Breast, lung, prostate, thyroid, and kidney carcinomas are the primary tumors that are known to have bony metastasis. Hepatocellular carcinoma (HCC) frequently involves the lung and lymph nodes and less commonly the osseous system. Numbness/persistent pain in the distribution of the trigeminal nerve is more likely a neuropathy. The causes are idiopathic(common), unintentional injury to the trigeminal nerve during surgery or trauma, blood vessel pressing the trigeminal nerve, tumor infiltration, multiple sclerosis, and stroke. Unresolved facial pains after conventional treatment should prompt additional investigation to rule out other causes. In this case, we report a trigeminal neuropathy of rare cause, which is a solitary metastasis from an inactive HCC involving the osseous structures. Breast, lung, prostate, thyroid, and kidney carcinomas are the primary tumors that are known to have bony metastasis . HepatocA 69-year-old lady, who is on treatment for diabetes, hypothyroidism, and hypertension, was detected to have HCC three years ago, a solitary space-occupying lesion in segment six of the liver. The patient underwent successful transarterial chemoembolization (TACE) and was cancer-free for the next three years.The patient presented with a 10-day duration of right hemifacial pain that was of electric shock-like quality occurring frequently and precipitated by chewing food, wind blowing, and water splashing on the cheek, characteristic of trigeminal neuralgia. Cranial nerve examination at that time was normal. She was started on oxcarbazepine 300mg twice daily and the non-contrast MRI of the Brain was reported to be normal. Two weeks later, she presented with worsening hemifacial pain and doubling of vision. She described the pain as burning, associated with tinnitus, numbness of the right maxillary and mandibular divisions of the trigeminal nerve. Clinical examination revealed a right lateral rectus palsy, absent corneal reflex, and reduced pin sensation of the V2-V3 divisions of the right trigeminal nerve. The remainder of her neurological examination was normal.Given the above findings, an alternate diagnosis was suspected. A contrast MRI brain Figures -1C reveaDiagnostic considerations included metastasis, primary aggressive lesion/ malignancy involving the middle skull base. Hence she underwent whole-body PET CT Figures -2C, whicThe patient underwent an endoscopic skull base biopsy. Via a transnasal approach, the mass of 2.9x2.4x3.4 cm was located just lateral to the paraclival and petrous portion of the carotid artery. The entire mass and a small fungal ball in the posterior ethmoid sinus were removed.The histopathological analysis showed fragments of a neoplasm composed of trabeculae and glands lined by cells with eosinophilic nuclei and pleomorphic nuclei with nucleoli and occasional inclusions. Immunohistochemistry analysis Figures -3E showeThe patient improved symptomatically and is currently symptom-free.According to the International Classification of Headache Disorders, typical trigeminal neuralgia is characterized by recurrent paroxysms of unilateral pain in the distribution of one or more divisions of the trigeminal nerve, which does not radiate beyond the distribution, lasts for seconds to two minutes, the severe intensity with an electric shock-like quality and precipitated by innocuous stimuli . This waThe differential diagnosis to consider in the setting of multiple cranial nerve palsy in the background of HCC are meningeal carcinomatosis, meningeal infections, and skull base metastasis, although radiological and spinal fluid analysis would help in differentiating most cases.Incidence of HCC metastasis accounts for about 5%, most often involving the lungs and the regional lymph nodes . InvolveHsieh et al.  found 68Trivedi et al.  reportedThe uniqueness about our patient is that she had a cancer-free liver and presented with symptoms suggestive of trigeminal neuropathy. Our patient neither had a liver dysfunction at presentation nor at any time during her admission.Unresolved trigeminal neuralgia despite medical management, needs detailed evaluation and skull base metastasis from tumors, isolated or not, should be ruled out. Early investigation and management improve patients' quality of life.


Soft tissue sarcomas are group of malignant tumours arising from extra skeletal mesenchymal tissue. Presenting a patient with swelling over the posterior aspect of left arm for 6 months, gradually increasing in size for four months and rapidly increasing in size for last 2 months and not associated with pain. Peripheral pulses felt. Ultrasound showed large solid cystic intramuscular lesion arising from triceps muscle and Trucut biopsy showed poorly differentiated malignancy. Magnetic resonance imaging (MRI) left arm impression was large lobulated solid cystic space occupying lesion in triceps muscle, possibility of neoplastic lesion. Here the patient underwent wide local excision and reconstruction procedure followed by Radiotherapy, chemotherapy and regular follow up. Soft tissue sarcomas are group of malignant tumours arising from extra skeletal mesenchymal tissues. Most of soft tissue sarcomas are located in lower extremities and next to it 25% . LocatedA 28-year-old male patient came with complaints of swelling over the posterior aspect of left arm for 6 months. The swelling was initially small in size insidious in onset, gradual increasing in size for four months and rapidly increasing in size for last 2 months and not associated with pain. No history of trauma, loss of weight and any other specific complaints. Nil significant family history, No similar complaints in family members, No history of any other swellings in the body. No co morbidities for this patient, No similar complaints in past.Clinical findings: physical examination revealed , followed by the furthest point (23%) (with inclination for the shoulder), and the head and neck (9%). Further, it will in general be found near the huge joints of the limits, particularly the knee and lower leg.Surgery: the gold standard treatments of sarcomas are complete surgical resection. The primary goal of the procedure is enbloc resection of tumour through normal tissue planes and should include the previous incision biopsy scar. As the synovial sarcoma usually encases the neurovascular bundles, injury to these structures can be prevented by adequate exposure and meticulous dissection. For recurrent tumours, surgery is the gold standard option. As majority of patients undergo limb sparing surgery and amputation is still considered as a final resort, amputation rate is still elevated [elevated . For patelevated . Carefulelevated . Synoviaelevated . AnotherSynovial sarcomas are the most common malignant tumours usually less than 5cm in size. They usually present early and distant metastasis is rare. Diagnosis made depending on the lesion which is calcified not anywhere near the joint in a young individual. Cross-sectional imaging studies helps in careful dissection. The small size of tumour and its slow growing nature attributes to misdiagnosis. Since synovial sarcomas are high grade tumours, adequate margin clearance is essential to prevent recurrences.


When colorectal cancer presents with liver metastasis, hepatic resection remains the most important factor in prolonging survival, and new paradigms have been proposed to augment resectability. An adequate liver remnant and vascularisation are the only limits in complex liver resection, and parenchyma-sparing surgery is a strategy for minimising the complications, preserving liver function, and allowing patients to undergo further liver resection. The laparoscopic approach represents a new challenge, especially when lesions are located in the superior or posterior part of the liver. We discuss the case of an 81-year-old patient with a single synchronous liver metastasis involving the left hepatic vein and leaning into the middle hepatic vein at the common trunk, where we performed a simultaneous laparoscopic colonic resection with a left sectionectomy extended to segment 4a. The strategic approach to the Arantius ligament by joining the left and middle hepatic vein allowed us to avoid a major liver hepatectomy, preserve the liver parenchyma, reduce complications, enhance patient recovery, and perform the entire procedure by laparoscopy. Our example suggests that the Arantius approach to the left hepatic vein and the common trunk could be a feasible approach to consider in laparoscopic surgery for lesions located in their proximity. Colorectal cancer represents one of the most prevalent oncologic diseases and the third most deadly and fourth most diagnosed cancer worldwide . About 2The case is an 81-year-old patient with non-insulin-dependent diabetes, hypertension, and previous intervention for cisto-rectal prolapse. After a positive faecal blood occult test, the patient underwent colonoscopy with evidence of an ulcerated lesion of the right hepatic flexure. A CT scan raised doubt regarding a lesion involving the left hepatic vein in proximity to the bifurcation of the common hepatic trunk . An MRI The intervention started with the patient in a supine position and abducted legs, to allow simultaneous liver resection and colonic resection. Intraoperative ultrasound showed evidence of a lesion involving the anterior part of the left hepatic vein, including the upper portion of segment 4a, and lying along the last part of the middle hepatic vein up to the common trunk bifurcation. In consideration of the patient’s characteristics and indication for simultaneous resection, a left sectionectomy with en bloc anatomical subsegmentectomy 4a was chosen. The intervention started with the surgeon positioned between the legs. The pedicles of segments 2 and 3 were isolated and ligated, and partial dissection between segments 3 and 4b was performed A.Using ultrasound, we marked the liver surface along the middle hepatic vein up to the margin of the liver metastases between segments 4a and 8. The dissection was continued along the Arantius ligament, and the inferior margin of the common hepatic trunk was exposed B. The diThe subsequent section of the right margin of the left hepatic vein was performed B using aThe next part of the intervention was conducted with the surgeon positioned to the left of the patient. The colonoscopy of the marked area evidenced a lesion of the middle transverse colon. The middle colic artery was dissected until its origin at the pancreas, and the right colon was mobilised. Intrabdominal resection was performed with side-to-side colonic anastomosis. The pieces were extracted by Pfannenstiel incision.Further haemostatic control and drain placement were performed. The total time of intervention was 365 min, and the total blood loss was estimated to be 500 mL. Histopathology revealed T3N0 colon cancer with 20 negative lymph nodes and liver parenchyma with a metastasis of 3 cm × 2 cm with R1 vascular contact. The postoperative course was surgically uneventful, with a liquid diet introduced at j1 and a low-fibre diet in the following days. During the hospitalisation, she had direct contact with a SARS-CoV-2 inpatient, and for the hospital protocol, she was isolated in a separate room and remained asymptomatic; a thoracic CT scan was performed, and she was discharged after 10 postoperative days. The patient is asymptomatic and on a course of chemotherapy.The resection of liver metastases for colorectal cancer remains the gold standard treatment, combined with chemotherapy. This combination could increase the five-year overall survival from 31%  to 66% , even wiLaparoscopic resection is an emerging strategy in liver resections. Combining the concepts of open surgery and laparoscopy is essential for proceeding in performing liver surgery, with the application of parenchyma-sparing strategy and precise dissection having the advantage of reducing postoperative morbidity in an enhanced recovery program. The Arantius approach to the left hepatic vein and the common trunk could be a feasible approach to consider in laparoscopic surgery for lesions located in their proximity.


