Pentalogy of Cantrell is a rare syndrome of anomalous malformation. In the present case, the syndrome was initially diagnosed as a complete pentad, including a supra-umbilical abdominal wall defect, a sternal defect, pericardial defects, an anterior diaphragmatic defect, and heart malformation. Diagnosis required several imaging modalities, including computed tomography (CT) and magnetic resonance imaging (MRI). In this case report, we present an 8-month-old female patient with a thoracic wall defect with ectopia cordis and a bilateral cleft lip and palate. In addition, a head CT scan showed craniosynostosis, hypogenesis of the corpus callosum, and tonsillar cerebellar herniation. Thoracoabdominal CT revealed herniation of the transverse colon up to the subcutaneous layer, diaphragmatic hernia, atrial septal defects (ASD), ventral septal defects (VSD), and a persistent left superior vena cava (PLSVC). A multidisciplinary approach is required for the optimal management of this syndrome. We describe a female infant who presented with pentalogy of Cantrell syndrome and include the findings from postnatal CT imaging. Rare cases of Pentalogy of Cantrell have been reported since the first case was characterized by Cantrell and colleagues in 1958. It was consist of supra umbilical abdominal wall defect, sternal defect, pericardial defects, anterior diaphragmatic defect and heart malformation In 1972, Toyama suggested an alternative classification system to describe the more commonly occurring variants of this syndrome: [2]Class 1: definitive diagnosis .Class 2: probable diagnosis .Class 3: incomplete expression .The pathogenesis of this syndrome is not fully understood. Occurrence of the complete pentalogy is rare, and the survival rate of patients with complete pentalogy is low, with outcomes dependent on the severity of the cardiac malformation, extracardiac defects, and other associated anomalies Cleft lip, with or without cleft palate and encephalocele, tends to be specifically associated with ventral midline anomalies within the spectrum of pentalogy of Cantrell We report a case of an incomplete pentalogy of Cantrell and highlight the importance of diagnosis and the wide range of anomalies associated with this syndrome.Informed consent was obtained from the patient's mother  for the publication of this case report.The 8-month-old female patient described in the present case report was born in Pamekasan Hospital in East Java. The baby was delivered via normal vaginal birth, and the child cried spontaneously at birth. There was no history of maternal illness during the pregnancy, and the mother did not receive antenatal care. There was no history suggestive of exposure to teratogens during the pregnancy. Furthermore, there is no family history of birth defects.Physical examination revealed a defect in the thoracic wall with ectopia cordis, and a bilateral cleft lip and palate . Chest eThe patient subsequently underwent postnatal evaluation in our radiology department. A contrast computed tomography (CT) scan of the head and three-dimensional (3D) reconstruction showed craniosynostosis at the left lambdoid suture, left coronal suture, and frontalis suture, leading to an appearance of turricephaly, along with maxillofacial defects in the nasal bone, orbital cavity, and cleft lip and palate. With the brain window setting, we identified hypogenesis corpus callosum and tonsillar herniation .Fig. 2HeThe patient also underwent contrast chest and abdominal CT. The results showed a defect of the sternum accompanied by left ventricle herniation of the heart outside the thoracic cavity that was covered with a thin serous membrane. The upper abdominal wall and anterior diaphragm were absent, with a hernia in the transverse colon through the defect and up to the subcutaneous layer . We alsoThe etiology and pathogenesis for pentalogy of cantrell are not well understood and are thought to have a heterogeneous origin. Cantrell et al. suggested that a developmental failure in a segment of the lateral mesoderm occurred around embryonic days 14-18. Consequently, the transverse septum of the diaphragm does not develop, and the ventromedial migration of the paired mesodermal folds of the upper abdomen fails to occur. Due to the subsequent sternal and abdominal wall defects, the organs may eviscerate Most reported cases of pentalogy of Cantrell are thought to be sporadic in occurrence; however, mutations in genes located on the X chromosome have been associated with ventral midline disruptions. An X-linked dominant inheritance pattern has been observed in some families ,7. In suALDH1A2, which encodes for retinaldehyde dehydrogenase type 2, an enzyme with a critical role in normal cardiac and diaphragm development, thus demonstrating a biologically plausible association with this disorder A specific gene that could be responsible for this disorder has not yet been identified; however, a recent case report described a novel maternally inherited microduplication of chromosome 15q21.3 on the postnatal chromosomal microarray of a newborn with a prenatal diagnosis of pentalogy of Cantrell. This region includes the gene Kumar et al. proposed that variations of pentalogy of Cantrell could be classified according to the number of defects: Class 1 is defined as a definitive diagnosis in which all five defects are present; Class 2 is defined as a probable diagnosis in which 4 defects are present, including intracardiac and abdominal wall abnormalities; Class 3 is defined as an incomplete diagnosis in which at least 2 of Cantrell's anomalies are present, one of which is sternal Ghidini et al. described complete and incomplete variants of the pentalogy CT is an effective diagnostic tool for infants suspected of having pentalogy of Cantrell and generally provides high spatial resolution, truly multiplanar capabilities, and fast acquisition times without the need for anesthesia. However, the ionizing radiation and iodine contrast required for CT must be weighed against these benefits Cantrell suggested that a developmental failure in the lateral mesoderm around embryonic days 14-18 leads to partial developmental failure of the transverse septum of the diaphragm, in which migration of the paired mesodermal fold does not occur Our patient also presented with a rare PLSVC vascular anomaly that began at the junction of the left subclavian and internal jugular veins and passed through the left side of the mediastinum adjacent to the aortic arch. It mostly drains into the right atrium through the coronary sinus. Although PLSVC is rare among vascular anomalies, it is the most common thoracic venous anomaly. For the most part, PLSVC is asymptomatic and is typically detected incidentally in diagnostic examinations In addition to the findings described above, the results of a head CT scan with 3-dimensional reconstruction revealed another congenital anomaly in the present case, namely craniosynostosis, which is a condition of premature fusion of one or more cranial sutures, resulting in characteristic skull deformity and facial asymmetry. Approximately 85% of cases of primary craniosynostosis occur as isolated conditions, and the remaining 15% are part of a multisystem syndrome Imaging is essential for obtaining an accurate diagnosis, planning surgical treatments, post-treatment evaluation, and identification of other anatomic abnormalities and complications associated with craniosynostosis. In this regard, because of the superior bone depiction, 3D CT reconstruction is the primary imaging technique used for craniosynostosis Treatment strategies and the prognosis of pentalogy of Cantrell depend on the severity of intra and extracardiac defects, pulmonary hypoplasia, the extent of abdominal wall defects, cerebral anomalies, and diaphragmatic herniation The fusion defect leaves the mediastinal viscera unprotected, resulting in an increased risk of harm to the underlying mediastinal organs. Surgical repair should be performed in the first weeks of life to restore the protective function of the skeleton by establishing bony integrity, as soon as possible, and to take advantage of the high flexibility of the chest wall in newborns A multidisciplinary team should follow up on milder forms of this disorder in order to determine the best time for delivery. After delivery, repair of the omphalocele should be carried out as soon as possible. Repair of the sternal, diaphragmatic, and pericardial defects can also be attempted at the same time Pentalogy of Cantrell is a rare anomalous malformation syndrome, the etiology and pathogenesis of which are not fully understood and are considered to be of heterogeneous origin. The diagnosis of the present case required several imaging modalities, including CT scans and magnetic resonance imaging (MRI). In general, CT scans can diagnose and categorize pentalogy of Cantrell and provide high spatial resolution, truly multiplanar capabilities, and fast acquisition times, without the need for anesthesia. Moreover, during the diagnostic process, it is important for clinicians to be aware of the range of imaging possibilities and other assessments.


