pinkish‐pearl right nasal tumor with a smooth, vascularized, ovoid surface that hangs from the back of the middle meatus and occupies the choana. Using a 70 degree endoscope retrogradely, nasopharyngeal occupation is seen incipiently. Right thick mucus is aspirated. Mouth, pharynx, and otoscopy without alterations. Hematic biometry and blood chemistry within normal values.2.1The differential diagnosis should be made with other nasopharyngeal tumors, such as choanal polyp, adenoid hyperplasia, nasopharyngeal tuberculosis, chordoma, craniopharyngioma, paraganglioma, teratoma, lymphomas, epidermoid carcinoma, adenoid cystic carcinoma, sarcomas, among others.While talking specifically of the differences between the similar diagnosis and JNA is beyond the scope of this investigation, we can assure that the major differentiator of almost all the pathologies mentioned before is the chronic behavior they have, compared to the JNA, which is more of an acute and aggressive proliferative tumor, with invasion to the entire nasal cavity.Simple skull tomography with contrast was made with the following findings:Tumor in the depth of the nasal passage on the right side, apparently hypervascular that is insinuated in the sphenoparietal fossa, is related to a nasoangiofibroma as the first diagnostic possibility, other findings associated with inflammatory changes in the maxillary sinuses and hypertrophy in the mucosa of the lower cortices.Hypervascular lesion of the choana and right nasopharynx with slight pterygomaxillary extension. As a finding, there is a 2‐cm arachnoid cyst. Figure .Nuclear magnetic resonance imaging of the skull and facial mass: Tumor in the right nose and nasopharynx, without 2x3x5 cm extensions. Arachnoid cyst in the posterior fossa. Slight septal deviation. Concluding as probable diagnosis a right JNA (Stage I‐II/IV).  was endoscopic surgical correction. This procedure is complicated but when performed correctly is an effective surgery, managing to limit recurrence with follow‐up to 24 months, attributing it to the advantageous multi‐angle view of the anatomical structures, enabling effective, atraumatic dissection using bipolar coagulation with gentle manipulation of the tumor. Among the decisive factors that were carried out correctly in our patient was a correct perforation of the bone invaded by the tumor, endonasal surgery combined with a preoperative embolization of the arterial supply of the tumor, thus achieving serious blood loss avoidance. In tumors that fall within the Radkowski IA‐IIB classification, endoscopic surgery remains an appropriate option. If the tumor extends laterally in the fossa infratemporalis or deep into the skull base, endoscopic surgery is no longer recommended. 4As a rare disease, the JNA accounts for only 0.05% of all head and neck tumors. As mentioned before, the symptoms of the clinical behavior of this type of tumors can go from nonspecific to mild to moderate symptoms, making it a clinical challenge to most clinicians to have the JNA as a differential diagnosis. Regarding the treatment, thanks to the advances in technology, endoscopic resection is the preferred method in almost all the tumors, making the endoscopic resection of JNA a difficult but effective operation, with the preoperative embolization of the artery supply an important step on preventing big amounts of blood loss. With a well‐executed surgical management, and a correct clinical follow‐up, the prognosis for this type of tumors is generally favorable.None declared.Author 1: Head of the project, leader of the literature revision. Author 2: Head surgeon of the endoscopic procedure. Author 3: Head neuroradiologist that made the supraselective embolization. Author 4: Assistant neuroradiologist that made the supraselective embolization. Author 5: Assistant surgeon of the endoscopic procedure. Author 6: Assistant of the literature revision.Subscription of the protocol for evaluation by the ABC medical center teaching department and ethics committee. Follow‐up of the four principles of bioethics distinguished by Beauchamp and Childress: nonmaleficience, beneficence, autonomy, and justice. Follow‐up to the Declaration of Helsinki. Follow‐up of the agreements for research in humans established by the Good Medical Practice protocol. Follow‐up of the official norm in medicine number 004 regarding the clinical file. Follow‐up of the official norm in medicine number 012 on the criteria for the execution of research projects for health in human beings.“Clinical case reports” reserves the right to contact your institution.I certify that my institution or the appropriate regional institution has approved the protocol for any investigation involving humans or animals and that all investigations were conducted in conformity with the protocol and the ethical and humane principles of research. The authors declare that no experiments were performed on humans or animals for this study.The authors have obtained written informed consent from the patients and/or subjects mentioned in the article. The corresponding author is in possession of this document.The authors declare that no patient data appear in this article.Supplementary MaterialClick here for additional data file.Supplementary MaterialClick here for additional data file.