Although one of the serious manifestations of advanced malignancies is pericardial involvement, pericardial involvement of lymphoma is extremely rare. We present a case of a 6‐year‐old girl arriving at the hospital with dyspnea and pleuritic chest pain, which is eventually diagnosed with massive pericardial effusion due to mediastinal non‐Hodgkin lymphoma. Because non‐Hodgkin lymphomas with mediastinal disease at presentation have a natural tendency for rapid dissemination and worse prognosis when invading the pericardium; hence, minimal delay in diagnosis is essential. She was mildly agitated and could not lie down due to pain and orthopnea; She was also febrile during this period. In her past medical history, she is the first sibling, was born term, with no previous hospitalization. Her parents reported intermittent fever for 3 months before admission. There was no close contact with a suspicious COVID‐19 patient.Upon physical examination, general appearance was good and the child was mentally oriented. Lung auscultation was clear but a region of decreased sounds was detected on the lower right side, heart sounds were muffled, and the peripheral pulses were normal at the time. The blood pressure reading was 100/65, heart rate was 120, and respiratory rate was 30. Abdominal examination revealed hepatomegaly.Initial paraclinical studies included chest radiograph which showed a mediastinal widening Figure  and 12‐lFurther evaluations were performed based on the primary findings, which included echocardiography showing significant pericardial effusion, and thorax computed tomography (CT) scan revealing a mediastinal mass accompanied by pleural and pericardial effusion Figures  and 4.To rule out thymoma, lymphoma and also germ cell tumors, a core needle biopsy (CNB) from the mass was obtained, which was in favor of mediastinal non‐Hodgkin lymphoma , although the bone marrow aspiration and biopsy were normal. The morphologic features and IHC study results were consistent with T‐cell lymphoblastic lymphoma Figure . Chemoth3One of the frequent and serious manifestations of advanced malignancies is pericardial involvement, which includes various degrees such as pericarditis, pericardial effusion, pericardial tamponade, and constrictive pericarditis.Lymphoma is a hematological malignancy that emerges from the clonal proliferation of lymphocytes at different maturation stages. It presents with fatigue, night sweats, enlarged painless lymph nodes, and weight loss. Lymphoma is classified into two major subgroups: Hodgkin (10%) and non‐Hodgkin lymphoma (90%).Chen et al compared pediatric and adult lymphomas involving the mediastinum and reported that pediatric patients had a higher incidence of T‐LBL/T‐ALL, prevalence of dyspnea, stage IV tumors frequency, and relative tumor diameter, compared to adults. They were also more likely to be male.Cardiac and pericardial involvement of lymphoma is extremely rare. It accounts for 0.5% of cardiac involvement and 1% of all extranodal non‐Hodgkin lymphomas. It is more common in high‐grade lymphomas, particularly double‐hit/triple‐hit subtypes, and shows a poor prognosis, as in other malignancies.On the contrary, malignancy involving the pericardium is usually a late secondary feature.A chest x‐ray identifies an anterior mediastinal mass mostly when it is very large (bulky disease) and produces mediastinal widening. It demonstrates tumor bulk and pleural effusion. Chest computed tomography (CT) scans are examined for precise tumor location, presence of necrosis, pulmonary parenchymal involvement, chest wall invasion, and more assessment of pleuropericardial effusion.Currently, there is an emerging evidence on the underlying molecular mechanisms of malignancies, which may improve the outcome of patients through introducing new drugs targeting tumor‐specific pathways.4Although pericardial effusion is rare as the first demonstration of pediatric mediastinal lymphoma, it is important to take this differential diagnosis into consideration. Because non‐Hodgkin lymphomas with mediastinal disease at presentation have a natural tendency for rapid dissemination and worse prognosis when invading the pericardium; hence, minimal delay in diagnosis is essential to avoid incorrect initial management and initiate the true approach as soon as possible.All authors declare that they have no conflict of interest.B.A., Z.B., Z. Sh., and P.D. contributed in conception, design, and drafting of the manuscript. Z. Sh. contributed in data collection. B.A., Z.B., and Z. Sh. contributed in drafting of the manuscript and P.D. supervised the study. All authors approved the final version for submission.The study protocol was approved by the Ethics Committee of Mashhad University of Medical Sciences and was conducted according to the Declaration of Helsinki.Undersigned informed consent form was obtained from patient prior to the enrollment.


Rhizomelic chondrodysplasia punctata (RCDP) is a devastating medical condition for patients and their families. It is a rare peroxisomal autosomal recessive disorder. It was recognized clinically with skeletal abnormalities and intellectual disabilities mainly due to plasmalogen deficiency. Here, we report a case of a 16-day-old girl who was referred to King Abdulaziz Medical City Jeddah, Saudi Arabia because of dysmorphic features. Her growth parameters were below the 3rd centile with short proximal long bones and multiple joint contractures in the extremities. The radiographs showed rhizomelic and shortening of both humeri and femurs. Moreover, punctate ossification was identified in the upper spine, humeri around the shoulders, and femurs around the knees. We observed other classical features, and the genetic testing confirmed the diagnosis of RCDP type 3. Although RCDP is a rare condition, it is a distressing burden necessitating early diagnosis and a holistic approach for management. PEX7, GNPAT, and AGPS genes, respectively. Genetic testing is needed to differentiate between these three RCDP subtypes because they are indistinguishable clinically. Recently, two more subtypes were described, type 4 (RCDP4) and type 5 (RCDP5), attributed to the FAR1 and PEX5 genes, respectively [AGPS gene. Of these variants, nine were missense, and two were frameshift [AGPS. To date, this variant is the third reported pathogenic frameshift variant in AGPS.There are plenty of inheritable diseases worldwide affecting the lives of many individuals in their infancy, childhood, and even adulthood; one of which is rhizomelic chondrodysplasia punctata (RCDP). RCDP is a rare peroxisomal disorder that is autosomal recessive. This disorder intervenes with the normal development of many parts of the body. The key features of this condition are skeletal abnormalities, distinguishing facial features, intellectual disability, and respiratory problems. It is a rare inherited disorder with a prevalence of only one in 100,000 individuals . RCDP isameshift . Due to A 16-day-old baby girl patient was referred to King Abdulaziz Medical City Jeddah, Saudi Arabia, due to dysmorphic features. She was a product of normal spontaneous vaginal delivery at 35 weeks of gestation with a good Appearance, Pulse, Grimace, Activity, and Respiration (APGAR) score, and a birth weight of 2.2 Kg. The antenatal history was unremarkable for the baby girl. The mother is 35 years old, and the father is 36 years old. They are second-degree relatives, and this is their first child. There is a positive family history of a child with severe short stature.On physical examination, her length was 44 cm (below 3rd centile), her weight was 1.9 kg (below 3rd centile), and her head circumference was 29 cm (below 3rd centile).oC.The case vital signs were as follows: pulse, 150 beat/min; blood pressure, 97/60 mmHg; respiratory rate, 36 breath/min; and temperature, 37.2Physical examination revealed facial dysmorphism in form of low set ears, long philtrum, and upslanting palpebral fissures of both eyes. In addition, there was a short proximal bone in the upper limb with contracture in both elbows and talipes equinovarus. She had normal first, second heart sounds, and a pansystolic murmur. She was hypotonic; other examinations and an overall systemic review were unremarkable. Laboratory investigations for the patient are illustrated in Table A skeletal survey showed bilateral rhizomelic and shortening of the humeri and femurs. Punctate ossification was identified at the proximal femur, distal femur, and around the knees. Moreover, punctate ossification is noted at the proximal humeri bilaterally around the shoulder joint and along the upper spine Figure .The hip ultrasound showed femoral head dislocation on both sides Figure .Furthermore, a chest x-ray revealed cardiomegaly with prominent lungs markings. Radiography did not show evidence of air space opacities, pleural effusion, and pneumothorax Figure .In addition, an echocardiogram confirmed the diagnosis of tetralogy of Fallot.AGPS gene, which led to a premature stop codon. This confirms the diagnosis of RCDP3. Furthermore, the genetic testing results found that both parents are heterozygous carriers for RCDP3. Therefore, upon reviewing the baby's condition and the associated medical anomalies by the multidisciplinary team, parents were counseled about the natural history of the condition and its poor prognosis.Genetic testing with whole-exome sequencing (WES) revealed homozygous 4-bp deletion variant c.1639_1642del p. (Arg547*) in the Chondrodysplasia punctata (CDP) is a rare genetic disorder that includes disorders of skeletal dysplasia, which are heterogeneous in their clinical features and mode of inheritance. There are three types of CDP based on the mode of inheritance: arylsulfatase E deficiency CDPX1), an X-linked recessive mode of inheritance; Conradi-Hünermann-Happle syndrome (CDPX2), an X-linked dominant disorder; and RCDP, which is autosomal recessively inherited , an X-li,11. DiagThe case reported in this article showed severe short stature. All her growth parameters were below the 3rd centile, which is consistent with one case of the same condition by Itzkovitz B et al. They reported a four-year-old girl ("patient 6" in the report) who fell below 3rd centile in all growth parameters . AnotherAGPS. Numerous key features of RCDP were present in this case, some of which are bilateral shortening of the humeri and femurs, and bilateral femoral head subluxation.RCDP is a rare proximal autosomal recessive disorder. It is characterized by classical features, such as short proximal limb bones, congenital cataracts, growth and mental retardation, and dysmorphic facial features. This article reports a case of RCDP type 3, which was caused by a pathogenic frameshift variant in