Parathyroid cysts are a rare clinical entity that may arise in the neck or mediastinum. They are more common in women and generally present in the fourth and fifth decades of life. Diagnosis of parathyroid cysts is challenging, and despite thorough radiological and cytological investigation, they are often mistaken for thyroid pathology. Definitive diagnosis is often only confirmed following complete surgical resection and histopathological analysis. We present the case of a woman who was referred to our outpatient clinic with a left-sided neck mass and associated compressive symptoms. Initial examination and investigation appeared consistent with a large thyroid nodule. Following surgical resection, the lesion was found to be a parathyroid cyst. Subsequently, we review the available literature on parathyroid cysts with particular emphasis on the diagnostic challenge they pose to clinicians. Parathyroid cysts (PCs) are a rare clinical entity with approximately 350 cases reported internationally . PCs posWe present the case of a 47-year-old female who presented to our head and neck clinic with a long-standing, enlarging left sided anterior neck mass. At presentation, the patient had compressive symptoms, including dysphagia and dyspnoea while supine. Review of symptoms was otherwise noncontributory. She was clinically and biochemically euthyroid. She was a nonsmoker and denied heavy alcohol use. Her medical history was significant for well-controlled asthma with no significant family history.Clinical examination demonstrated an obvious swelling over the left lobe of her thyroid gland approximately 6.0 × 5.0 cm. On palpation, the mass was firm, nontender, not tethered to any overlying or underlying structures, and had no overlying skin changes. Flexible rhinolaryngoscopy showed no suspicious pathology in the upper aerodigestive tract and symmetric vocal cord mobility. Thyroid ultrasonography was obtained and demonstrated a 6.9 × 5.0 × 4.2 cm benign appearing cystic mass in the left thyroid lobe. All haematological parameters including thyroid function testing and corrected calcium were within the normal range.She was subsequently discussed at our institutional endocrine multidisciplinary meeting (MDM). The recommendation from the MDM was to perform left hemithyroidectomy in view of her compressive symptoms. Preoperative fine-needle aspiration cytology was deemed to be unlikely to change the management plan due to the benign appearance of the nodule on ultrasound scan. Before her surgery, the mass had noticeably enlarged, and a subsequent preoperative CT neck  was obtaA transverse anterior neck incision was made within a natural skin crease. Subplatysmal flaps were raised, and infrahyoid muscles retracted laterally. The thyroid gland was visualised and inspected. A large cystic mass was visualised deep to the left lower pole of the thyroid gland that was noted to be inseparable from the thyroid gland. The left recurrent laryngeal nerve was identified, and the mass was excised along with the left thyroid lobe. The mass was completely excised without complication. Histopathological assessment of the mass subsequently confirmed completely excised benign parathyroid cyst . PostopeAt 6-month follow-up, she remained symptom free with no clinical signs of recurrence and will remain under surveillance at our outpatient department.PCs are a rare clinical entity. They are usually observed in the fourth and fifth decades of life with a noted female preponderance . They arPCs provide a diagnostic challenge for clinicians. Nonfunctioning PCs classically present as a solitary neck mass, often mimicking a thyroid nodule . Other sInitial radiologic investigation should include ultrasonography of the neck to classify the mass and determine the need for tissue sampling or further diagnostic imaging. Classically, at ultrasound scan, PCs are described as having a nonspecific cystic structure . PCs areFine-needle aspiration should be obtained as a diagnostic and occasionally therapeutic intervention. While, in general, clear cystic fluid may not narrow the differential diagnosis of neck masses, a high c-terminal PTH within the fluid can be diagnostic of a PC. Aspirated cystic fluid analysis may show epithelial cells or fibroblasts which may raise false suspicion for malignancy . ComputeThe pathogenesis of PCs is still unclear, but a number of hypotheses exist . Some hyA number of treatment options exist for PCs. Surgical resection is historically the most widely cited treatment for parathyroid cysts . Open suPCs are a rare pathology requiring a high degree of clinical suspicion for diagnosis. They should be considered as a differential diagnosis for any cystic mass in the neck or mediastinum, particularly if associated with symptoms of hypercalcemia. Definitive treatment of PCs is warranted in all cases, preferably by surgical resection.


Most cases were multiple and asymptomatic. This is the first case of perforated terminal ileum diverticula in a 32-year-old woman and successfully managed surgically.We describe a rare case of terminal ileal diverticulosis, one of the diverticula was perforated, in a 32-year-old female who presented with a 2-days history of sudden epigastric and periumbilical pain and had been managing surgically. At surgery, we found multiple diverticula, one of them was perforated. Microscopically, the specimen confirmed that the diagnosis was diverticulosis.Diverticulosis should be included in the differential diagnosis of a sudden epigastric and periumbilical pain even in this age. •Small bowel diverticulosis is not common comparing with colonic diverticulosis and occurs in up to 1% of patients.•Diverticula is more common in males than females.•We report a case of terminal ileum diverticulosis and a perforation of one diverticulum. AcquireThe majority of cases are located on the mesenteric border and are often multiple and asymptomatic except in cases with complications such as perforation, inflammation and hemorrhage .We present a case of a 32- year-old woman who has a terminal ileum diverticulosis and a perforation of one diverticulum diagnosed by pathology report and managed successfully. In our knowledge, this is an unusual clinical scenario encountered in clinical practice.This case report has been reported in accordance with the SCARE criteria .2A 32-year-old woman presented to the emergency department complaining of a sudden epigastric and periumbilical pain responds to analgesics with vomiting, fever and constipation during the last two days.A detailed surgical history revealed that the patient underwent a hemi-resection of medullary carcinoma of thyroid which led to hypothyroid later, as well as she also underwent a resection of leiomyomas uteri two years ago. In addition, she had some cardiac arrhythmias. But she had no significant allergic or familial history.Vital signs were normal and the chest examination was also normal,but her abdominal examination confirmed a rigid abdomen with general tenderness.All her lab tests were within normal ranges except left shift and mild anemia . Chest plain radiograph showed free air under the diaphragm which referred to perforation .Fig. 1PrIn result of acute abdomen condition and perforation which was indicated by radiographic findings, the surgical decision was taken to do an exploratory laparoscopy as a surgical abdomen intervention. Surprisingly, we found a loop of ileum with a diameter of 40 cm, containing of multiple diverticula along the mesenteric border and one of them was perforated which was a 20 cm away from the ileocecal conjunction . And theThe pathology report showed normal intestine mucosa, marked edema of intestinal wall with presence of 9 diverticular herniation through muscular wall, one of them was perforated , with noConsequently, she was discharged home on the third post-operative day with six months of follow-up which demonstrated a good result and her state was improved.3Diverticulosis is a disease characterized by the presence of one or more small, bulging pouches which form in the lining of particular structures in the gastrointestinal tract . The divDiverticula is more common in males than females, with an approximate ratio of 2:1. In addition, the age wise distribution of diverticulosis increases in the elderly patients especially from (60s–70s) [Non-Meckel small bowel diverticulosis is not common comparing with colonic diverticulosis and occurs in approximately 1% of the population. It mostly affects the jejunum (80%), while the ileum 15%) and (5%) in both jejunum and ileum ,3,5.% and :Dr. Ahmad Ghazal.A written informed consent was obtained from the patient for publication of this case report and accompanying images.Not commissioned, externally peer-reviewed.The authors declare no conflict of interest.