Extracranial meningiomas are rare, accounting for 1–2% of all meningiomas with just published in the form of a case report or series. They are categorized as primary or secondary tumors. In this paper, we present a case of extracranial meningioma in the scalp skin, which is believed in Vietnam's first case.A 38 years old male with a history of brain epidural hematoma surgery in 1995 presented a subcutaneous protruding mass, which revealed approximately 3-centimeters from an incision scar in the left frontoparietal scalp. The tumor was firm, mobilized with contrast enhancement on magnetic resonance image. The complete surgical excision of the whole mass was performed, and histological examination revealed a WHO grade I meningioma.Epidemiology, classification, diagnosis, treatment as well as prognosis of extracranial meningioma were discussed. We also hypothesized the emergence of extracranial meningioma after an epidural hematoma surgery.Extracranial meningioma is quite rare, but it could be presented after a traumatic brain injury. Neurosurgery was the first choice, safe and effective treatment. They ar2A 38-year-old man in otherwise good general health presented with a large mass in the left frontoparietal scalp. The mass was progressively enlarged over three years. He gave a history of a prior surgery of bilateral extradural hematoma in 1995. He had an MRI examination in 2018 with the conclusion of lipoma in another medical center. As a result, he was considered to continue observation. Due to the enlargement of the mass, he decided to come to our center. Clinically, it is a firm, mobile, and subcutaneous tumor in the left frontal region, nearly 3 cm from his old scar. He received a preoperative MRI and other routine blood work. The Magnetic resonance imaging (MRI) with Gadolinium revealed a mass in the left frontoparietal scalp, which is well-circumscribed separated from the calvarium (30 mm at the greatest dimension) with slightly low signal intensity on T1-weighted imaging A and wit3Meningioma is a common tumor, occupying 24–35% of the central nervous system's tumors . AlthougExtracranial meningioma could be classified into two forms, which are primary and secondary tumors. Primary tumors are completely extracranial, and not associated with leptomeninges or intracranial mass, while secondary extradural meningiomas refer to intradural meningiomas with extradural extension and/or metastasis ,13. The Primary extracranial tumors are often found in many locations. Rushing at el. analyzed 146 cases of the primary extracranial tumor and reported that common locations, including the skin scalp (n = 59), ear and temporal bone n = 38), nasal cavity, and nasopharynx (n = 35) . It has , nasal cExtracranial meningiomas are rarely diagnosed in pre-operation stages because of their infrequency and atypical characteristics, especially when there are no images of intracranial tumors and the association between them . EnhanceThe first line and most successful outcome treatment for extracranial meningioma are complete or partial surgical excision. Preoperative imaging plays a crucial role in the surgery schedule. Hence, clinicians and radiologists have to discuss all the tumor aspects thoughtfully, the association to other involved lesions, and the degree of bony invasion and underlying dural infiltration. It is essential to expose broadly enough to resection of all complicated extracranial parts . Our casThe treatment strategy of extracranial meningioma is different between primary and secondary types. The primary tumors usually separate without intracranial involvement, so it is uncomplicated to resect completely. In terms of secondary, along the growth and infiltration of tumors exist many problems to accomplish the treatment .Histologically, meningiomas are usually divided into different subtypes; most tumors were meningothelial 77.4%) in the general location and 78% in the scalp. However, the other subtypes of grade I or grade II and III also sometimes present. It is no different regarding histology between intracranial and extracranial tumors ,7, which7.4% in tRushing et al. revealed that 26 recurrent cases in 110 patients are followed-up for at least ten years . They al4Extracranial meningiomas are relatively rare, but they could be presented after a traumatic brain injury. The anatomic site of involvements is most common at the scalp. Neurosurgery was the first choice, safe and effective treatment.The authors report no declarations of interest.The authors declared no funding for this research.The study was approved by the Research Ethics Committee of Hanoi Medical University. The procedures used in this study adhere to the tenets of the Declarations of Helsinki.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.Hung Dinh KIEU: assessed the protocol, summed up, revised manuscript.Hien Thi TRINH: followed up, wrote manuscript.Lanh Sy NGUYEN: data curation, followed up, revised manuscript.Hung Manh NGO: the main doctor conceived the original idea and operated the patients, wrote and revised manuscript.Not applicable.Hung Manh NGO, MD, PhD.Not commissioned, externally peer-reviewed.Data is available upon reasonable request and with permission of Viet Duc Hospital. No patient or author details are included in the figures.