Enteric duplication is a congenital disease that occurs throughout the entire gastrointestinal tract. Although it may sometimes cause intestinal volvulus, a few reports have described cases of enteric duplication twisted on itself. We experienced a rare pediatric case of long-segment tubular ileal duplication showing torsion. Torsion of enteric duplication is extremely rare. We herein report a pediatric case showing unusual torsion of ileal duplication requiring emergency surgery.A 3-year-old boy presented with abdominal pain and vomiting. Contrast-enhanced computed tomography (CT) revealed a cystic luminal structure with a blind end and fluid collection in the pelvic cavity. CT also showed no findings of ileus or intestinal dilatation except for a cystic luminal structure. The preoperative diagnosis was torsion of Meckel’s diverticulum. The patient underwent emergent explorative diagnostic laparoscopy. As a result, a necrotic luminal structure and bloody ascites were recognized, and small-scale laparotomy was performed. Long-segment ileal duplication was recognized. The long-segment tubular ileal duplication shared the anti-mesenteric side of the intestinal wall along one-third of its length. The residual two-thirds of its length was free from the ileum and its blind end was twisted in a manner that looked similar to Meckel’s diverticulum. Normal ileum and the duplication, including the twisted necrotic portion, were resected, and ileal anastomosis was performed. The postoperative course was uneventful. A pathological examination confirmed the definitive diagnosis of enteric duplication.We reported the unusual presentation of intestinal duplication mimicking torsion of Meckel’s diverticulum. Enteric duplication shows various clinical symptoms and presentations. We must understand that the classification of digestive enteric duplication is diverse with a variety of associated clinical symptoms. Enteric duplication is a congenital disease that occurs throughout the entire gastrointestinal tract. Although it may cause intestinal volvulus, there are few reports of enteric duplication itself being twisted.We herein report a pediatric case in which the diverticulum-like site of tubular intestinal duplication was twisted and necrotic. Torsion of enteric duplication is extremely rare, and this case is reported along with a review of the literature.A 3-year-old boy presented with complaints of acute abdominal pain. The patient had visited the primary care pediatric clinic the day after the onset and been diagnosed with viral gastroenteritis and received medication conservatively. However, the abdominal pain persisted, so the patient was referred by his primary care physician to the previous local hospital. An abdominal X-ray did not show dilation of intestine and findings of ileus Fig. a. The paWe reviewed the contrast-enhanced CT findings again. There were no findings of ileus or intestinal dilatation except for the cystic luminal structure. The blood supply of the entire gastrointestinal tract was also preserved, except for the cystic luminal structure. Based on the clinical symptoms and these imaging findings, the preoperative diagnosis was torsion of Meckel’s diverticulum.We decided to perform exploratory laparoscopic inspection to obtain a definitive diagnosis. Exploratory laparoscopy revealed that the dark-colored twisted cystic luminal structure was located at the right lower abdomen, and its root seemed to be continuous with the small intestine Fig. a. After Small-scale laparotomy using the umbilical trocar wound was then performed to clarify the pathophysiological mechanism. The entire small intestine was extracted through the wound retractor. A long segment of tubular ileal duplication was recognized at 120 cm from the oral side of the ileocecal region  a well-developed smooth muscle coat; 2) mucosal lining found within some portion of the alimentary tract; and (3) contiguity to any segment of the alimentary tract [ mucosal The broadly accepted classification of intestinal duplication consists of five types: intestinal membrane type, intestinal wall cyst type, extra-intestinal cyst type, extra-intestinal tubular type and solitary type . The preOur case shared characteristics with the long-segment tubular type and was located at the anti-mesenteric side, and two-thirds of it was also free from the adjacent bowel. The free side had a blind end and the complete structure of the intestinal wall, so the duplication might have had some degree of motility. As a result, torsion would have been able to be induced that mimicked torsion of Meckel’s diverticulum around the root on the free side without the involvement of the mesentery.We experienced a rare case in which a portion of intestinal duplication located on the anti-mesenteric side protruded with a diverticulum shape and had become twisted. We should consider enteric duplication when encountering cases of cystic lesions presenting with twisting mimicking torsion of Meckel’s diverticulum. Enteric duplication can present with a variety of clinical symptoms.


Heterotopic pancreas (HP) is an uncommon congenital abnormality in the developmental process of the pancreas, with gastrointestinal heterotopic pancreas (GHP) being the most common HP. The clinical manifestations of GHP may have variable patterns of presentation, dictated by both the anatomic location and the functional ability of the lesion. The most common imaging modality in detecting GHP is computed tomography (CT), while gastrointestinal barium fluoroscopy, endoscopic ultrasonography, and magnetic resonance imaging (MRI) are also applied. The density and enhancement patterns of GHP are consistent with histological classifications. GHP with a predominantly acinar tissue component manifests homogeneous and marked enhancement on CT images, whereas a predominantly ductal GHP presents heterogeneous and mild enhancement. On MRI, the appearance and signal intensity of GHP were paralleled to the normal pancreas on all sequences and were characterized by T1-weighted high signal and early marked enhancement. This article provides a comprehensive review of the histopathology, clinical manifestations, imaging features of various modalities, and differential diagnosis of GHP. It is hoped that this review will improve clinicians’ knowledge of GHP and aid in accurate preoperative diagnosis, thereby reducing the misdiagnosis rate. Gastrointestinal heterotopic pancreas is an uncommon congenital abnormality in the developmental process of the pancreas.The clinical manifestations of gastrointestinal heterotopic pancreas may have variable presentations decided by both the anatomic location and the functional ability of the lesion.Some distinct imaging features of various modalities may support the diagnosis of gastrointestinal heterotopic pancreas.Gastrointestinal heterotopic pancreas and other gastrointestinal subepithelial tumors could be differentiated by non-invasive imaging.Heterotopic pancreas (HP) is a kind of congenital abnormality occurring during the developmental process of the normal pancreas, also known as ectopic pancreas, aberrant or accessory pancreas, pancreatic choristoma, or adenomyoma –4. The tGastrointestinal heterotopic pancreas (GHP) is essentially analogous to the normal pancreas in terms of gross and histological specimen. GHP can be classified as a subepithelial lesion, which is defined as a mass covered by normal mucosa . GHP appPatients with GHP are often asymptomatic, but some patients may illicit significant clinical manifestations. Characterization of GHP symptomatic lesions is slightly difficult due to the relative infrequency of this diagnosis and the variability and non-specificity in presentation, often leading to misidentification and suboptimal management in many cases. GHP is susceptible to the same pathological conditions as the normal pancreas, so its complications are like those of the normal pancreas, such as pancreatitis, pancreatic pseudocysts, even malignancy. In severe cases, it may cause acute abdomen such as gastrointestinal hemorrhage (hematemesis or melena), or even intestinal obstruction and intussusception Fig. . Some coEsophageal lesions often bring about progressive dysphagia or epigastric pain, occasionally leading to gastroesophageal reflux and hiccup, and rarely hematemesis . SecondaIn addition to the anatomic location, histological location within the layers of the visceral wall may also influence symptomatic presentation. GHP most commonly occurs between the submucosa and lamina propria but can be found in all layers of the visceral wall . SubmucoAlthough location can often explain symptomatic presentation, it does not fully explain why some lesions are symptomatic and others found in similar locations are not. The remaining factor can be explained by the function of the lesion and the ability to perform the normal exocrine and endocrine functions of the pancreas. A histopathological evaluation by Heinrich in 1909 showed differences in the composition of these lesions which were later revised by Fuentes in 1973 , 19. The, which reported that over 54% of patients with GHP were misdiagnosed preoperatively [Accurate identification of GHP during diagnostic tests is important in determining the appropriate management. Unfortunately, many patients are misdiagnosed at the time of surgery or are thought to have other pathological changes. This often influences surgical decisions making and can lead to a more extensive resection than would otherwise be required. This was evident in the study by Zhang et al.ratively . Many leratively . Duodenaratively . Esophagratively .In the past, the common imaging modality for GHP was gastrointestinal barium fluoroscopy. With the development of imaging technology, endoscopic ultrasonography (EUS), contrast-enhanced computed tomography (CT), and magnetic resonance imaging (MRI) are increasingly used for the detection and follow-up of GHP. Currently, the most applied imaging modality for GHP description is contrast-enhanced CT.On barium examination, GHP has a typical appearance of an intramural tumor with a broad base and a smooth surface , 24. An Endoscopically, GHP shows as an endoluminal submucosal mass , 25. StuGHP often presents as an intramural oval mass with indistinct margins on CT coronal images , 30. GHPThe appearance and signal intensity of GHP resembles that of the normal pancreas in all MRI sequences , 35. GHPGHP is still prone to misdiagnosis in clinical practice. Identifying the imaging features of GHP can help to distinguish it from tumors and thus avoid unnecessary surgery. The main differential diagnostic tumors of GHP are other gastrointestinal subepithelial tumors, including gastrointestinal stromal tumor (GIST), gastroduodenal glomus tumor (GGT), schwannomas, leiomyomas, and so on. The differential diagnosis of the complications of GHP pancreatitis includes groove pancreatitis, myoadenomatosis, pancreatic hamartoma of the duodenum, pancreatic adenocarcinoma involving the biliary tree, and so on –39. The Contrast-enhanced CT is the most applied imaging modality for differentiating GHP from other subepithelial tumors. Kim et al.  initiallNevertheless, previous studies didn’t make usage of receiver operating characteristic analysis to determine the diagnostic performance of contrast-enhanced CT. Later, studies filled the gap by addressing the shortcoming, though lack of pathologic to radiologic correlation , 32, 41.At present, studies of EUS bring into play a role in describing and figuring the endosonographic features of GHP , 42. FewMRI has a superior ability in identifying GHP based on its multi-parameters imaging and multiple sequences, especially on MRCP and T2-weighted images. The only study assessed MRI with diffusion-weighted imaging findings of GHP and other gastrointestinal submucosal tumors, suggesting that qualitative and quantitative parameters could be helpful in differentiation diagnosis . PreoperGIST is the most common submucosal tumor that originates from interstitial Cajal cells in the muscular layer of the gastrointestinal wall and can occur anywhere along the digestive tract. The most common location of GST is the fundus or body . Most ofThe typical appearance of primary GIST is a submucosal mass with irregular contour and heterogeneous enhancement, which may be accompanied by mucosal ulceration, necrosis, cystic degeneration, hemorrhage, calcification, and presentation of enlarged draining vessels . Nearly Gastrointestinal schwannoma is thought to originate as an over proliferation of Schwann cells within the autonomic nervous system of the gastrointestinal tract and often manifests as a prominent lymphatic cuff-like mass . GastroiGastrointestinal leiomyoma is benign, not requiring surgery unless obstruction or compression symptoms. The most common location of gastrointestinal leiomyoma is the gastroesophageal junction, while in the stomach it is usually located in the cardia . GastroiGastrointestinal neuroendocrine tumor is a rare and slow-growing tumor derived from intestinal villous-like cells . More thCharacteristic CT signs help to distinguish GHP from other submucosal lesions in the gastrointestinal tract that are seem similar oval or flat in shape. GHP is mostly a small, solitary lesion located distal to the gastric antrum with a large LD/SD ratio, and its density and enhancement pattern resemble that of the normal pancreas. GIST is mostly a mixed-growing, abundant bloody supply mass in the submucosa of the gastrointestinal tract, commonly with mucosal ulceration and amorphous calcification. Gastrointestinal schwannoma generally shows as a round, homogeneous, hypodense lesion in the stomach. Gastrointestinal leiomyoma customarily presents as a small, hypodense, mild enhancement, and intraluminal-growing lesion in the esophagogastric junction. Neuroendocrine tumor of the gastrointestinal tract varies according to its classification and location and thus imaging performance. Familiarity with their respective imaging manifestations can assist clinicians in making a diagnosis before treatment.GHP is a variant congenital of development, which is rarely detected on prospective imaging, while most cases are discovered at surgery or autopsy. Comprehension of the common sites of occurrence and imaging features of GHP is key aspects of accurate preoperative diagnosis. Clinician and radiologist should also be aware of the histoembryological and pathological features of GHP, mastering with clinical manifestations and related complications of GHP, and clear about the diagnostic points of differentiation between GHP and other gastrointestinal submucosal tumors, to avoid as much as possible underdiagnosis and misdiagnosis of such disease during work.