Hair Tourniquet Syndrome (HTS) is defined as strangulation by a hair thread of the appendage of the human body, especially in the children population, it rarely occurs in external genitalia . Herein, we aimed to present a rare occurrence of HTS around the labia minora in a 12-year-old child treated by excision of the cystic lesion with an uneventful follow-up. Special care should be given to every swollen appendage in children to avoid severe consequences such as amputation and disfigurement. HTS is an emergent condition that requires immediate treatment. If the diagnosis is delayed, immediate complications such as necrosis of the involved area or amputation develop. It has been described for the first time in 1612, and the first case was published in Lancet in 1832 [Patient information: a 12-year-old girl with an unremarkable medical history presented to the pediatric emergency department with acute painful swelling of the right labia minora that appeared two days ago. No history of fever, vaginal discharge, or urinary tract symptoms was mentioned. Her family had no history of malignancy or other chronic medical illness.Clinical findings: the physical exam the patient was in a good general state with normal vital signs. The vulvar examinations showed a 1.5 cm cystic lesion at the dependent portion of the right labia minora  ,3. This Only a few cases of labia minora tourniquet have been described, and this is the tenth case in the literature . The incThe diagnosis of tourniquet hair syndrome is based on the history and clinical exam which can find swollen appendages, oedema and tenderness. Magnification may be useful to identify the tourniquet. Every physician should be aware of this pathology and sexual abuse should be considered. Differential diagnosis may include infection, insect bites, allergic reactions and other . The HTSHair tourniquet syndrome involving the labia minora is extremely rare in children. Examination under a microscope makes the correct diagnosis and eliminates differential diagnoses. Every physician should be aware of this pathology and adequate treatment should be instaured as early as possible after the diagnosis.


This allowed for novel treatment with Peptide Receptor Radionuclide Therapy (PRRT) using 177Lutetium-DOTATATE after all other standard of care options were exhausted.The authors present a 45-year-old lady with a rare undifferentiated round cell tumour of the lung with a ESWR1-CREM fusion gene that progressed despite multiple lines of therapy. The tumour was Somatostatin Receptors Type 2 (SSTR2) positive and avid on  In neuroendocrine tumours, the use of 68Gallium-DOTATATE imaging to select patients for PRRT has become standard of care. This raises the possibility of the use of PRRT in other rare tumours which express high levels of SSTR2.Somatostatin Type 2 Receptors (SSTR2) are commonly found in normal cells of the brain, pituitary, gastrointestinal tract, spleen, pancreas and adrenals as well as tumours such as hepatocellular carcinomas, meningiomas, neuroendocrine tumours and small cell lung cancers.Our patient was a 45-year-old lady with no significant past medical history. She was a non-smoker who had a positive family history for lung and colorectal cancer.18[F]Fluoro-2-deoxy-2-D-glucose Positron Emission Tomography Computed Tomography (18F-FDG PET CT) showed avid uptake in her right mediastinal and supraclavicular lymph nodes. She subsequently underwent video-assisted thoracoscopic surgery (VATS) biopsy of the pleural nodules. Histological subtyping proved challenging as fairly monotonous cells with ovoid nuclei and clear cytoplasm arranged in solid sheets surrounding dilated blood vessels, with vague papillary architecture but no clear nests were seen.She initially presented with one month of cough, shortness of breath and fever. A computed tomography (CT) of her thorax, abdomen and pelvis revealed a right lower lobe lung mass with pleural nodules. Magnetic resonance imaging (MRI) of her brain did not detect any intracranial metastasis and Immunohistochemical (IHC) staining for basal cell (BerEp4), breast (ER and PR), epithelial , germ cell (PLAP), lymphoid , melanocytic (HMB45 and Melan-A), mesothelial , neuroendocrine (Chromogranin and Synaptophysin), sarcoma (CD31 and Myogenin) and urothelial (GATA3) markers as well as non-specific markers  were negative. Although tumour cells did stain strongly positive for cyclin D1, CD56, CD79A, inhibin and SSTR2  and weakOn Next Generation Sequencing (NGS), the sample was negative for ALK, BRAF, CTNNB1, EGFR, ERBB2, ERBB4, KRAS, MET, NRAS, PIK3CA, PTEN and TP53 mutations but positive for a EWSR1-CREM fusion gene. Tumour mutational burden was low with only one mutation per mega base pair. After a multidisciplinary team discussion, the working diagnosis was revised to an undifferentiated round cell tumour of the lung, most likely a sarcoma.Thus, the patient was commenced on neoadjuvant chemotherapy with Vincristine, Adriamycin and Cyclophosphamide (VAC) but shortly after, suffered massive hemoptysis requiring Intensive Care Unit (ICU) admission and repeated bronchoscopic intervention for hemostasis. She recovered and completed a total of five cycles of neoadjuvant chemotherapy, alternating between VAC and IE (Ifosfamide and Etoposide). An interval FDG PET CT suggested partial response with decreased FDG uptake in the right lung mass, right-sided pleural metastases and mediastinal adenopathy, and resolution of her right supraclavicular adenopathy.She then underwent an Extrapleural Pneumonectomy (EPP) and on pathological assessment, a 29-cm malignant solid epithelioid neoplasm with pseuodpapillary features, pleural nodules and positive hilar bronchovascular margins were noted.Approximately four months post-operatively, an interval FDG PET CT suggested possible disease recurrence as a FDG avid soft tissue mass in the right apical pleura with hypermetabolic foci in the right anterior, pericardiac, posterolateral and posterior pleura were seen. She was started on targeted therapy, Pazopanib with palliative intent. Two months later, re-staging CT scans showed disease progression with the right apical pleural mass and pleural nodules now larger. Pazopanib was stopped in view of lack of response and palliative radiotherapy to the lung cavity (50 Gy in 20 fractions through Tomotherapy) was delivered for temporary local control.68Gallium-DOTATATE imaging in rare tumours. 68Ga-DOTATATE PET CT scan was performed which showed uptake in the right apical pleural mass, subpectoral and lower paratracheal adenopathy that were above that of the liver .  