Congenital absence of the portal vein (CAPV) is a rare malformation in which intestinal and splenic venous blood bypasses the liver and drains into systemic veins. CAPV is classified into two types based on the absence (type I) or presence (type II) of portal venous flow into the hepatic parenchyma and is associated with multiple other anomalies such as usually benign hepatic tumors. There have been only two case reports describing hepatocellular carcinoma (HCC) in patients with CAPV type II to date. We report the third such patient. A 50‐year‐old woman was referred to our hospital for management of a giant hepatic tumor. Contrast‐enhanced computed tomography (CECT) indicated a huge mass occupying the right lobe of the liver; the radiological diagnosis was HCC. CECT also demonstrated that the superior mesenteric vein (SMV) and the splenic vein (SpV) joined to form a shunt draining into the left renal vein and that a hypoplastic portal vein branched from the confluence of the SMV and SpV and drained into the liver, indicating that the CAPV was type II. Liver resection was successfully performed to treat the HCC, and the pathological diagnosis was well‐differentiated HCC. Seven months after the operation, a recurrent small HCC was detected and treated with radiofrequency ablation without complications. The patient has been carefully followed for 6 months to date without any evidence of further recurrence. Patients with CAPV are predisposed to developing HCC and require close surveillance. A 50‐year‐old woman was diagnosed with a giant hepatocellular carcinoma (HCC) that was associated with congenital absence of the portal vein (CAPV) type II. Patients with CAPV type II are predisposed to developing HCC and require close surveillance. Congenital absence of the portal vein (CAPV), which was first reported by John Abernethy in 1793 and is also known as an “Abernethy malformation,” is a rare anomaly of the splanchnic venous system where intestinal and splenic venous blood bypasses the liver and drains into systemic veins, such as the renal veins, hepatic veins, or inferior vena cava.Here, we describe a rare CAPV type II in a 50‐year‐old woman with a giant well‐differentiated hepatocellular carcinoma (HCC) that was successfully treated with liver resection. To our knowledge, this is the third reported incidence of CAPV type II that is associated with HCC development.A 50‐year‐old woman was referred to our hospital for management of a giant hepatic tumor. She had been well until 1 month prior, at which time she experienced a sensation of abdominal distension and noticed a loss in appetite. She underwent abdominal ultrasonography at another hospital, whereupon a giant hepatic mass was discovered in the right lobe. The patient was then referred to our hospital for further investigation and treatment of the hepatic tumor. Physical examination demonstrated hepatomegaly; her laboratory test results at the initial visit were as follows: liver function tests showed evidence of nonicteric cholestasis with mild transaminase enzyme level elevation. Screening for viral hepatitis yielded negative results. Levels of the liver‐specific tumor marker “protein induced by vitamin K absence‐II” were highly elevated. No hyperammonemia was detected, and no episodes or signs of encephalopathy were noted. Abdominal ultrasonography showed a giant hypoechoic mass measuring 139 × 120 mm in the right lobe of the liver. Contrast‐enhanced computed tomography (CECT) demonstrated a huge mass that occupied the right lobe of the liver and showed inhomogeneous hyperenhancement in the arterial phase, as well as uniform isoenhancement in the portal venous phase  ‐enhanced magnetic resonance imaging (MRI); this lesion was successfully treated with radiofrequency ablation. She has been carefully followed for 6 months to date without any evidence of further recurrences.CAPV is a rare condition in which the intestinal and splenic venous drainage routes bypass the liver and deposit directly into systemic veins through various venous shunts.CAPV is classified into two types based on whether the portal vein is present.CAPV can have various clinical presentations ranging from no symptoms to systemic manifestations such as encephalopathy, hepatopulmonary syndrome, or symptoms related to hepatic masses. CAPV is often associated with metabolic disorders such as hypergalactosemia and hyperammonemia,et al. reported that patients with CAPV type I have a significantly higher prevalence of hepatic tumors and other anomalies than do those with type II.et al. reported a 5‐year‐old girl who had multiple liver tumors associated with CAPV type II and had experienced learning disabilities. The liver tumors were not treated, and after she died at the age of 19 years, postmortem examination indicated that the tumors were multifocal HCC and multiple adenomas.et al. reported an HCC within an adenoma in a 42‐year‐old woman with CAPV type II.CAPV often causes hepatic tumors such as focal nodular hyperplasia, nodular regenerative hyperplasia, hemangioma, and HCC. In most cases, hepatic tumors in patients with CAPV are benign.et al. reported that 20% of HCCs arise in noncirrhotic livers, where their risk factors are non‐alcoholic fatty liver disease, chronic viral hepatitis, inherited diseases such as alpha‐1 antitrypsin deficiency, and intake of genotoxic substances such as aflatoxin B1.It remains unclear how CAPV causes HCC. Previous investigators have hypothesized that the absence of portal venous flow compensatively increases the hepatic arterial flow, which might provide a permissive environment for tumor development,et al. suggested that the risk of HCC among patients with CAPV may be similar to that among alcoholics or patients with hepatitis C‐induced cirrhosis.The lifetime risk for HCC development in patients with CAPV is yet to be determined owing to the rarity of the disease and lack of longitudinal studies. Konstas In conclusion, ours is the third reported patient with CAPV type II to develop HCC, for which she underwent a radical resection. A postoperative recurrent lesion in the remnant liver was successfully treated with radiofrequency ablation. Taken together, the data suggested that patients with CAPV type II are predisposed to HCC and therefore require close surveillance.