Case Presentation. A 47-year-old man who was referred by a private practitioner for a CBCT scan in reference to a proliferative soft-tissue growth in the hard palate. The growth was mild and tender and there was Grade III mobility in relation to all the maxillary teeth. Panoramic radiograph taken previously had revealed evidence of alveolar bone loss in relation to the maxillary teeth and was inconclusive of any other findings. The CBCT scan revealed evidence of moth-eaten appearance of maxilla with destruction of medial and lateral walls and floor of maxillary sinus. There was also evidence of involvement of right eustachian tube, ethmoidal wall, and nasopalatine canal. An intraosseous malignancy of the palate was suspected, and a total maxillectomy was performed. The tissue sample was sent for histopathological assessment wherein changes diagnostic for polymorphous low-grade adenocarcinoma of the palate were observed.  Polymorphous low-grade adenocarcinoma (PLGA) is a slow growing malignant tumor of minor salivary glands and is generally of indolent nature. However, according to the most recent WHO Classification of Salivary Gland Tumors (2017), the cancer is classified as Polymorphous AdenoCarcinoma (PAC). PAC presents as a less aggressive tumor, though it could on rare occasions demonstrate distant metastasis.  PAC is a distinct, yet commonly occurring, minor salivary gland tumor with varied clinical and histologic appearance. This case report highlights the importance of CBCT in diagnosing the intraosseous involvement of such tumors which can help shed some light in enhancing our knowledge about the minor salivary gland malignancies like PAC. Polymorphous low-grade adenocarcinoma (PLGA) is a slow growing malignant tumor of minor salivary glands and is generally of indolent nature. However, according to the most recent WHO Classification of Salivary Gland Tumors 2017), the cancer is classified as Polymorphous AdenoCarcinoma (PAC) 7, the ca categoriDue to its architecturally diverse structure, clinically indolent behavior, and cytological uniformity, PAC is thought to be a unique entity , 6. BeinAlthough the third most commonly presenting malignant salivary gland tumor, our knowledge about PAC remains restricted to published case reports. Also, the architectural diversity associated with the name can be confusing for histopathological evaluation and can potentially be a diagnostic dilemma. Salivary gland neoplasms can be aggressive, and most of their clinical features often overlap; hence, a detailed examination and clinical investigation should be carried out in order to reach a definitive conclusion. Radiology is an important diagnostic tool for salivary gland pathology. It helps to determine the extent of involvement of bony structures, which can provide foresight into the treatment planning and provide a valuable tool in assessing the clinical picture. Cone Beam Computed Tomography (CBCT) is useful in the assessment of osseous structures and can help the clinician to determine the aggressive nature of the tumor as well as bony involvement due to a clear image of structures that are highly contrasted, and in comparison to most 2-D imaging methods, CBCT can provide a three-dimensional image of structures of the head and neck . The folA 47-year-old man was referred to the Department of Oral Medicine & Radiology with a history of soft tissue growth in the palate for the past two months. The growth was sudden in onset, growing rapidly in size over the next two months and localized to the palate. The patient reported no pain during the onset of growth. However, for the past one week, the patient reported mild pain. On clinical examination, there was a diffuse, proliferative soft tissue growth of the maxilla extending from the marginal gingiva in the palatal aspect of the anterior tooth and involving the entire surface of the hard palate up to the level of the hard and soft palate junction along with erythema of attached gingiva in relation to posterior teeth palatally and buccally as well as Grade III mobility of all the anterior and posterior maxillary teeth Figures  and 2. TThe referring private practitioner had advised a panoramic radiograph earlier which revealed severe bone loss in relation to maxillary teeth suggestive of generalized periodontitis and was inconclusive for any other relevant findings except for a root stump in the lower right quadrant .The patient was advised for a CBCT scan which revealed a moth-eaten appearance of the palate noticed bilaterally with involvement of nasopalatine canal anteriorly and external resorption of roots in relation to all the maxillary teeth present, with evidence of involvement of anterior and middle ethmoidal sinus, eustachian tube, and nasopharynx on the right side. Based on CBCT and clinical findings, a preliminary diagnosis of intraosseous malignancy involving the palate was suspected Figures –9. The pThe surgical procedure consisted of visualization of hard and soft palate and incision extending laterally around the maxillary tuberosity exposing the posterior border. Following exposure of the maxillary tuberosity, attachment to the soft palate and hard palate was divided. After soft tissue dissection up to the extension of the tumor, the extent of the bone which was tailored to the primary tumor including the lateral wall of the orbit and zygoma to be resected was marked. Following bone guttering, the bony resection was carried out using a chisel and mallet through the frontal process of the maxilla and lacrimal bone. Bleeding encountered during this procedure from the greater palatine artery and the branches of internal maxillary artery was controlled using pressure packing followed by ligation to achieve haemostasis. After mobilizing the segment, final osteotomy was done to separate the maxillary tuberosity from the pterygoid plates. Before removal, thorough clinical examination of the specimen was done to determine the adequacy of the tumor Figures .The tissue sample was sent for histopathological examination which revealed an unencapsulated tumor within the underlying connective tissue approximating the surface epithelium, with a varied pattern of arrangement of tumor cells that included solid nests, strands, ducts, and tubular and papillary patterns and was lined by 1-2 layers of oval/cuboidal cells along with a peripheral layer of flat cells suggestive of polymorphous low-grade adenocarcinoma of the palate Figures , and 14.Clinically, PAC presents as a firm, well-circumscribed, painless, and slow growing mass mostly covered by nonulcerated mucosa resembling a benign neoplasm and could be fixed to underlying structures, eroding and infiltrating the underlying bone, and is even present with perivascular and perineural invasion. Metastasis when reported is mostly confined to the regional nodes while spread to distant sites is a rarity and so is the transformation of this low-grade entity into a high-grade one .Immunohistochemistry (IHC) has been utilized in a number of case series to distinguish between PAC and ACC, but there is a considerable overlap. Past studies conducted regarding the expression of ki-67 have consistently reported a very low labelling index (LI) in PAC which can assist in differentiating it from adenoid cystic carcinoma (ACC) –14. In aThe differential diagnosis for PAC includes pleomorphic adenoma and adenoid cystic carcinoma. PAC presents with a mucoid to the hyalinized matrix in comparison to pleomorphic adenoma which presents with a chondromyxoid matrix. Also, when compared to pleomorphic adenoma, PAC presents with perineural invasion. Another differentiating feature between the two is that PAC stains positive for S-100 and epithelial membrane antigen (EMA) whereas pleomorphic adenoma stains positive for glial fibrillary acidic protein (GFAP). When compared to adenoid cystic carcinoma, staining is positive for p-53, ki-67, bcl-2, and CD117. Perineural invasion is a feature of both PAC and adenoid cystic carcinoma, but the presence of the targetoid arrangement of perineural invasion is characteristic of PAC .In the published literature, radiographic imaging of PAC has been mainly restricted to CT imaging since they provide a better soft-tissue contrast when compared to CBCT . HoweverAdjuvant radiotherapy has been indicated in cases where there is metastasis of cervical lymph nodes. A wide excision has been shown to minimize the rate of recurrence in cases of PAC, and radical excision has been suggested for the recurrent cases .Minor salivary gland malignancy is an uncommon occurrence in daily practice. However, being the third most common minor salivary gland malignancy, it is necessary for the diagnostician to be well-versed with the clinical, histopathological, and radiographic features of PGLA. Although the knowledge available on this entity is limited, it is necessary to be able to segregate it from the miscellaneous tumors of minor salivary glands in order to provide appropriate treatment for the patient. Due to its tendency for recurrence, it is important to regularly follow-up the patient posttreatment. The importance of radiography especially 3D imaging in the form of CBCT in dental practice is highlighted in this case report as we have noticed that even though salivary gland malignancies usually limit themselves to soft tissue involvement, there are cases wherein extensive involvement of bone with a varied radiological presentation can be observed.PAC is a distinct, yet commonly occurring, minor salivary gland tumor with varied clinical and histologic appearance. This case report highlights the importance of CBCT in diagnosing the intraosseous involvement of such tumors which can help shed some light in enhancing our knowledge about the minor salivary gland malignancies like PAC.