study (CIRB Ref. No. 2018/2320) to assess the use of x 10.4). .Thereafter, she was treated with immunotherapy with dual check point inhibition (Nivolumab and Ipilumab) but despite nine cycles and palliative radiotherapy to the abdomen (24 Gy in three fractions through Volumetric Modulated Arc Therapy-Image Guided Radiotherapy), her tumour progressed locally in the mediastinum and distally with new abdominopelvic lymphadenopathy and bony pelvic metastases. After several multidisciplinary team discussions and institutional ethics board approval, the decision was made to attempt PRRT as an off label therapy as she had exhausted all available standard treatment options.177Lu-DOTATATE) over two months with mixed response as seen from her pre-treatment 18FDG-PET CT scan (She was treated with two cycles of PRRT (201.6 mCi and 180.1 mCi of  CT scan  and post CT scan .−1 from 11.2 g dl−1 pre-treatment) and thrombocytopenia (Platelet Count 30 × 109  l−1 from 389 × 109/L pre-treatment). In this time, she also required increasing amounts of supplemental oxygen and an interval FDG PET CT scan revealed overall progressive disease  fusion tumours are rare and can have very varied phenotypes including angiomatoid fibrous histiocytomas, peri rectal tumours, meningiomas and even unclassified tumours. There are five subtypes (SSTR1-5), expressed in varying amounts in different tissue types, such as the brain and leptomeninges, anterior pituitary, endocrine and exocrine pancreas, mucosa of the gastrointestinal tract, and cells of the immune system. SSTR2 has been identified in more than 70% of soft tissue tumours with positive uptake on scintigraphy and reverse transcriptase polymerase chain reaction. The presence of these receptors in osteosarcomas in particular confer a worse prognosis with a 0 vs 72% 4 year event free rate and 50 vs 76% 3-year overall survival but this has not been seen in other sarcoma subtypes.Somatostatin receptors (SSTRs) are G-protein-coupled plasma membrane receptors expressed in both normal and cancerous cells and their activation frequently results in inhibition of cell proliferation.e.g.,111In-Octreotide, 90Y-DOTATOC, 177Lu-DOTATATE, 213Pb- and 225Ac-DOTApeptide) has been shown to successfully relieve symptoms, shrink lesions, slow progression and improve quality of life and functional status. Radiolabelled somatostatin analogs bind to overexpressed SSTRs on the surface of NET cells, bringing specific tagged radionuclides which via β decay, exert tumouricidal effects. While conventionally PRRT was used in metastatic, inoperable NETs, with palliative intent, the role of PRRT in the neoadjuvant (converting inoperable metastatic lesions to operable ones) and in the adjuvant setting (post-operative treatment with PRRT) is currently being explored. PRRT with 177Lu-DOTATATE has been described to induce a durable (one year) partial response (50%) of lung metastasis in a SSTR2 positive synovial sarcoma that progressed despite six lines of conventional therapy. However, this is yet to be described in ESWR1 or ESWR1-CREM fusion tumours and SSTR2 maybe a targetable receptor in these rare tumours.In adult neuroendocrine tumours (NETs), peptide receptor radionuclide therapy (PRRT) with high-activity radiolabelled somatostatin analogues . Chest wall metastases appeared to respond the least with moderate residual FDG uptake over anterior and posterior aspects. PRRT could potentially be tried in patients with ESWR1-CREM fusion and SSTR2 positive tumours, especially when all other standard of care options have been exhausted.177Lu-DOTATATE.Irrespective of underlying histology, tumour SSTR2 positivity can select patients for PRRT with 177Lu-DOTATATE may have a more pronounced response in bone metastases as compared to soft tissue disease.PRRT with 177Lu-DOTATATE can be used with some benefit after patients progress through standard of care therapies.PRRT with


Cavernous malformations (CVMs) located in the thalamus are uncommon. However, they pose difficulties for resection because of their close proximity to eloquent areas of the brain and vascular structures, and all surgical corridors to access them are narrow. In this video, we report the case of a 19-year-old woman who presented with a long-standing history of right hemiparesis with recent deterioration. MRI revealed a large CVM located in the left thalamus, with signs of recent hemorrhage extending to the left cerebral peduncle. Resection was achieved with a paramedian contralateral supracerebellar infratentorial approach in a semisitting position, with an uneventful postoperative course.https://youtu.be/Arvu52FkHOE.The video can be found here:  However, DTI imaging was not available at that time.01:45 The semisitting position was chosen to allow a paramedian contralateral supracerebellar infratentorial approach, giving access to the cisternal portion of the left pulvinar. The operative site was marked with the aid of neuronavigation. Typical prepping and draping was performed.02:09 A posterior linear incision was made, and muscle dissection was performed through the midline in a relatively avascular plane. The suboccipital region and the posterior arc of C1 were exposed.02:26 A median suboccipital craniotomy with paramedial extension was performed, and venous sinus hemostasis was achieved with oxidized cellulose and fibrin glue.02:59 A C-shaped infratentorial durotomy followed, exposing both cerebellar hemispheres.01:13 Careful dissection of the pineal region was performed.03:47 With further dissection we reached the cisternal portion of the left pulvinar, where hemosiderin deposits were identified.03:57 The quadrigeminal cistern was identified, as well as the pineal gland, the vein of Galen, the left basal vein of Rosenthal, and the precentral cerebellar vein.04:17 The operative corridor was checked with the aid of neuronavigation, aimed at the cisternal portion of the left pulvinar.04:27 The lesion was exposed after white matter dissection. It had a typical cavernous malformation aspect.05:00 The lesion was gradually devascularized.05:21 We removed the cavernous malformation in a piecemeal fashion, using meticulous dissection and coagulation and avoiding excessive manipulation of the adjacent structures.06:14 After complete removal of the lesion, careful and thorough hemostasis was performed.06:20 It is generally reported that the PCCV can be sacrificed without any clinical symptoms, as it was necessary at the end of this case. In this final picture, it is possible to visualize the surgical corridor after dissection and removal of the cavernous malformation.06:42 The dura was closed in a watertight fashion, followed by sutures of layers of muscle, subcutaneous tissue, and skin.06:52 The patient had an excellent postoperative recovery.06:58 She underwent postoperative MRI 3 months later, with good results after surgery.07:06 Her motor strength returned to the baseline immediately after surgery, and showed even further improvement after 3 months, reaching grade 4+.