Background: Intramedullary disk extrusions has rarely been described in veterinary medicine, more especially in cats, with only two cases are reported in the veterinary literature. Diagnosis may be difficult, even though clinical presentation and imaging studies, such as MRI or CT, can present specific features. Treatment and prognosis are not clearly described.Case presentation: A 10-year-old domestic shorthair female cat was evaluated for a 12 h-history of peracute-onset of paraparesis with flaccid tail and urinary and fecal incontinence. The patellar reflexes were normal, the pelvic flexor reflexes were decreased (more markedly on the right limb) and the perianal reflex was absent. The tail was flaccid, without nociception. Abdominal palpation revealed a small urinary bladder, easily expressed. Manipulation of the lumbar vertebral column elicited marked pain. Neurological examination was consistent with a L7-caudal segments lesion. A lumbosacral MRI and CT evaluations were performed and revealed a focal intramedullary hemorrhagic lesion, with an associated vertical linear tract communicating with the L5-L6 intervertebral disk space, and a suspected intramedullary focus of mineralization. These imaging findings were highly suggestive of an L5-L6 intramedullary disk extrusion. A dorsal L5-L6 laminectomy confirmed the presence of intramedullary degenerative nucleus pulposus fragments, which were surgically removed. Rapid and progressive neurological improvement was observed post-surgery. At the 1-year follow-up, right plantigrade stance and mild paraparesis were still noticed, but jumps and voluntary tail movements were observed. Occasional urinary and fecal incontinence episodes remained.Conclusions: This is the first feline case report of an intramedullary disk herniation with long-term follow-up available. Clinical description, CT and High-Field MRI findings, surgical procedure and histological results are reported, and help describing the characteristics of this rare non-compressive category of peracute intervertebral disk extrusion. Surgical management may be considered in feline cases of intramedullary disk herniation and may be associated with a good outcome. Intervertebral disk disease rarely occurs in cats, representing ~5% of feline spinal cord disorders –3. MajorA 10-year-old female spayed domestic shorthair cat was referred for peracute-onset of ambulatory paraparesis, more pronounced on the right, with a flaccid tail and fecal and urinary incontinence for the past 12 h. The cat was in the garden for a few hours and there were no known traumatic event reported. The patient had no history of spinal pain or neurological deficit prior to this episode. On physical examination, the cat was moderately overweight and the perineal hair were soiled with urine. The caudal abdominal palpation revealed a small urinary bladder, easily expressed. Neurological examination demonstrated normal thoracic limbs, ambulatory asymmetric paraparesis and mild pelvic ataxia, more pronounced on the right side. Pelvic postural reactions were delayed. Patellar reflexes and pain sensation were normal, and pelvic flexor reflexes were reduced, more markedly on the right side. Lumbosacral hyperalgesia was observed during palpation of vertebral column. The tail was flaccid without nociception. Anal tone and perineal reflex were absent. These neurologic signs were consistent with a cauda-equina syndrome with a neurolocalization caudal to the L6 spinal cord segment, with sciatic/L7, sacral and caudal spinal cord segments or nerve roots involvement.MRI of the lumbosacrocaudal region was performed using a high-field scannerAfter intravenous administration of GadodiamideA dorsal laminectomy was performed over the L5-L6 intervertebral space. The epaxial musculature was normal and there was no evidence of extruded disk material or hemorrhage within the dorsal extradural space. The dorsal dural tube was intact and enlarged, and a subdural mixed discolored and hemorrhagic focal spinal cord lesion was identified. A dorsal linear durotomy was performed, and the spinal cord appeared focally swollen with yellow-reddish color noted at this level. Parts of hematoma were gently removed and solid white material mixed with hemorrhage was observed , within The histologic analysis on the surgically removed intramedullary material showed a fibrino-hemorrhagic material associated with fragments of degenerated cartilaginous tissue, independent of vascular structures, consistent with degenerated nucleus pulposus disk material , which cThe lumbar pain of the cat improved shortly after the procedure. The urinary incontinence was assumed to be related to urinary bladder and striated urethral sphincter dysfunction secondary to neuronal lesion of the pelvic and pudendal nerves. Betanechol  and manual bladder expression were initiated to improve voiding, by stimulating bladder emptying and avoiding deleterious bladder distension. Voluntary micturitions remained rare 1-week postoperatively and manual bladder expression became more difficult, with moderate bladder distension, despite increased Betanechol dosage . PrazosinIntervertebral disk disease (IVDD) is uncommon in cats , 2 and mAs in our case, in the context of peracute onset of asymmetric myelopathy with minimal extradural involvement, three hypotheses should be prioritized: ischemic myelopathy , and peracute non-compressive disk extrusions (PNDE) represented by ANNPE or intramedullary intervertebral disk extrusion (IIVDE) , 8. DiffConsidering previously published data on feline cases, IIVDE appear to more commonly originate from the caudal lumbar IVD spaces, while IM has been reported more frequently in the cervical spinal cord , 7, 18. Due to the paucity of reported IIVDE cases, there is currently no treatment consensus or known prognosis available in veterinary medicine. In the seven canine cases published, four of the affected dogs underwent surgery, and half of them had a good outcome. For the two reported cats with IIVDE, one was euthanized at the time of diagnosis , and theTo the authors' knowledge, the prognostic significance of intramedullary hemorrhage for the recovery of dogs and cats with disk herniation has not been studied –23. HoweTo the authors' knowledge, this is the first report of a confirmed feline case of intramedullary disk herniation with a successful outcome, after surgical treatment and long-term follow-up. It illustrates the clinical, CT and MRI findings contributing to the high suspicion of an unusual type of peracute intervertebral disk herniation in old animals. The presence of suspected intramedullary mineralized disk material associated with an hemorrhagic spinal cord lesion and vertical intramedullary linear tract overlying an IVD space, are highly suggestive of IIVDE. Because of the suspected mineralized feature of the extruded nucleus pulposus fragments, thus not easily degradable, and its consequences on the intraspinal environment, surgical excision might be recommended over conservative treatment. A favorable outcome, with rapid and progressive improvement might be expected, although irreversible neurologic deficits may persist.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The authors declare that this work involved the use of client-owned animal only, and followed established international recognized high standards (Best practice) of individual veterinary clinical patient care. Therefore, ethical approval form a committee was not needed. Written informed consent was obtained from the owners for the participation of their animals in this study.MD and J-LT were responsible for the case management and MRI studies. MD was responsible for writing the manuscript. PD was involved in the CT studies. IV performed the surgery. DP performed the histopathological evaluation. All authors read, corrected, and approved the final manuscript.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.