Colorectal cancer progresses without any symptoms early on or those clinical symptoms are very discrete and so are undetected for long periods of time. Complicated colorectal carcinoma has several symptoms, the most common being bleeding and obstruction. Occasionally, it will cause perforation, which carries a worse prognosis. It is rare for a carcinoma colon to present as abscess of the anterior abdominal wall that forms as a result of direct invasion and perforation of the colon by cancer. We hereby report an unusual case of perforated colon carcinoma presented as an abscess infiltrating the abdominal wall. Colorectal cancer is the third most common cancer and the second leading cause of death in the USA. Approximately 140 000 new cases are diagnosed each year, and almost 55 000 people die from the disease each year . CarcinoA 63-year-old female patient with diabetes mellitus presented to the emergency department with the complaints of a painful swelling over the right lower abdominal pain for 4 days. It was insidious in onset and was associated with fever, nausea and reduced appetite. She did not have jaundice and hematemesis. However, she gave history of intermittent constipation with occasional bleeding per rectum (PR), abdominal discomfort and sense of incomplete evacuation after defecation for past 6 months. She had undergone an open laparoscopic cholecystectomy 1 year ago for symptomatic gall stone disease and her postoperative period was uneventful. She also gave history of significant weight loss for past 4 months.On her physical examination, she was pale and malnourished. She was afebrile, tachycardia and tachypnea were present, and she was in acute distress due to pain. On examination of the abdomen, there was a tender fluctuant area over the right lower abdomen, predominantly in the subcutaneous and muscular plane. It measured around 6 × 8 cm with the erythema of the skin and surrounding induration . There wAfter resuscitation with intravenous fluids, administration of broad-spectrum intravenous antibiotics and intravenous analgesics, a contrast-enhanced computed tomography (CECT) scan was done to further characterize the lesion. CECT of abdomen showed large abscess extending from the subcutaneous and intermuscular planes over the right iliac fossa and breaching the peritoneum. It showed heterogeneous wall thickening of the proximal part of the ascending colon with loss of mural stratification and causing luminal narrowing, measuring 4.8 × 5.1 × 4.5 cm with surrounding inflammatory changes with adjacent well-defined collection 11.3 × 4.5 × 10.9 cm and few air pockets and infiltrating the right lower abdominal wall . Pus aspDuring laparoscopy, a right-sided colonic mass adherent to the lateral abdominal wall with infiltration of the subcutaneous tissue by the tumor was noted .en bloc) of the involved part of the lateral abdominal wall using a closure technique.Ascending colonic cancer complicated by an anterior abdominal wall abscess was diagnosed. Exploratory laparotomy was performed and a right colonic mass ~9 × 8 cm in diameter was found, which was widely adherent to the lateral abdominal wall, with extension through a fistulous track into the subcutaneous tissue . There wThe patient had an uneventful postoperative recovery. The patient was discharged on the 14th postoperative day with a healthy wound. The patient was referred to Department of Medical Oncology for further adjuvant therapy. Consent was obtained from the patient for her case to be reported.About 15% of patients with carcinoma of the large intestine present with surgical emergencies such as perforation and obstruction. The incidence of perforated colorectal cancer ranges from 3 to 10%. Colorectal cancers have the tendency to spread locally, and advanced cancers frequently show direct invasion to adjoining organs and peritoneal dissemination . CarcinoWhen encountering such a case, early diagnosis, appropriate drainage and definitive management may reduce patient morbidity and mortality rates, especially if the patient was in poor condition in which elimination of the source of sepsis may be lifesaving . Confirmen bloc excision of the full thickness of the anterior abdominal wall, including the abscess. Complete resection of the malignant colon tumor and the abscess wall is the preferable method. Unless surgery is contraindicated by the patient’s general condition, percutaneous aspiration and drainage by indwelling catheter combined with broad-spectrum antibiotics may be applied without further diagnostic work up [Once abdominal wall involvement with colon cancer forming an abdominal wall abscess is detected, the existence of cancer cells is highly likely in both the abdominal wall penetrated by the fistula and the wall of the abscess. In such cases, the most appropriate curative procedure would be an  work up , 12. Rad work up .A differential diagnosis of carcinoma colon should be considered when an elderly patient presents with abdominal wall abscess accompanied by bowel symptoms such as altered bowel habits or per rectal bleeding, even if there are no other significant clinical symptoms and a thorough investigative work up is required to confirm the diagnosis, to avoid untimely delay in treatment and reduce morbidity and mortality among the patients.


Osteoblastoma and osteoid osteoma are rare benign neoplasms of the jaws. We reviewed current literature surrounding the ongoing debate over similarities and differences of osteoblastoma and osteoid osteoma and present two cases. Both cases are well-demarcated mixed radiodensity mandibular lesions with histological features of osteoblastoma. They exhibit, however, distinctly unique and contrasting clinical and imaging characteristics suggesting that the first case is osteoblastoma and the second is osteoid osteoma. The first case of a 37-year-old male presents with a large, expansile lesion at posterior mandible, surrounded by a thick sclerotic band. Unusual features include significant buccal/lingual expansion, extensive new bone apposition, and soft tissue edema in the masseter muscle. This is in contrast to the second case of a much smaller lesion in a 17-year-old male with history of recent third molar extraction in the left posterior mandible. In this case, CT imaging revealed a circular, nonexpansile lesion with a sclerotic border surrounded by a radiolucent rim. Both patients underwent surgical excision of the lesion with extraction of the adjacent tooth. We discuss herein the distinct clinical and imaging features. Osteoblastoma and osteoid osteoma are benign and slow-growing neoplasms of the bone with similar, if not identical, histopathological features. Some have proposed that any benign neoplasm of the maxilla or mandible that is composed of osteoblasts forming osteoid and bone trabeculae set in a well-vascularized connective tissue stroma be classified as a single entity, osteoblastoma , 2. HoweOsteoblastoma is a rare tumor of the bone, accounting for approximately 3% of all benign bone tumors and 1% of primary bone tumors . OsteoblOsteoid osteomas are more common than osteoblastomas accounting for approximately 10-12% of all benign bone tumors and 3% of primary bone tumors . These lRadiographically, both lesions usually appear as well-circumscribed, mixed density lesions surrounded by a sclerotic rim. Osteoid osteoma usually displays a larger area of sclerotic bone formation than osteoblastoma , 10. OstHistologically, osteoblastoma and osteoid osteoma appear similarly and are characterized by the abnormal proliferation of osteoblasts forming osteoid and woven bone superimposed on a well-vascularized fibrous connective tissue stroma , 7, 10. We report herein the details of two cases with histological features of osteoblastoma with distinct clinical and imaging characteristics. We will review case reports of osteoblastoma and osteoid osteoma in the maxilla and mandible. The literature search was conducted using PubMed and Google Scholar search. The search term for osteoblastoma literature was “osteoblastoma AND mandible OR jaw”. The search term for osteoid osteoma literature was “osteoid osteoma AND mandible OR jaw”. We will further discuss the ongoing debate over the similarities and differences between osteoblastoma and osteoid osteoma.A 37-year-old male presented to the clinic with pain in the left posterior mandible and preauricular area that had been progressively worsening over the past four months. Pain was managed with nonsteroidal anti-inflammatory drugs (NSAID) and acetaminophen at the time of initial exam.Clinically, there were no changes to occlusion or paresthesias. The range of motion was within normal limits. Both intraoral and extraoral exam demonstrated swelling in the ascending ramus area of the left mandible with pain on palpation. All four wisdom teeth are impacted and not visible clinically. Probing depths were less than 4 mm in the lower left quadrant.Past medical history is significant for treatment of a mandibular fracture 11 years prior to presentation with no complications but is otherwise noncontributory.Medical grade CT revealed a well-defined, mixed radiodensity lesion within the body of left mandible, measuring 1.6 × 2.1 × 2.0 cm. The lesion is located in the left posterior mandibular angle, involving the root of full bony impacted #17 and extending to the inferior third of the mandibular ramus. The internal structure was mixed but predominantly densely mineralized with a surrounding lucent halo. Buccal and lingual expansion, thinning, and areas of perforation at buccal and lingual cortices can be appreciated. There was a broad zone of reactive sclerotic bone surrounding this lesion . Large pDue to size and radiographic presentation, the differential diagnosis for this lesion in the left posterior mandible includes osteoblastoma, osteosarcoma, osteoid osteoma, ossifying fibroma, focal cemento-osseous dysplasia or calcifying odontogenic cyst with odontoma. Radiographically, osteosarcoma usually presents with poorly delineated borders, making an osteosarcoma diagnosis less likely for the lesion in question. Given the large size of the lesion, a diagnosis of osteoid osteoma would be unlikely. Ossifying fibroma does not typically present with a wide zone of sclerotic bone formation as seen in this lesion. Due to the expansile nature of the lesion, focal cemento-osseous dysplasia is unlikely in this case.A 17-year-old male was referred to an oral surgeon for surgical extraction of complete bony impacted third molars . For hisPanoramic and CBCT imaging revealed a small well-circumscribed lesion distal and lingual of tooth #18 Figures . The oraThe patient initially said he was feeling better after the surgical exploration, but by the next month, the pain had returned and was continually worsening. The patient was then referred to the Oral and Maxillofacial Surgery Department at the University of Washington for further evaluation and treatment. Upon exam at the University of Washington, the patient was found to have radiating pain to his ear. The patient described it as “shooting nerve pain that gets worse at night.” Patient reported intermittent vague swelling, but no bad taste. At this time, he was controlling the pain with ibuprofen. Intraoral examination revealed scar tissue at extraction site but no swelling, erythema, drainage, or abnormally appearing tissue .Panoramic imaging 8 months post extraction when the patient began feeling pain in the area revealed a small mixed density lesion associated with the distal root of #18 which was not present in the panoramic image prior to #17 extraction Figures .Cone beam CT image obtained by the oral surgeon prior to initial biopsy revealed a well-defined, mixed density lesion with a soft tissue capsule surrounding a cortical border on the distal and lingual aspects of #18. The lesion has eroded and expanded beyond the lingual cortical plate . No rootA medical CT scan was obtained several months after the CBCT to evaluate the changes from the CBCT as well as potential soft tissue involvement. Imaging revealed a 1.2 × 1.0 × 1.1 cm lesion emanated from the distal root of #18 that had eroded and expanded beyond the lingual cortical plate. A small area of bony sclerosis is noted at the buccal and superior aspects of the lesion. The lesion appeared similarly on bone window CT as the previous CBCT. There was no soft tissue involvement .Given the radiographic findings and characteristic pain presentation, osteoid osteoma was considered to be high on the differential diagnosis followed by osteoblastoma. Close relation the distal root of #18 added cementoblastoma to the differential. However, the distal root of #18 showed no evidence of root resorption making this diagnosis unlikely.Case #1 was treated with enucleation and full bony extraction of #17. The lesion was removed in pieces Figures . CauteriPanoramic radiograph obtained one month post excisional biopsy demonstrated complete removal of the lesion and #17 with evidence of bony sclerosis still visible .Case #2 was treated with enucleation, curettage, and extraction of #18. The site was irrigated and closed using multiple interrupted 3-0 chromic sutures. Adequate hemostasis was obtained with primary closure. Histopathological interpretation of both biopsy samples obtained from excisional biopsy was consistent with osteoblastoma. Biopsy specimens were represented by multiple fragments of decalcified hard and soft tissue made up of bone at different stages of development Figures , some miThough both cases presented were diagnosed as osteoblastoma histopathologically, they differ significantly in both clinical presentation and imaging characteristics. Taken together, case #1 is considered osteoblastoma and case #2 is considered osteoid osteoma . It has Clinically, both osteoblastoma and osteoid osteoma commonly present with pain; however, the pain pattern is specific for each neoplasm. Pain reported in osteoblastoma is described as dull, aching, and progressively worsening over time. It is thought to be caused by local expansion of the tumor and does not typically respond to treatment with NSAIDs . A typicAnother important difference between the two lesions is size. Osteoblastoma typically presents as >1.5-2 cm in diameter and is known for its tendency to cause bony expansion , 8. The The average age of patients in our review diagnosed with osteoid osteoma was similar but slightly older than those diagnosed with osteoblastoma with averages of 26.63 years and 22.76 years, respectively. This is consistent with previous reports of these lesions predominantly arising in the first and second decades of life , 11; howRadiographically, most osteoblastoma and osteoid osteoma cases reviewed in Case #1 demonstrated a unique degree of soft tissue edema in the adjacent masseter muscle as well as the extensive formation of new, reactive bone Figures  and 2. TThe lesion in case #2 developed shortly after routine third molar extractions. While history of extraction has been reported in other cases of osteoblastoma and osteoid osteoma, the timing of this presentation is closer to time of extraction, occurring only 8 months after the procedure , 16. It With so few reports of these benign bony neoplasms available in the literature, misdiagnoses can be common and preoperative diagnoses are often variable. In 24 cases of osteoblastoma, Jones et al. found 37.5% of preoperative diagnoses to be a fibro-osseous process such as fibrous dysplasia, ossifying fibroma, or focal osseous dysplasia, 25% to be a bone tumor or bone disease, 20.8% to be an odontogenic cyst, 12.5% to have no preoperative diagnosis, and 4.2% to be a salivary gland neoplasm [The recommended treatment for osteoblastoma and osteoid osteoma can be very different, thus highlighting the importance of distinguishing between the two lesions despite similar histopathological features. Our reviews here as well as previous case reports support that osteoid osteoma and osteoblastoma can and should be distinguished from one another based on reported features , 4, 17. Recurrences of both lesions have been described in the literature. It has been found that osteoid osteomas recur slightly less often than osteoblastomas, with a recurrence rate of 4.5% compared to 9.8%, respectively , 20. To While the WHO still classifies osteoblastoma and osteoid osteoma as separate tumors, it has been recommended by some to reclassify these two neoplasms as single and separate disease processes –4. Other