Thyroglossal duct cysts (TDCs) develop papillary carcinomas in very rare cases. Mostly, in such cases, the diagnosis is established after the excision of a clinically benign TDC. An anterior neck mass was found in a 43-year-old man with papillary carcinoma arising in a TDC. Clinical, radiological, and analytical controls are necessary for the management of papillary carcinoma in the TDC. There is no need for thyroidectomy unless there are palpable abnormalities in the gland or significant changes on an ultrasound. The outcome of the papillary carcinoma does not seem to be significantly impacted by routinely performed thyroidectomy. Around 7% of adults are affected by thyroglossal duct cysts (TDCs), a congenital anomaly . The tumA 43-year-old male patient, with no relevant personal or family history, presented to our department with complaints of odynophagia within two months of evolution, associated with the appearance and progressive growth of cervical swelling in the midline. The patient denied any previous similar episodes and presented no other complaints. On physical examination, the patient presented an anterior cervical swelling, approximately 3.5 cm of the greatest axis, with hard-elastic consistency and mobile, with protrusion of the tongue, suggestive of a TDC. No other signs were found on ENT examination, including neck palpation and laryngoscopy.Ultrasound and cytopathological examination (the latter performed through fine needle aspiration (FNA)) were highly suggestive of TDC diagnosis, with no evidence of malignant cells. Ultrasound confirmed the presence of normal thyroid gland tissue with no clinically detectable disease, and no cervical adenopathies were found. A pre-surgical CT scan was also performed, which demonstrated a well-defined midline infra-hyoid multicystic lesion, anterior to the pre-epiglottic space of the larynx, with slight capsular enhancement, in favor of TDC diagnosis Figure .Surgical removal of the lesion was performed according to the principles of the Sistrunk procedure: excision of the cyst, the middle portion of the body of the hyoid bone, and a core of tissue around the thyroglossal tract until the foramen caecum Figure . The surOn anatomopathological evaluation, the presence of papillary carcinoma was observed in a cystic lesion of the thyroglossal canal Figures , 4.The postoperative course was uneventful, with the resolution of symptoms and no complications. Figure In approximately 1% of congenital remnants of thyroglossal ducts, malignant transformation is detected . There m131 ablation and thyroid-stimulating hormone suppression is suggested [An evaluation of TDCs for primary carcinoma must differentiate between carcinomas of a clearly discernible TDC or tract, and carcinomas of thyroid glands ,11. In muggested . In thisTDC is the most common congenital anomaly related to the thyroid gland. In a TDC, papillary carcinomas typically present as swellings along the midline of the neck, which move with deglutition and tongue projection. A very low percentage of all TDC cases present with papillary carcinomas. This type of pathology has a good prognosis and can be successfully managed with cyst removal (Sistrunk procedure) and careful follow-up. Thyroidectomy, neck lymph node dissection, or adjuvant treatments are not required in most cases.


Sarcomas in the head and neck area are rare diseases with an incidence of under 1% of all head and neck malignant tumours. Osteosarcomas or osteogenic sarcomas consist of neoplastic cells that produce osteoid bone or immature bone. Sarcomas develop more in the mandible than the maxilla. The exact diagnosis of different types of sarcomas is based on the immunohistochemical investigation. These rare tumours are of mesenchymal origin; osteosarcomas and chondrosarcomas are the most common types—Ewing’s sarcomas. The use of proton beam radiotherapy in the treatment of osteosarcoma of the maxilla is rarely reported in the literature. We present a case of successfully treated (surgery and proton beam radiotherapy) poorly differentiated highly aggressive osteosarcoma in the ethmoids and maxillary sinus and morbidity after the treatment. We were presented with a case of a 65-year-old man with anaesthesia and palsy of the right face. The stomatology department performed the extraction of a tooth. One month later, the wound was still open. The histology showed an osteogenic sarcoma in the area of the wound. The oncologist and maxillofacial surgeons in a catchment hospital recommended a nonsurgical approach. Hence, we performed a radical maxillectomy and ethmoidectomy, after which we continued with proton bean radiotherapy. The patient is now five years after therapy without signs of sarcoma; however, he has blindness in the right eye and reduced vision in the left eye, as well as gliosis of the brain, vertigo, tinnitus, trismus, and ancylostomiases. Head and neck osteosarcomas treatment is considered a complex multidisciplinary task. It is currently argued that there is no clear therapeutic protocol for successful treatment. Innovations in treatment modalities such as proton beam radiotherapy appear to have potential, although their effects on long-term morbidity and survival outcomes are still undetermined. We present a rare case report of an osteosarcoma of the maxilla involving an innovative, successful treatment procedure combining surgical excision followed by proton beam radiotherapy. This treatment approach may enable maximum tumour control. This protocol has not been adequately described in the world literature for this diagnosis. Osteosarcoma  is a malignant tumour composed of osteoid bone or immature bone cells. Osteosarcomas account for less than 7% of all bone sarcomas. In the head and neck area we can find fibrosarcomas, chondrosarcomas, and osteosarcomas as frequent sarcomas, and more rarely leiomyosarcomas, liposarcomas, and rhabdomyosarcomas. The exact diagnosis of different types of sarcomas is based on the histology and immunohistochemical investigation of the expression of different proteins. These rare tumours are of mesenchymal origin; osteosarcomas and chondrosarcomas are the most common types—Ewing’s sarcoma. Compared to the clinical presentation of osteosarcomas of the long bones, osteosarcomas of the jaw occcur in an older patient population (30–50 years). The mandible is more often affected than the maxilla .The Enneking classification with UICC is used for osteosarcomas 7th ed., 2011) [011 [2]. The treatment of the sarcoma is based on radical surgery, irradiation of the tumour origin and/or lymph nodes, and chemotherapy if needed. The treatment of sarcomas is difficult, as there are only moderate data about the survival of the patients and disease-free interval. The determination of treatment depends on the exact diagnosis, large-cell typing of the sarcomas, and the low incidence of these patients in the population . The surThe use of proton beam radiotherapy in the treatment of osteosarcoma of the maxilla is rarely reported in the literature.The moderate quality of life with the radical therapy of sarcomas is described in literature, too. The treatment of sarcomas is difficult in clinical practice because of its low frequency in the population. Many papers refer to low numbers of treated patients and/ or described case reports only . Early dWe present a case of successfully treated (surgery and proton beam radiotherapy) poorly differentiated highly aggressive osteosarcoma in the ethmoids and maxillary sinus with survival data, disease-free interval, local recurrence, metastases, and morbidity of one patient. This patient has survived over 5 years—overall survival interval (OS). This case report describes the morbidity and complications of the surgery and proton beam therapy. The patient signed an informed consent and gave his consent for publication.A 65-year-old man (ethnic group—Western Slavic origin) was referred to the Otorhinolaryngology outpatient clinic of the Department of Otorhinolaryngology and Maxillofacial Surgery in the Military University Hospital Prague, 3rd Faculty of Medicine of the Charles University Prague with 3 months of difficulty swallowing and pain in the right face that was treated by extraction of tooth 18 (only). He did not smoke and consumed a small amount of alcohol—200 mL wine each second day.A tumour mass was found in the gingival arch where tooth 18 was extracted. The CT scan showed an enlargement of the tumour mass spread into the maxillary bone see .The maxillary sinus was completely filled by the tumour mass, which spread into the floor of the orbit and lateral nasal wall. A sample was taken from the tumour mass by the stomatology outpatient department. A histopathological diagnosis of an undifferentiated sarcoma was made. Radical surgery was the next step in the treatment protocol. We performed a total maxillectomy with partial orbital resection, resection of the zygomatic complex, ethmoidectomy, and complete resection of medial nasal wall with conchae on the right side. The extension of the surgical radicality was focused on the radical resection of the whole tumour tissue with an R0 margin. The wound was closed by a face flap. No surgery was performed on the neck lymph nodes, because there were no suspicious lymph nodes detected by imagining methods. The histological analysis revealed a sarcoma with detection of a fascicular or storiform structure of cells. The cells were irregular and hyperchromic with multiple and/or polymorphic nuclei.There were also large atypic cells associated with high mitotic activity and atypia of mitotic activity (25/10HUF). These cells are highlighted by black arrows in The neoplastic cells did not express desmin, MSA, or CD34; the S-100 protein was positive in arterials including CD31. The immunobiology for MDM2 and CDK4 were positive. This sign was typical for liposarcomas and osteosarcomas. Hence, the diagnosis of a poorly differentiated highly aggressive osteosarcoma was made see .One month later, the patient had a recurrence of the lesion in the ethmoid region, with a size of 4 × 5 × 6 mm. After a multidisciplinary discussion made by an oncologist, otorinolaryngologist and radiation oncologist, the resection of this tumour was performed by an endoscopic approach, and external proton beam