This case highlights the need for both tissue biopsy for diagnosis in suspected CNS malignancy and comprehensive immune profiling for accurate subclassification. This case highlights the need for both tissue biopsy for diagnosis in suspected CNS malignancy and comprehensive immune profiling for accurate subclassification. A computed tomography (CT) scan revealed a hypodense left temporal lobe mass with surrounding vasogenic edema; initially, concern was high for a metastatic tumor. A biopsy was performed, and pathology demonstrated a high‐grade diffuse large B‐cell lymphoma with double expression of both CD5 and CD10.Central nervous system lymphoma (CNS) is a rare disease; primary CNS lymphoma accounts for 2 to 3% of all brain tumors. Median patient age for primary CNS lymphoma is 56 years with a slight male predominance.2The patient is a 68‐year‐old woman with a history of hypothyroidism and vertigo presented with an onset of seizures. A few months prior to presentation, the patient's family had noticed she was having progressive deterioration of balance and the development of ataxia, weakness, and expressive aphasia. She denied any weight loss or constitutional signs. There was no known history of immunodeficiency or medications that would impair immunocompetence. Physical examination was remarkable for a positive Babinski sign, bilateral lower extremity weakness, and difficulty with speech. Complete blood count (CBC) was normal, and CT and magnetic resonance imaging (MRI) were performed. MRI showed a 1.4‐cm. mass in the left temporal lobe with surrounding vasogenic edema raising concern for possible metastatic neoplasm. Chest abdomen and pelvis CT and MRI and positron emission tomography (PET) were performed as a part of a complete evaluation for a primary non‐CNS malignancy. There was no evidence of a primary lesion to confirm the impression of metastatic disease. Because of the location of the lesion, a biopsy was not initially indicated and she received 2 doses of CyberKnife radiosurgery. However, the tumor did not respond to treatment and repeat MRI showed evidence of a new lesion in the left frontal region. Neurosurgery was consulted and subsequently performed a left temporal craniotomy with resection of the left temporal and left frontal masses. The histopathology showed a lymphoid malignancy with large pleomorphic cells, marked nuclear enlargement, prominent nucleoli, and prominent starry sky appearance Figures , 4 and 53Mature B‐cell neoplasms usually express CD5 or CD10 or neither in addition to expressing a lineage defining marker such as CD19 or CD20. Correct assignment of a diagnostic category to lymphoid malignancy usually involves determination of the immunophenotype of the malignant cells by either one of two diagnostic modalities, immunohistochemistry, or flow cytometry.Simultaneous CD5+ and CD10+ neoplasms are extremely rare and are estimated to make up only 0.4% of all B‐cell lymphomas.None declared.All authors approved the final manuscript as submitted and agreed to be accountable for all aspects of the work.This case report used only de‐identified archival case material; no additional institutional approval was required.