CTNNB1  in one case and a homozygous loss associated with breakage targeting APC (5q22.2) in the second.Pancreatoblastomas are unfrequent tumors usually found in children. We report two cases of metastatic pancreatoblastomas observed in young women. A systemic chemotherapy (FOLFIRINOX regimen) was associated with a disease control in one case and a partial response in the second with an improvement of general status for both. A high-throughput sequencing of the tumor described in both cases alteration in the Wnt/β-catenin pathway: a mutation in  Pancreatoblastoma is a very rare malignant tumor of the pancreas, usually found in children <10 year-old –4, and tThis 33-year-old woman, with no prior familial or personal medical history, presented in 2009 epigastric pain and a palpable left upper abdomen quadrant mass. The diagnosis of large pancreatic tumor invading the gastric wall leads to perform a radical R0 distal pancreatectomy. On histopathological examination, the diagnosis of pancreatoblastoma was done in front of an encapsulated mass, with epithelial components with acini, some tubular structures and squamoid corpuscles. No adjuvant treatment was delivered. The follow-up was uneventful until 2012, when three liver metastases were found. Resection and external beam irradiation (Cyberknife®) of metastases were then performed. Liver metastases recurred a few months later (two in the left lobe and a large one in the remnant right lobe) but progressed at a very slow pace and were only submitted to radiological monitoring until October 2019. Then, a clear progression was observed on the large metastasis  of the rALK exons 23, 24, and 25, MET exons 14, 16, 17, and 18, and BRAF exon 15. On pathological exam, tumor cells displayed a strong cytoplasmic and nuclear staining for β-catenin by IHC. In 2019, after obtaining written informed consent from the patient, a NGS analysis (FOUNDATION ONE® CDx) from DNA extracted from one of the resected liver metastases was performed before initiating chemotherapy. The tumor was classified as MicroSatellite Stable (MSS), the Tumor Mutational Burden (TMB) was low (1 mutation/Mb), and only two somatic gene mutations were retained: CTNNB1 ; these two mutations are hotspot mutations reported in COSMIC database (respectively COSM5679 and COSM750462). Some variants of unknown significance were also noted: DIS3 (P666L), DOT1L (G724S and R544K), FGF19 (S147T), KDR (R541G), MYCL1 (S26R), PTEN (S294R), and SPEN (D1701E). No other base substitution, insertion/deletion, gene fusion, and copy number alteration was observed.In 2014, in order to find out possibilities for targeted therapy, immunohistochemistry (IHC) of the primary tumor searching for ALK, CMET, and ROS-1 was negative as well as the search for gene mutations in 18FDG-PET. The serum levels of CEA, CA19-9, Chromogranin A, AFP, and lipasemia were normal. Only NSE was slightly increased  as well as γ-GT (6xN). Four cycles of FOLFIRINOX regimen were delivered from February to April 2020. As side effects the patient presented an asthenia (grade 2) and a thrombopenia (grade 3 then 2), requiring dose reduction and delays. CT-scan and hepatic MRI performed after the fourth cycle showed a RECIST 1.1 partial response with a decrease of the maximal diameter of the pancreatic lesion from 44 to 23 mm and a decrease in number and size of all hepatic lesions that became infracentimetric  was submitted to NGS analysis  and array-CGH (Agilent 4x180k) as previously described ( 5q22.2:  was obseIGF2 and CDKN1C) (APC gene)  , and FamPC gene) . At diagPC gene) . SquamoiPC gene) . On IHC,PC gene) .Surgery of the primary tumor but also of the liver metastases is the first therapeutic option, but recurrences are frequent, and second surgery can be performed. When surgery is not possible, some chemotherapy regimens have been proposed. In our two patients, the FOLFIRINOX regimen, gold standard in pancreatic adenocarcinomas, allowed some improvement after respectively 6 and 4 cycles, with significant decrease in tumor size and improvement in the patients' performance status. As cases are scarce only a few reports with new systemic chemotherapy regimen has been published, but similar response has been reported in one case in the literature . The devCTNNB1 exon 3 mutations. Such mutations are rare in cancers other than liver, uterine, and adrenocortical carcinomas . In the pancreatic ductal adenocarcinoma, gene alterations are quite different: the most frequent ones concern KRAS (95% of the cases), CDKN2A (>90%), TP53 (75%), and SMAD4 (55%) (CTNNB1 mutations are very rare (1%) and only found in KRAS wild-type tumors  , while Ce tumors .APC/β-catenin pathway was observed in 6/9 cases . On 2003, Tanaka et al. reported a series of seven pediatric cases (CTNNB1 mutation (exon 3) was found in 2/5 cases tested. On 2012, one case of congenital pancreatoblastoma in a 3-day-old child was reported with nuclear/cytoplasmic accumulation of β-catenin and an activating CTNNB1 mutation in exon 3 (via somatic mutations of CTNNB1 (90%) or copy-neutral loss of heterozygoty of APC (10%), with concurrent imprinting dysregulation of IGF2  and Wnt pathway effectors (such as LEF1 and TCF7). Of note, the authors identified a concurrent imprinting dysregulation of IGF2, as already reported, although at lower frequency in other pediatric tumors like hepatoblasoma  in the first case and a deletion involving APC in the second case, likely responsible for activation of Wnt/β-catenin signaling pathway. These alterations are frequent in pediatric and adult cases, and can help to differentiate acinar cell carcinomas, solid-pseudopapillary neoplasms and pancreatoblastoma from pancreatic ductal adenocarcinoma. Unfortunately, until now, no therapy targeting such alterations is available. Molecular analysis of larger series is warranted.In conclusion, we report the clinical outcome and molecular profile of two additional cases of sporadic metastatic pancreatoblastoma in young adults. Both patients benefited from systemic FOLFIRINOX regimen that provided a long-lasting minor response in one case and an ongoing partial response in the second one. Exome sequencing showed a The original contributions presented in the study are included in the article/supplementary material, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.J-LR, SO-T, and FB: writing of the manuscript. J-LR, SO-T, TL, and LB: cares of the patients. FP and M-FH: pathology. FB, JA, AG, and MC: genomics analysis. All authors: corrections, modifications, and final acceptation.The authors declare that the research was conducted in the absenceof any commercial or financial relationships that could be construed as a potential conflict of interest.