radiotherapy was used post-surgically.Proton Beam RadiotherapyEight weeks after the oncological surgery, proton beam radiotherapy was performed in the Proton Therapy Centre Czech Republic in Prague. Full doses of proton beam therapy were applied to the region of the tumour, with a focus on the maxilla and ethmoidal part on the right side. The right eyeball was spared during the therapy. A skin defect in the face flap developed during the therapy, which caused prolonged healing for the following seven weeks. The target volume was the paranasal sinuses tumour right side + rim. The dose applied (CGE) was 70 Gy, with 35 fractions, and the dose per fraction (CGE) was 2 Gy.The radiation technique was proton therapy using the pencil beam scanning technique See .In terms of the organs at risk-dose: glandula parotis right side D mean 4.89 Gy, cochlea right side D mean 1.4 Gy, nervus opticus right side D max 48.4 Gy, nervus opticus left side D max 41.7 Gy, chiasma D max 47.4 Gy, retina right side D max 47.8 Gy, retina left side D max 30.6 Gy, brainstem D max 4.1 Gy, spinal cord D max 0.3 Gy, right eye 48.6 Gy, and left eye 31.6 Gy.Treatment was included for advisory dermatitis grade 2, stomatitis grade 2, soor, and conjunctivitis grade 1.We observed the patient for the next five years. Two years after the start of the therapy, the right eye was blind. Five years later, the patient presented with a decline in the function of his left eye. The patient showed structural changes in the brain on the left side-the MRI showed large gliotic parts in the frontal and parietal part of the brain see .Ancylostomiases and trismus started in the first year following the proton therapy. The patient could open his mouth 6–7 mm only, which influenced his nutritional status. The patient ingested minced food and beverages. However, his weight remained stable for five years.Postoperatively, the patient was referred from the otorhinolaryngologic surgeon to the oncologist. Regular MRI follow-up examinations were performed every 6 months. A CT scan of the lungs was performed once a year. A PET-CT was indicated in the year 2021 (4 years after proton beam radiotherapy). None of the images revealed any evidence of locoregional cancer recurrence or metastatic involvement. No local recurrence was observed (OL 0); the overall survival time has been 6 years as of the date of publication (OS 6 years); the disease free interval has been 5 years and 6 months, and no metastases were observed (MT 0).Many case reports of osteosarcomas of the jaws are described, including recommendations for diagnostic and therapeutic management. The main reason for this report was the successful treatment of osteosarcoma with surgery followed by proton beam radiotherapy. The use of proton beam radiotherapy in the treatment of osteosarcomas of the maxilla is not adequately described in the literature.Osteosarcomas in the head and neck region are rare, difficult to diagnose, and may present with varying signs and symptoms . OsteosaMaxillary osteosarcomas usually develop in people between 20 and 40 years of age. In our case report, the patient was a 65-year-old man. In contrast, the case report of French authors described a case of osteosarcoma of the maxilla of a 17-year-old patient .Due to the anatomical location of maxillary osteosarcomas, dentists are the first to assess the lesion in 45% of cases. Unfortunately, tooth extraction occurs in two-thirds of cases . These lCT and/or magnetic resonance imaging are recommended for maxillary tumours. These imaging methods are necessary to assess the exact description of the spread of the tumour into the maxillary bone, ethmoids, and infratemporal fossa. These facts are important for planning the resection of a tumour as well as for reconstruction. Diagnostic ultrasound can be used for the lymph nodes on the neck, which can be associated with the tumour only. The CT and MRI criteria typical for osteosarcoma were summarized by Luo et al. .Chen et al. described a prognostic factor for osteosarcoma. They discussed that histological grade and negative margins were significant factors affecting the survival of patients . For sarClinical investigation of all parts of the head and neck (endoscopy to provide evidence of tumour).A tissue sample can be taken, if it is a safe procedure.CT, MRI scan , if necessary, PET scan, and USG.Histopathology: complete investigation and extensive immunohistochemistry tests are necessary.Many authors recommend a diagnostic set including:The treatment of osteosarcomas combines two modalities: surgery and beam therapy ,12. Laskar et al. evaluated the efficacy and toxicity of dose-escalated IGI modulated radiation therapy in osteosarcoma. The five-year local control improved the survival in 66% ,13.A database collected 821 patients with osteosarcoma (HNOS) treated by neoadjuvant chemotherapy and radical surgery and adjuvant chemotherapy. The authors described no benefit in perioperative chemotherapy or radiation therapy .However, Brady et al. found a higher 5-year survival rate in paediatric patients with surgery treatment compared to radiotherapy alone .Kalvarezos and Sinha  providedSufficient data for proton beam radiotherapy currently do not exist. That is why we added our experience with the treatment of osteosarcoma in our patient. These data can improve the knowledge of proton beam therapy’s effect, namely good survival data and a moderate quality of life. In the literature, the use of proton beam therapy for maxillary sarcomas has been described in only one case report- Ewing sarcoma family tumours-from Japan, where a 4-year-old patient was treated with chemotherapy followed by partial maxillectomy. After surgery, proton beam radiotherapy was performed. Partial resection combined with proton beam therapy may enable maximal tumour control and minimal functional and cosmetic side effects .Netherlands authors mentioned that they did not use chemotherapy for their patient.The reason for that was the moderate data on its effect on osteosarcomas . The stuSurgery: surgical therapy must be radical; the surgical margins are very important for grading of sarcomas in general.Radiotherapy: beam therapy  depends on staging, including grading, histological type of sarcoma, and the radicality of surgery when performed.Chemotherapy: for aggressive histological subtypes of osteosarcoma, chemotherapy is necessary in many cases, a combination of vincristine, doxorubicin, cyclophosphamide, and etoposide have been used.Treatment:A clinical report including endoscopic methods, Narrow band imaging endoscopy (NBI) and Ultrasonography (USG) every 4 months;MRI, once yearly (PET scan if necessary).Follow-up for sarcoma:Some authors have stated that there is no standardized staging system for soft-tissue sarcomas of the head and neck area ,20. So fCurrent knowledge of osteosarcoma of maxilla:Osteosarcoma of the maxilla is a rare disease, that is mainly reported in the literature in the form of single case reports. Diagnosing osteosarcoma is very challenging and osteosarcoma is often misdiagnosed as osteomyelitis due to the nonspecificity of its symptoms upon initial presentation ,23,24.The vast majority of authors emphasise the importance of surgical resection of the lesion with clean margins.The management of head and neck osteosarcomas comprises a multi-disciplinary approach ,25.Portuguese authors have reported that surgical resection of a lesion with clear margins followed by chemotherapy is associated with the best prognosis for osteosarcoma of the jaw .Indian authors have discussed that osteosarcomas of maxillofacial region pose difficulties in obtaining tumour-free margins due to their complex anatomy and close proximity to the cranium. Surgery may be complemented by radiotherapy with or without chemotherapy . An American retrospective review of the literature on sinonasal sarcomas (over the last 30 years) reported a significant association between overall stage and overall survival on univariate and multivariate analysis, and overall stage appeared to be the most significant contributor to survival. Surgical excision with negative margins, when possible, combined with adjuvant radiation and/or chemotherapy, appears to offer the best survival outcomes .Japanese authors have reported the treatment protocol in a case report : chemotherapy followed by partial maxillectomy and after surgery, proton beam radiotherapy was performed . Authors from the United Kingdom have reported that heavy-particle therapy (proton beam) in combination with surgery is increasingly being used to treat otherwise unresectable diseases, particularly in children .The role of proton beam radiotherapy is promising, but to our knowledge no long-term data currently exist .Increasing studies have proved that the imbalance of the immune system and tumour immunity is a vital factor leading to the pathogenesis and deterioration of osteosarcoma ,27.It is currently argued that there is no clear therapeutic protocol for successful treatment, and that research into the pathogenesis and underlying molecular mechanisms of osteosarcoma is necessary to achieve new breakthroughs in osteosarcoma therapy ,27.A new study from China shows that CBR3-AS1 plays an oncogenic role in osteosarcoma through the modulation of the miR-140-5p/DDX54-NUCKS1-mTOR signalling pathway network .Head and neck osteosarcomas treatment is known as a complex multidisciplinary task. It is currently argued that there is no clear therapeutic protocol for successful treatment ,27.Surgical excision with negative margins when possible combined with adjuvant radiation and/or chemotherapy appears to offer the best survival outcomes ,17,24,25Innovations in treatment modalities such as proton beam radiotherapy appear to have potential, although their effects on long-term morbidity and survival outcomes are still undetermined.We presented a rare case report of an osteosarcoma of the maxilla involving an innovative successful treatment procedure combining surgical excision followed by proton beam radiotherapy. This treatment approach may enable maximum tumour control. This protocol has not been adequately described in the literature for this diagnosis. Still, the radical treatment protocol is associated with comorbidities and decreasing quality of life.