Jejunal diverticulosis is a rare phenomenon often identified either incidentally on imaging or intra-operatively. Complications of jejunal diverticulosis are associated with high rates of mortality. For this reason, it remains important that this pathology is considered amongst differentials for an acute abdomen. A 78-year old gentleman presented with a short history of generalized lower abdominal pain. Computer tomography scan revealed a large inflammatory abscess relating to a perforated jejunal diverticulum. The patient was taken to theatre where he underwent small bowel resection with primary anastomosis. Early cross sectional imaging is vital to allow early diagnosis and prompt management of this pathology. Small bowel resection with primary anastomosis was associated with an excellent clinical outcome. Jejunal diverticulosis is a rare phenomenon with an estimated annual prevalence of between 0.3 and 2.3% . AcquireA 78-year old male presented to the Emergency Department with a 24 h history of sudden onset, generalized lower abdominal pain. Associated symptoms included fever and nausea without vomitus. His past medical history was significant for anal squamous cell carcinoma for which he had been treated successfully with chemo- and radio-therapy 8 years previously. He also previously underwent a laparoscopic cholecystectomy for management of biliary colic. He had no other medical history of note and was taking no regular oral medications.Clinical examination revealed generalized abdominal tenderness, without objective evidence of peritonism. His temperature was 38.3°C in the emergency room and he was tachycardic at 103. His laboratory markers revealed an elevated white cell count of 10.8 with 92% neutrophilia. His serum C-reactive protein (CRP) was also elevated at 92.3, all other laboratory markers were within normal limits.A computer tomography (CT) scan of his abdomen and pelvis with oral and intravenous contrast was performed on Day 1 of admission . CT reveThe decision was made to take the patient to the operating room where an exploratory laparotomy was performed through an upper midline incision. The inflammatory mass was identified 40 cm from the duodeno-jejunal flexure. The mass was ~4 cm in greatest diameter and located on the mesenteric border of the jejunal segment, covered entirely by omentum a and b. The histopathology report confirmed the presence of a small bowel diverticulum with associated abscess formation and suppuration.Diverticulosis is a relatively common disorder, most commonly affecting the colon, however, other sites including the duodenum, jejunum and ileum may also be affected. The causes of small bowel diverticulae specifically are unclear; however, previous studies have postulated that abnormal motor innervation may result in an increase in intraluminal pressure leading to the formation of false diverticulae . Unlike Where the majority of cases of jejunal diverticulosis will be picked up incidentally on imaging or intra-operatively, an estimated 10–30% of cases will present with a complication such as diverticulitis, bleeding, obstruction or perforation . Few casNon-operative management of uncomplicated small bowel diverticulitis with antibiotic therapy and bowel rest has previously been reported . HoweverPerforated jejunal diverticulitis is a rare pathology associated with a high mortality rate. We recommend that it is considered within the differential diagnosis of an acute abdomen, particularly in an elderly population. Timely cross-sectional imaging is crucial to allow diagnosis and effective management. Small bowel resection with primary anastomosis was again associated with an excellent outcome.None declared.


Mesenteric cysts have various histological forms, including mesenteric cystadenomas and borderline cystic neoplasms. Primary cystadenocarcinoma of the mesentery is extremely rare; therefore, the clinical and radiological features of this tumor have not been fully elucidated.18F-fluorodeoxyglucose (FDG)-positron emission tomography/computed tomography (PET/CT) revealed mottled mild FDG uptake in the cyst wall and intense FDG uptake in several mural nodules. The cystic mass with the descending colon was completely removed. Pathological examination of the specimens revealed various histologic patterns of adenocarcinoma, including mucin production in the mural nodules. We eventually diagnosed a primary cystadenocarcinoma arising from the mesentery of the descending colon.A 50-year-old Japanese woman had a complaint of a left-sided abdominal distention. Enhanced computed tomography and magnetic resonance imaging revealed a unilocular cystic lesion measuring approximately 10 cm located in the left side of the abdomen. Malignancy should be suspected in mesenteric or retroperitoneal cystic tumors with high FDG uptake, and complete resection should be performed with adequate margins. Mesenteric cysts are relatively rare and have various histological forms –3. Mesen18F-fluorodeoxyglucose (FDG)-PET/CT revealed mottled mild FDG uptake in the whole cyst wall and intense FDG uptake in the mural nodules with standardized uptake value (SUV)-max of 16.15, and no abnormal FDG uptake in other organs  level of 21.4 ng/mL  and carbohydrate antigen 19-9 (CA19-9) level of 804.0 U/mL . Other laboratory results were normal except for a low hemoglobin level of 10.4 g/dL. Abdominal US revealed a tumor located in the left abdomen with a maximum diameter of about 9 cm. The cyst wall was about 4 mm thick and relatively uniform, with no septa or nodules visible within the cyst. Enhanced computed tomography (CT) and magnetic resonance imaging (MRI) of the abdomen revealed a 10.2 × 9.1 × 9.3-cm-sized unilocular cystic lesion with maximum 12-mm-sized nodules located on the left side of the abdomen  at 100 mg/kg body weight per day for 8 courses (4 weeks of administration and 2 weeks of discontinuation). Approximately 1 year after the surgery, abdominal CT revealed no signs of disease recurrence, and serum CEA and CA19-9 levels had returned to normal.Mesenteric cysts are lesions that occur in or near the mesentery; they are not parasitic or dermoid and are not derived from normally placed retroperitoneal organs . MesenteThus, we performed a search of studies published in English between 1933 and August 2020 and reviewed the clinicopathologic features of cystadenomas, borderline malignant cystic neoplasms, and cystadenocarcinomas. Cases of retroperitoneal cysts and adenocarcinomas without cystic lesions were excluded. After excluding cases with insufficient information, a total of 14 cases of primary benign cystic neoplasms (cystadenomas) and 6 cases of borderline malignant cystic neoplasms were identified , 8–23. WThe median ages of patients with benign cystadenoma, borderline malignant cystic neoplasm, and cystadenocarcinoma of the mesentery were 43 (14–80) years, 38 (32–54) years, and 41 years (23–72 years), respectively. There was no significant difference in age between patients with benign and malignant tumors. These tumors were more common in women, and only 3 cases have been reported in men , 22, 29.Regarding preoperative imaging modality, in our literature review, US was more frequently used before 2000, whereas CT was used more frequently than after 2000, and MRI findings are gradually being reported. CT and MRI can confirm the positional relationship between the cystic tumor and adjacent organs, which is useful for estimating the origin. Although it has been speculated that the amount of soft tissue and internal septations may suggest malignancy, as with cystic tumors of the ovary, in this review, there were only two cases each with solid components in cystadenoma and cystadenocarcinoma. There were no reports containing information on internal septation , 31. To In the current case, we considered the possibility of a malignant cystic tumor based on the high serum tumor marker levels and high uptake of FDG in mural nodules on FDG-PET/CT. FDG-PET/CT provides important physiologic information on altered tissue metabolism that forms the basis for detection and diagnosis of carcinoma. Compared to normal cells, malignant cells develop significant alterations in carbohydrate metabolism . AdditioThe recommended treatment for cystadenomas is surgical resection to obtain a histopathological diagnosis and manage tumor-related symptoms , 28, 40.We report the first case of primary cystadenocarcinoma of the mesentery in which FDG-PET/CT was performed preoperatively. If high FDG is accumulated in the mesenteric or retroperitoneal cystic tumor, we should consider the possibility of malignancy and perform complete resection with adequate margins.