A 40-year-old woman presented with a huge right labial swelling that had been enlarging gradually for the last four years. A 10-cm labial swelling was revealed on clinical examination. Preoperative magnetic resonance imaging (MRI) was used to rule out other possible diagnoses, such as hernias, vulvar cysts and tumours. When it came to assessing and diagnosing soft tissue swelling, MRI was the method of choice. It determined that the swelling had a bright T1 and T2 signal intensity similar to that of subcutaneous fat, with a decline in signal in fat-saturated sequences, indicating a diagnosis of vulvar lipoma. The mass was surgically excised, and histopathology confirmed the diagnosis of a benign lipoma. Lipomas are considered the most common soft tissue tumours accounting for a prevalence of 1% in the population. Their occurrence in the vulva in premenopausal women is considered rare and only a few cases of vulvar lipomas have been reported in the literature. Herein, we report a rare case of a patient with vulvar lipoma and review the available literature. Lipomas are considered the most common soft tissue tumours with a prevalence of 1% in the population and an incidence of 2.1/1000 individuals/year . VariousUltrasonography (USG) is thought to be the first imaging modality that should be used for the diagnosis of groin masses ,4,11. USHerein, we report a case of a lipoma of the labia majora that was carefully investigated and successfully excised surgically in a tertiary-care hospital.The case report has been conducted in line with the Surgical CAse REport (SCARE) criteria . The patA 43-year-old, newly married patient was referred to the general surgery ward from the gynaecology department. She presented with a painless, slowly growing swelling in the right labium for the past four years with no other symptoms. The mass was found as part of the clinical exam done by the gynaecologist as the patient was undergoing investigation for infertility. The patient had believed that this mass was the cause of her infertility. No previous history of trauma was present. She had undergone laparoscopic cholecystectomy in the past.Examination revealed a single soft 5 x 9 cm mass in the right labium majus, non-tender, non-fluctuant, and irreducible with no transillumination, a negative cough impulse test, and no inguinal lymph nodes enlargement. The overlying skin was normal.InvestigationsMRI pelvis Figure  showed aDifferential diagnosisClinically, vulvar lipomas must be differentiated from other lesions such as the cystic enlargement of the canal of Nuck and Bartholin's gland ,13 and tTreatmentSurgical excision was planned. The operation was performed under general anaesthesia and the patient was placed in a lithotomy position. Blunt and sharp dissection was carried out, and a mass measuring 11 cm was excised Figure . The vulOutcome and follow-upThe patient was discharged on the first postoperative day. Histopathological examination confirmed the diagnosis of a benign lipoma. She was followed up in the clinic after one and four weeks postoperatively. The postoperative course was uneventful, there was no discharge from the surgical site, and sutures were removed.Vulva benign tumors are characterized according to whether they originate from epithelial or mesenchymal cells . Vulvar Lipomas are most frequent in people in their fourth to sixth decades of life, but they can occur at any age. A vulvar lipoma has a presentation similar to that of other lipomas, reported in any age group -5,13.The aetiology and pathogenesis of lipomas remain unclear. Trauma, obesity, and genetic abnormalities are some of the reported risk factors involved in the development of lipomas . Our patLipomas typically present as single or multiple soft tissue swellings that gradually growing, non-painful, mobile, non-adherent to the overlying skin with a characteristic doughy feel ,13. ThisWhen the clinical diagnosis is not clear, imaging like USG, CT, and MRI are helpful in distinguishing vulvar lipomas from vulvar cysts, liposarcomas, and inguinal hernias ,10,13,15It has been reported that USG is highly sensitive, specific, and reliable , and it In determining the anatomical extent of vulvar lipomas and distinguishing them from liposarcomas, CT scans and MRI are useful ,15. NoneHistologically, a lipoma must be differentiated from a well-differentiated lipoma-like liposarcoma by substantial tumour dissection . SurgicaBecause the lump was big and situated in an unusual location, we opted for surgical removal to confirm the diagnosis of a lipoma, as the vulva is considered an uncommon location for the formation of this mass.Lipoma in the vulva is a rare occurrence. A comprehensive imaging study is necessary to rule out alternative diagnosis. The surgical treatment of vulvar lipoma is similar to that of other lipomas. It is unclear if all individuals require excision or whether radiological follow-up is necessary. Furthermore, there is no evidence of long-term follow-up in the literature.This study concludes that a labial lipoma is rare and needs to be investigated carefully to exclude other malignant masses like liposarcomas. Since it is painless and slowly growing, patients can have the mass for a long time without needing to seek medical advice. There is a chance that the lesion may harbour a malignancy if it is not investigated and managed appropriately. MRI is considered the imaging modality of choice. MRI is helpful in differentiating benign from malignant soft tissue lesion, hence wide excision is not recommended in cases of benign-looking lipoma. We recommend providing awareness to the women and encouraging them to seek medical advice if they have a vulvar mass.


Ovarian torsion is one of the common abdominal and gynecologic surgical emergencies with serious morbidity in the form of ovarian infarct or necrosis. Various imaging modalities like USG, CT, and MRI have been used in the evaluation of ovarian torsion. Two middle-aged females presented with lower abdominal pain. Imaging showed an ovarian cystic lesion in the first patient and thickened and twisted ovarian pedicle or ‘helical swirling’ sign in the second. Intraoperatively, the first case turned out to be a 180* twist with mild ovarian edema and the second showed more than 720* torsion with ovarian infarction. The CT findings of twisted pedicle with pericystic fat stranding might be predictive of hemorrhagic infarction in cases of torsion. The ovary is a female pelvic organ suspended in the pelvic peritoneal cavity by three peritoneal folds or ligaments - the infundibulopelvic fold or suspensory ligament, which attaches the ovary to the lateral pelvic wall, the utero-ovarian ligament connecting the ovaries to the uterus, and the mesovarium connecting the ovaries to the broad ligament of the uterus . TorsionCase 1A 48-year-old female presented with right lower abdominal pain for one month. She gave a history of a right adnexal cyst of small size. On USG, a multiloculated cystic lesion was noted in the right adnexa, which was relatively tender and had a mildly swollen appearance. The lesion showed normal surrounding vascularity with part of the normal ovary in continuity with the lesion. A CT scan confirmed the findings and did not show any surrounding fat stranding or fluid collection Figure .No para-uterine or adnexal soft tissue thickening area was noted. On surgical exploration, a 180* twist of the cystic lesion and the ovary was seen Figure . The ovaCase 2A 50-year-old female presented with a history of long-standing right lower abdominal discomfort that had increased since four days. She had moderate-to-severe right para-umbilical and lower abdominal pain with guarding. Ultrasonography revealed a large unilocular cystic lesion extending up into the para-umbilical region. The lesion showed increased peripheral vascularity and the right ovary was not separately visualized. The uterus and left ovary were normal. The cyst showed thick walls and subtle pericystic fat stranding, which are unusual for otherwise benign-looking features. There was helical soft tissue thickening that showed contrast enhancement extending from the lateral aspect of the uterus towards the cyst Figure .There was a strong suspicion of torsion and the patient was taken for surgery. On surgery, the cyst showed more than two twists around its pedicle (nearly 720*) and it also showed hemorrhagic congestion Figure . The hisOvarian torsion is one of the acute gynecologic surgical emergencies that need early identification for proper management . The firCT and MRI have been used in the evaluation of torsion in more subacute settings. As early as 1994, Kimura et al.  describeCT is routinely used as a problem-solving modality in acute abdomen that can also help to rule out torsion . Rhe et Ito et al.  first deCT findings of twisted pedicle with pericystic fat stranding may be useful to predict those cases of adnexal torsion leading to infarction by indicating more than 360* twisting of adnexal torsion and hence correlate with operative and gross findings of infarction in cases of torsion.


Introduction: Primary cutaneous adenoid-cystic carcinoma (PCACC) is a rare malignant tumour reported in only about 450 cases in the literature, with only two adolescent cases reported. PCACC seems to occur between the fifth and seventh decade of life, and the most frequent regions involved are head and neck (46%). Aplasia cutis congenita (ACC) has an incidence of 1:10,000, and it seems to be rarely associated with neoplastic lesions. Interestingly, the association between PCACC and ACC has, so far, never been described. Methods: We report a case of PCACC in the scalp associated with ACC in a four-year-old patient. Discussion: The patient was under follow-up at the dermatology unit, but suddenly a red lesion appeared within the ACC. This red, ulcerated area increased rapidly over six months, so it was surgically removed, and the pathological examination results were suggestive for cribriform PCACC. According to the guidelines for skin tumours, the patient underwent widening resection, and an advancement-sliding skin flap was performed to recreate the scalp. After one year of follow-up, the patient has no local or widespread recurrence of the PCACC, and the surgical scar appears to have healed well. Conclusions: This clinical case is the first known patient with PCACC associated with ACC. A skin excision biopsy should be performed with wide margins to avoid a second widening resection of skin in a similar scenario. Genetic studies may help to identify the origin of this rare association. Primary cutaneous adenoid cystic carcinoma (PCACC) is a rare, slow-growing malignancy tumour that usually affects middle-aged and older individuals. It frequently develops on the head, neck, chest or abdomen, and is characterised by aggressive local behaviour, with a very low incidence of metastasis ,5,6,7,8.Aplasia cutis congenital (ACC) is a rare skin disorder characterised by a localised absence of skin, usually located on the scalp. Still, it can occur anywhere on the body, including the face, trunk and extremities . ACC mayWe describe a case of PCACC in a four-year-old female referred to our clinic for an aplasia cutis congenital of the scalp.Case ReportThe female patient has been under clinical follow-up at the dermatology unit since her neonatal period due to an ACC of the scalp. At the age of four, a small hyperemic granulomatous area  and showed areas of ulceration .Under general anaesthesia, the lesion was surgically removed with the entire ACC area mainly involved; the excised tissue was sent for pathological examination. The postoperative course was regular, and the patient did not develop wound infection or any other complications.The histological aspect of the lesion was suggestive of cribriform PCACC A,B, withAccording to the guidelines for this rare tumour , the patThe patient was referred to the oncology unit for clinical follow-up following the histological results.After six months of follow-up, the patient has no local or widespread recurrence of the PCACC, as determined by clinical examination and radiological exams (Chest X-ray and Abdomen-US). The surgical scar appears to have healed well .ACC is a congenital condition in which skin is absent, with the variable absence of underlying structures such as bone . It mostIt generally presents as a solitary lesion, although there may be multiple locations on or near the vertex, with alopecia ranging from a few millimetres to more than 10 cm .Most patients have no associated abnormalities, but some non-isolated ACC can cause gastrointestinal , cardiac (33%), genitourinary (19%) (epispadias/hypospadias), musculoskeletal (19%), central nervous system (5%), and chromosome syndrome-associated diseases .The cause of this condition is unclear and appears to be multifactorial.ACC diagnosis is often clinical, followed by radiological examinations, and treatment usually involves a conservative approach to allow secondary intention healing . Sometimes skin flaps and grafts can be used for more extensive and profound defects to prevent haemorrhage and infection .PCACC is a rare malignant skin appendageal tumour first described by Boggio in 1975 .Remarkrishnan et al. reported that PCACCs occur more frequently on the head and neck (46%), on the upper limbs (17%), trunk (15%), and lower limbs (13%). Reports of distant metastases were very low, with about 4% lymph node and 7% distant organ involvement .Reviewing the literature, only about 450 cases of PCACC have been reported ,26,27,28The histogenesis of PCACC is still uncertain: an apocrine or eccrine derivation is debated . Three mHistological examination shows a tumour that consists of basophilic cells with a distinct adenoid or cribriform pattern in the mid to deep reticular dermis, with often myoepithelial cells surrounded by true lamina .In his study, Alkan BI et al. reported that PCACC showed BerEp4, CEA, CD117(C-kit), and CK7 expression in the tumour periluminal regions, and p63 and SMA positivity in myoepithelial cells at the periphery of the cell islands . ImmunocDefinitive diagnosis relies on histological and immune–histochemical characteristic features.The standard treatment of PCACC described in the literature is wide surgical excision with at least a 2 cm safety margin from the tumour. Wide excision has the purpose of avoiding frequent recurrence .In our case, the tumour was resected entirely after the first surgery, but a second surgery with the widening of the resection and advancement-sliding skin flaps to recreate the scalp was required after the histological close-margins report.The benignity of the basal condition (ACC) made it difficult to hypothesise the onset of a malignant disease not previously reported. In the reported case report, the tumour showed an atypical behaviour compared to the cases previously reported in literature, although only two adolescent cases are reported in the literature . MoreoveA second surgery, widening the resection (more than 2 cm from the tumour margin) and using advancement-sliding skin flaps to recreate the scalp—a complex region to perform a primary closure of a wound—was performed.No recurrence of the tumour was noted as of twelve months post-operation, with ongoing oncological follow-up.We consider this an interesting case because ACC seems to be rarely predisposed to neoplastic lesions. Only two young patients (14 years of age) were described in the literature as being affected by PCACC. The association between PCACC and ACC has, so far, never been described.Thus, we recommend that in a similar scenario, lesions appearing on ACC should not be underestimated, and a skin excision biopsy should be performed with wide margins to avoid a second widening resection of the skin.A multidisciplinary approach with oncologists, paediatric surgeons, dermatologists, anatomopathologists, and other professional figures, remains of fundamental importance in improving these patients’ management and short- and long-term results.