Appendiceal diverticula present as rare clinical finding and are most confused with acute appendicitis. A 65-year-old female was presented to our surgical service after 1 day of right lower quadrant abdominal pain and a computed tomography evaluation, read as a diagnosis of acute appendicitis. Due to the location and quality of pain and intraoperative findings acute appendicitis was our preliminary diagnosis. Follow up with histopathology confirmed acute inflammation of an appendiceal diverticulum. With discordance in original diagnosis from final pathological evaluation, we suggest appendiceal diverticula as an important differential to consider in patients of similar clinical presentation. Surgical treatment with appendectomy and final histological diagnosis are essential in the proper treatment of this rare clinical finding. Appendiceal diverticula are confirmed as an incidental pathologic finding rarely reported amongst cases involving appendectomies with similar clinical presentation. It is frequently confused with acute appendicitis . We presA 65-year-old female with a past medical history of hyperlipidemia presented to the emergency department with 1 day of right lower quadrant (RLQ) abdominal pain. These symptoms were confirmed on physical examination as moderately tender to palpation. Clinical assessment was otherwise unremarkable. Yet, a computed tomography (CT) was obtained confirming high suspicion for acute appendicitis with an enlarged appendix of 9 mm and associated with wall enhancement and evidence of fat stranding see .Given the clinical assessment and CT findings, the patient was taken to the operating room for a laparoscopic appendectomy, which was uncomplicated. During the procedure, copious adhesions were noted in the lower midline involving the omentum. The appendix was found to be edematous and hyperemic with no perforation. Pathological analysis demonstrated focal acute inflammation of the mucosa with a normal appendiceal wall see . PatientWe present a rare case of appendicitis with diverticulosis. The incidence in such cases is reported at a rate no greater than 1%. Diverticula of the appendix are commonly classified as congenital or acquired. The former being significantly less common. It is most found in men over the age of 30, which is not the typical demographic for acute appendicitis . Very feDiverticula of the appendix are commonly identified incidentally post appendectomy for patients with a clinical presentation of acute appendicitis . The LipInflammation may be more common with diverticulosis due to the structural change of the appendix . When noSeveral studies have noted association between appendiceal diverticula and epithelial neoplasia of the appendix, placing greater importance on post appendectomy specimen pathology for patients who are found to have incidental appendiceal diverticula , 3, 4. FAppendiceal diverticula present as rare clinical findings and may often present similarly to acute appendicitis , 5. The


Next, the donor tooth was transplanted into the created socket (#45), and stabilized using an enamel bonding agent. The wet pDDM was loaded into the location of the congenital missing tooth, and the flap was repositioned. The bonding agent for stabilization was removed at 28 days, and also small contact points between the transplanted tooth and the upper premolar (#14) were added using photopolymerizable composite resin. X-ray photos were taken sequentially, and there were no postoperative complications. The radiographic images showed that the periodontal ligament space and alveolar ridge line could be observed at 18 months. The pDDM was harmonized with the mandible, and the remodeled bone-like shadow was observed in the graft region. We concluded that immediate tooth transplantation with root canal fillings and autogenous pDDM may be a valuable alternative to dental implanting or bridge formation for patients with a congenital missing tooth, followed by orthodontic treatment.This clinical report describes immediate tooth auto-transplantation with an autograft of partially demineralized dentin/cementum matrix (pDDM), based on an orthodontic treatment plan for a 16-year-old male patient with a congenital missing tooth (#45). First, vital teeth  were extracted, and root canal filling (#14) was immediately performed with the support of a fixation device. Simultaneously, the tooth (#24) was crushed in an electric mill for 1 min, and the crushed granules were partially demineralized in 2% HNO Tooth autotransplantation is a well-established surgical procedure for the replacement of missing or unrestorable teeth ,2. The sThis clinical report describes autotransplantation of a premolar with complete roots, simultaneously with immediate RCF and autograft of a partially demineralized dentin matrix (pDDM), based on the orthodontic treatment for a 16-year-old male patient with a congenital missing tooth. Both the tooth-fixing device for protection of the periodontal ligament cells and the mill for the pDDM preparation contribute to a new dental procedure for immediate tooth transplantation and towards the quality of life of patients. The aim of this study was to evaluate the radiographic evidence after autotransplantation of a tooth, with immediate RCF and partially demineralized dentin matrix (pDDM) granules, into the missing tooth region using the support of a newly developed tooth-fixing device and an electric mill. The radiographic evidence highlighted that the periodontal ligament space and alveolar ridge line could be observed at 18 months after the simultaneous and immediate tooth autotransplantation with RCF and pDDM.A 16 year-old-male was introduced from a private orthodontic dental clinic to our dental unit in 2011. The patient presented with a congenital missing lower left second premolar (#45), examined on initial panoramic X-ray photo . He had Based on examinations, including computed tomography (CT), the surgical treatments were planned in 2012, as follows: Distance between alveolar crest and mandibular canal: 19 mm. Upper second premolars : length 15mm, orthodontic extraction. The right premolar (#14): immediate RCF and transplantation into #45. The left premolar (#24): pDDM graft for bone augmentation.®, WiSM Mutoh Co., Ltd., Tokyo, Japan)  and sealer  immediately, with a device using a rubber dam for transplantation  were extracted using forceps A under l, Japan) B, which , Japan) C, and thantation D. The ex2) vessel with ZrO2 blade at 12,000 rpm for 1 min using a mill  (3 solution for 20 min. The pDDM granules including cementum were extensively rinsed in cold distilled water (D.W)  A–C. The , Japan) D were imer (D.W) E.