Salivary gland tumors (SGT) comprise 3% of all head and neck tumors, are mostly benign, and arise frequently in the parotid gland. Pleomorphic adenoma (PA) is the commonest SGT, representing 60-70% of all benign parotid tumors. Clinically, parotid PA presents as irregular, lobulated, asymptomatic, slow-growing preauricular mass, involving both superficial and deep lobes, and could grow to gigantic proportions. Histologically, PA has epithelial and mesenchymal elements in chondromyxoid matrix and is managed surgically. Based on a review of 43 cases reported in English literature since 1995, giant parotid PA is reported as large as 35 cm (diameter) and 7.3 kg (resected weight). Although rare, 10 cases of malignant transformation were reported in the review. Surgical management included extracapsular dissection (ECD), superficial parotidectomy, and total parotidectomy for benign tumors, and adjuvant radiation or chemotherapy for malignant tumors. We further present the case of a 36-year-old healthy male with slow-growing and asymptomatic giant parotid PA, of 4-year duration. The patient presented with firm, lobulated preauricular swelling, provisionally diagnosed as PA based on radiographic and cytological findings. The tumor was resected through ECD, and the patient had uneventful postoperative recovery and a 7-year recurrence-free follow-up period. Histological examination revealed epimyoepithelial proliferation punctuated by chondromyxoid areas, with extensive squamous metaplasia and keratin cysts. To the best of knowledge from indexed literature, giant parotid PA is rarely reported in Saudi Arabia. In addition to its rarity, this case is reported for its benign nature despite atypical histological presentation, successful surgical management without complications, and long-term recurrence-free follow-up. Based on this report, clinicians must be aware of atypical histological presentations associated with PA and plan suitable surgical management and follow-up to avoid morbidity. Nevertheless, attempts must be made to diagnose and manage these lesions at an early stage and before they reach gigantic proportions. Neoplastic lesions of the salivary glands are uncommon and comprise less than 3% of all reported head and neck tumors , 2. NearAs the name suggests, PA is histologically categorized as a benign mixed (pleomorphic) tumor of ductal and myoepithelial cell origin. Owing to the pluripotential nature of myoepithelial cells, the tumor is composed of epithelial and fibrous, myxoid, and cartilaginous mesenchymal elements surrounded by a pseudocapsule, with occasional squamous metaplasia , 4, 6. WAlthough uncommon, giant pleomorphic adenomas of the parotid gland have been reported. An electronic search of the English-language articles through the Medline, Scopus, and Google Scholar databases revealed 43 reports of giant parotid PA since 1995 –32, withA 36-year-old healthy male patient reported to the oral and maxillofacial surgery outpatient clinic at the College of Dentistry and Dental University Hospital, King Saud University, in October 2012. The patient sought medical attention for a slow-growing, painless swelling in the right preauricular region. History revealed that the patient noticed the swelling almost 4 years ago, and since then, it had gradually increased in size with no obvious symptoms or changes to the overlying skin. Upon interviewing, the patient reported no relevant medical or surgical history and mentioned fear of surgery and absence of discomfort as reasons for delaying medical consultation, in spite of an unesthetic facial appearance.Clinical examination revealed a firm, nontender, nodular, and mobile swelling with apparently normal overlying skin. The swelling extended superoinferiorly from the level of the external ear to the lower border of the mandible and anteroposteriorly from the angle of the mouth to the posterior border of the mandible. There was no lymph node involvement or facial nerve deficit . PreoperSurgical removal of the right parotid SGT under general anesthesia was planned and explained to the patient. Following informed consent, the lesion was excised completely through ECD, with preservation of all branches of the facial nerve. The right parotid gland was approached using a cervically extended preauricular skin incision. A clearly discernible plane of dissection around the tumor was used for dissecting the tumor mass, without any iatrogenic damage to the facial nerve branches. Owing to the long-standing nature, multiple small feeder vessels had to be ligated circumferentially around the tumor to achieve hemostasis. The intraoperative period was unremarkable, and the patient did not require any blood transfusions . The resHistopathological examination of the excised specimen gave a gross appearance of a partially encapsulated mass containing myoepithelial and ductal proliferation. There was marked stromal hyalinization, squamous metaplasia, and keratinization. Some epithelial islands exhibited papillary configuration, along with large cysts and inflammation. Chondromyxoid changes and fibrosis were evident throughout the tumor. Certain foci of tumor islands were seen approaching and breaking through it. Hematoxylin and eosin (H&E) stained sections revealed a partially encapsulated tumor with variable histopathological features and focal effacement of the fibrous capsule. The tumor typically showed epithelial/myoepithelial