Colonic metastasis is uncommon in patients with hepatocellular carcinoma (HCC). In the past, extrahepatic metastasis of HCC was not treated aggressively because of its poor prognosis. Herein, we describe the case of a patient with HCC who survived for 30 months following resection of a metastatic tumor in the ascending colon.An 80-year-old man presented at our hospital with symptoms of abdominal pain on the right side and fever. He had undergone transcatheter arterial chemoembolization and posterior segment resection of the liver because of HCC, followed by radiofrequency ablation for a recurrent intrahepatic lesion 5 and 3 years, respectively, prior to the visit. He was diagnosed with retroperitoneal hematoma, which was thought to be associated with diverticulitis and an extramural tumor in the ascending colon. A definitive diagnosis could not be reached; however, a right hemicolectomy of the colon was performed because of progression to anemia. A pathological examination revealed a metastatic tumor in the ascending colon extending from the subserosal layer to the muscularis propria layer. The patient was treated with lenvatinib after surgery, but presented with intrahepatic recurrence, lymph node metastasis, and peritoneal dissemination metastasis 15 months later. The progression of the disease could not be controlled and his postoperative survival time was 30 months.Resection of metastasis of HCC might contribute to prolonged survival in cases, where radical resection is possible. Colonic metastasis is an uncommon occurrence in hepatocellular carcinoma (HCC). In the past, extrahepatic metastasis of HCC was not treated aggressively because of its poor prognosis; however, recent studies indicate that surgery can improve the prognosis if the metastatic lesion is completely resected –3. In thAn 80-year-old man visited a primary care physician, complaining of pain in the lower abdomen on the right side and fever An evaluation of the blood sample showed an increased inflammatory response, and abdominal ultrasonography revealed fluid retention in the right side of the abdomen. After referral to our hospital, a computed tomography (CT) scan was performed and the fluid accumulation in the abdomen was diagnosed as a retroperitoneal hematoma.Previously, the patient had been followed up at our hospital for chronic hepatitis C. In addition, he had undergone transcatheter arterial chemoembolization (TACE) and posterior segment resection of the liver for HCC  5 years before this visit and radiofrequency ablation (RFA) for intrahepatic recurrence (S8) 3 years ago. The pathological findings of primary HCC are as follows. It was nodular type and showed expansive growth, fibrous capsule formation, capsule invasion, and septum formation. There was no serous membrane invasion, vascular invasion, and bile duct invasion. The liver in the non-tumor area had chronic hepatitis, and surgical margin was negative.He also achieved a sustained virological response to hepatitis C virus with Ledipasvir acetonate / Sofosbuvir 2 years ago. The patient’s medical history included surgery for appendicitis, the use of stents for acute myocardial infarction, transurethral resection of a tumor in the bladder, hypertension, hypercholesterolemia, hyperuricemia, and osteoporosis. His medications included aspirin, losartan potassium, fenofibrate, benzbromarone, alendronate sodium hydrate, a proton pump inhibitor, and laxatives.The patient’s blood pressure and pulse rate were stable, and mild anemia  was observed during the first visit at our hospital; therefore, we assumed that the bleeding had stopped, and the patient was admitted to the hospital for follow-up. The symptoms seemed to improve for a while, but 1 week later, the patient experienced abdominal pain. The preoperative blood test showed a decrease in the Hb level to 8.2 g/dL. Contrast-enhanced CT at the time of visit showed a retroperitoneal hematoma centered in the right paracolic gutter . Paralytic bowel obstruction occurred during the postoperative course, but improved with conservative treatment, and the patient was discharged home on the 20th POD without any other complications.After the patient was discharged from the hospital, he was followed up without postoperative adjuvant chemotherapy at his own request. However, 6 months after surgery, CT showed peritoneal dissemination, and lenvatinib 8 mg was started on a daily administration as a patient with unresectable HCC. Because of side effects such as decreased appetite, the dose was reduced and the administration interval was spaced accordingly. However, 15 months after surgery, he presented with intrahepatic, lymph node recurrence. The same chemotherapy was continued thereafter, but the progression of the disease could not be controlled. 26 months after surgery, he was switched to palliative care and his postoperative survival time was 30 months.Colonic metastasis of HCC is an uncommon occurrence. According to the Report of the 21st Nationwide Follow-Up Survey of Primary Liver Cancer in Japan (2010–2011) , extraheInitially, the implantation of tumor cells by RFA was suspected in our patient. Subsequently, it was considered hematogenous metastasis, because the site of the lesion was away from the puncture site, the main locus of the metastatic lesion was limited to the subserosal and muscular layers, and vascular invasion was observed.In the case of hematogenous metastasis, the venous flow from the colon to the liver is reversed, possibly because of the increase in the portal pressure in patients with cirrhosis and during TACE. Table According to the Japanese guidelines for the treatment of HCC , the admWhen a ruptured tumor accompanied by retroperitoneal hemorrhage was found, HCC colonic metastasis should be ruled out. Although a preoperative diagnosis was not possible in this case, the use of Tc-99 m for the diagnosis of colorectal metastasis of HCC has been reported previously , which mResection of metastases of HCC contributed to a prolonged prognosis in the patient in this study and might prove useful in cases, where radical resection is possible.


Teaching Point: Snowball-like and icicle-like lesions in the corpus callosum suggest Susac Syndrome. A 45-year-old man presents with headache and photophobia over several weeks and word-finding difficulties, multifocal paresthesia, vertigo, and right-sided hearing loss for several days. He has no relevant past medical history.Figure 1). Round lesions of the corpus callosum (CC) are located in the genu and the splenium . Another wedged-shaped lesion extends from the roof in the truncus, without contacting the CC base . Diffusion-weighted imaging (D-WI) shows a diffusion restrictive lesion in the truncus . The genu and splenium lesions are hypo-intense on T1-WI .Magnetic resonance imaging (MRI) shows multiple hyperintense white matter lesions on fluid-attenuated inversion-recovery (FLAIR) sequence  shows peripheral retinal arterial occlusion with arterial plaques. Audiogram confirms right-sided sensorineural hearing loss.Based on the combination of clinical, FA, audiogram findings and imaging features, the diagnosis of Susac syndrome was made.Susac syndrome (SS) is a rare disease, mainly affecting women between 20 and 40 years old. It is typically characterized by a clinical triad of encephalopathy, branch retinal artery occlusion, and sensorineural hearing loss. In many cases, this triad is not completely present at diagnosis and occurs metachronously. The diagnosis is based on the combination of clinical findings, FA findings, and MRI features. SS is presumably caused by auto-immune mediated micro-infarctions in the brain, retina, and inner ear. The clinical course is mostly self-limiting and monophasic. In some patients, the disease is highly unpredictable and may have a polycyclic, relapsing, or chronic continuous course .MRI is the preferred imaging technique for diagnosis of brain involvement. T2-WI show small, multifocal hyperintense lesions of 3–7 mm. The CC is invariably involved. These CC lesions have a characteristic ‘snowball-like’ or ‘icicle-like’ morphology. ‘Snowball-like’ lesions have a round morphology and a central location in the CC. ‘Icicle-like’ lesions are wedge-shaped and extend from the roof of the CC without contacting the base of the CC. Other, periventricular located lesions are non-specific for SS. Diffusion restrictive lesions indicate SS micro-infarcts in the acute phase. Leptomeningeal enhancement may be present, especially in fulminant cases. CC atrophy is possible. MS and ADEM are the most important differential diagnoses. In contrast to SS, MS and ADEM lesions in the CC are typically ovoid and predominately located at the base and septal interface. Leptomeningeal enhancement and deep gray matter involvement are uncommon in MS and ADEM .An early, aggressive immunosuppressive treatment is indicated to prevent damage. However, an optimal immunosuppressant regimen has not been defined .