proliferation punctuated by chondromyxoid areas. Aggregates of plasmacytoid myoepithelial cells as well as ducal differentiation surrounded by clear myoepithelial cells were evident. Based on the above findings, a final diagnosis of benign PA was reached. While the tumor sections showed no evidence of malignant change, there was extensive squamous metaplasia and keratin cyst formation, which were atypical for PA . The patAt 6 weeks postsurgery, the patient had unremarkable wound healing without any neurological deficit of the facial nerve . As of Dth gestational week, it is anatomically located bilaterally between the mastoid process of temporal bone and ramus of the mandible. The terminal branches of the facial nerve are an important anatomic landmark which divide the parotid gland into its superficial and deep lobes [The parotid gland is the largest salivary gland with an average weight ranging from 0.015 to 0.021 kg and measuring approximately 5.8 × 3.4 cm in the craniocaudal and ventrodorsal dimensions, respectively. Being the first salivary gland to develop in utero, during the 6ep lobes . AlthougPleomorphic adenoma is the commonest mixed SGT arising in the parotid gland, and several cases have been reported in the literature. Although there are no specific physical criteria outlined in the literature to classify giant parotid PA, the earliest recorded case report dates back to 1863 . In thisA literature search was conducted to review giant parotid PA cases reported in Medline, Scopus, and Google Scholar databases. The search strategy involved a combination of search keywords including “GIANT”, “PAROTID GLAND”, and “PLEOMORPHIC ADENOMA”, based on which 288 articles were identified from the three databases . Reviewing their abstracts, articles published in English only were selected based on them reporting a case or series of cases of giant parotid PA, including clinical, radiographic, histological, and surgical outcomes. Twenty-six articles published in English language were identified , 8–32 sin = 30, 69.8%), the age at clinical presentation and surgery ranged from 21 to 92 years. The asymptomatic, slow-growing nature of pleomorphic adenoma was evidenced by the fact that the duration from the first observation of lesion to reporting for treatment varied from 5 to 35 years. Demographically, there were more females  than males, and all reported cases were unilateral, with the right side  affected more than the left. In terms of clinical dimension, the tumors ranged from 3 to 5 cm in diameter [n = 29, 70.7%) giving rise to the clinical presentation of a large preauricular mass. Nevertheless, when the deep lobe of parotid and parapharyngeal spaces were involved by the tumor, patients presented with an intraoral swelling leading to soft palate displacement, difficulty in swallowing and breathing, and obstructive sleep apnea [Although majority of the reported cases were in patients aged 45 years or older  [The treatment of PA irrespective of their size, severity, or malignant potential is only by surgery. Based on our literature review, giant PA involving the superficial lobe of parotid was managed either by SP or ECD , 31, 32.e (≥45%) . This lee (≥45%)  reportede (≥45%) . Moreovee (≥45%) .Several surgical approaches to remove PA and other benign parotid gland tumors involving the deep lobe and extending to the lateral pharyngeal and parapharyngeal spaces have been reported . These iIn addition to the long-standing nature of the tumor and its large size, the currently reported case also presented with atypical histological findings. While the histological picture was predominantly coherent with that ofPA characterized by epithelial tissue dispersed in a chondroid and myxoid matrix , there wMalignant transformation of PA is reported at around 12% and comprises nearly 4% of all SGT . AccordiIn the present review, PA with malignant transformation was treated surgically, followed by radiotherapy for locoregional disease and chemotherapy for distant metastases. Within postoperative follow-up periods ranging from 6 months to 5 years, only one case of recurrent benign PA was reported in the review . Among tGiant parotid PA is rare, and to the best of our knowledge from indexed literature, this is one of the very few cases reported in this geographical region (Saudi Arabia). Apart from its rarity, the present case is being reported for its long-standing clinical presentation, successful surgical management through ECD without any postoperative complications or facial nerve deficit, atypical histological presentation, and long-term recurrence-free follow-up. In addition to complete tumor removal, ECD helped preserve the integrity of facial nerve branches. Interestingly, the present case of giant parotid PA was benign in spite of associated squamous metaplasia and keratin cyst formation identified histologically in the resected specimen. Nevertheless, this mandated a long-term follow-up of the patient. Therefore, based on this case report, clinicians must be aware of atypical histological presentation associated with parotid PA and plan suitable surgical management and follow-up, to achieve complete tumor removal and avoid morbidity. Lastly, attempts must be made to diagnose and manage PA at an early stage and before they reach gigantic proportions, through better public health care initiatives and creating awareness among all physicians and patients.