®, Dentsply Sirona Co., Ltd., New York, NY, USA) was applied for the enlargement of the transplant cavity (#45). Cortical perforations were performed into the dense mandibular bone. The donor tooth (#14) after RCF and the removal of a part of crown portion was transplanted atraumatically into the cavity . Then, pDDMs were loaded onto the places on the mandible around the transplanted tooth  for only 3 days. The nylon threads were removed at 10 days after the surgery. The bonding agent for stabilization was removed at 28 days, and also slight contact points between the transplanted tooth and the upper premolar (#14) were added using photopolymerizable composite resin .A full-thickness mucoperiosteal flap was elevated, and the drill of the dental implant system  can protect periodontal ligament cells from debris and drying out during endodontic treatments, as shown in Our unique tooth-fixing device  than a pDDM. Human dentin/cementum granules after 2% HNOuman DDM . Fortunauman DDM . The resuman DDM . Our teaThe preservation of periodontal ligament cells is considered to be critical for the success of a transplanted tooth ,5,6. HowRegarding the technique of autotransplantation of mature teeth, the extraoral root-end resection of teeth with complete root formation showed promising outcomes for transplants, especially with a single root canal and uncomplicated root morphology . For forThree types of root resorption after tooth autotransplantation are classified, as follows: surface resorption, inflammatory resorption, and replacement resorption . OrthodoCollagen derived from cow or pig has been a commercially available organic material for medical use since the 1970s in Japan ,19. AutoHorizontal or vertical augmentation is a challenging field in bone regeneration. In this case, granules of pDDM were used for bone augmentation. In addition to granules, block, ring, and plate forms of pDDM can be prepared for bone regeneration ,27,28. IBased on dentin science and doctor’s ideas, dentin/cementum materials should advance bone regenerative therapy as highly acid-insoluble scaffolds for patients, in the near future.The clinical and radiographic examinations showed that the autotransplant of a premolar (#14) with immediate RCF and pDDM graft into the congenital missing tooth region (#45) was successfully performed, to reconstitute the occlusion. The radiographic evidence highlighted that the periodontal ligament space and alveolar ridge line could be observed at 18 months after the immediate autotransplantation with RCF and pDDM. The tooth-fixing device and the electric mill contributed to enabling the immediate procedure for autotransplantation. A slight root surface resorption of the transplanted tooth might cause an orthodontic rotation.We conclude that immediate tooth transplantation with RCF and autogenous pDDM should be a valuable alternative to a dental implant or bridge formation for patients with a congenital missing tooth followed by orthodontic treatment.


Melanoma is the most aggressive form of skin cancer, with a tendency to metastasise to any organ of the human body. While the most common body organs affected include liver, lungs, brain and soft tissues, spread to the gastrointestinal tract is not uncommon. In the bowel, it can present with a multitude of imaging appearances, more rarely as an aneurysmal dilatation. This appearance is classically associated with lymphoma, but it has more rarely been associated with other forms of malignancy.We report a case series of three patients with aneurysmal dilatation in the small bowel (SB) confirmed to be due to metastatic melanoma (MM). All patients had non-specific symptoms; most times being attributed initially to causes other than melanoma. On CT the identified aneurysmal SB dilatations were diagnosed as presumed lymphoma in all cases. In two cases, the aneurysmal dilatation was the first presentation of metastatic disease and in two of the cases more than one site of the gastrointestinal tract was concomitantly involved. All patients underwent surgical resection with histological confirmation of MM.Recognition of unusual SB presentation of MM, such as aneurysmal SB dilatation, is important to expedite diagnosis, provide appropriate treatment, and consequently improve quality of life and likely survival of these patients. As the most common cancer to metastasise to the SB and as a known imaging mimicker, MM should remain in any radiologist’s differential diagnosis for SB lesions with aneurysmal dilatation.The online version contains supplementary material available at 10.1186/s40644-022-00463-5. Different small bowel (SB) imaging patterns have been associated with metastatic melanoma (MM). More frequently, it presents with polypoid nodules  causing Melanoma is the most aggressive form of skin cancer, with a tendency to metastasise to all organs of the human body. It is the most common metastatic tumour to the SB , 11. IneCorrect and timely recognition of the different imaging patterns of SB MM can optimise and expedite patient care by guiding diagnosis, treatment and prognosis. Early diagnosis of SB metastases whilst small in size, can facilitate elective treatment and avoid non-elective hospitalisations. Here, we report the radiological and pathological findings of a series of patients found to have aneurysmal dilation of the SB due to MM, an imaging finding usually associated with lymphoma or SB primary tumours, which have different clinical management.An 80-year-old gentleman presented with a history of weakness, fatigue, breathlessness and chest pain, which he initially attributed to an episode of food poisoning. His past medical history included previous thyroidectomy for a thyroid nodule and radiotherapy-treated prostate cancer. A full blood count demonstrated a mild iron-deficiency anaemia. A CT scan of the chest, abdomen and pelvis was arranged, which identified a large cavitated aneurysmal mass arising from the ileum within the pelvis, adjacent to the sigmoid colon Fig. . A furthThe patient underwent oesophago-gastro-duodenoscopy, which found the duodenum to be replaced by tumour. Biopsies of this lesion revealed infiltration of the lamina propria and submucosal by tumour cells. Immunostaining showed the tumour to be compatible with metastatic melanoma with expression of melan-A, SOX10 with negative CKMNF , CDX2  melanoma excised from his right cheek 5 years previously, for which he declined any further treatment or follow up.On further questioning, the patient had had a pT4a  on his right auricle resected six years before, for which he had been discharged from routine surveillance one year before.Review of his past medical history revealed a prior diagnosis of pT2a melanoma . She underwent wide local excision and sentinel lymph node biopsy of left axilla. Two years later, surveillance imaging identified a solitary lung metastasis, which was treated by video-assisted thoracic surgical lingulectomy. Seven months later, she developed more widespread metastases involving the occipital lobe of the brain and the right adrenal gland. She received stereotactic radiotherapy to the brain lesion and two cycles of ipilimumab and nivolumab immunotherapy. Her immunotherapy was discontinued following grade 3 colitis which required two hospital admissions, intravenous methylprednisolone and infliximab. She remained well and in remission for the next 2 years.A 65-year-old lady presented with a pT3b melanoma in the left posterior upper arm (Breslow thickness 2.4 mm, mitotic index 8/mmHowever, when symptoms of diarrhoea, abdominal pain and fatigue began 12 h after receiving her flu vaccination, the symptoms were initially considered to be a flare of immune-related colitis. Her symptoms improved transiently with oral steroids and antidiarrhoeal medication. The patient went on to have a flexible sigmoidoscopy, which was limited to the proximal sigmoid due to patient discomfort. The visualised mucosa and obtained mucosal biopsies were normal. Four months later, the patient was admitted to the hospital with worsening of abdominal pain and diarrhoea, where she had an urgent CT scan of the abdomen and pelvis Fig. . The scaFurther clinical information of all cases can be found on the supplementary table Gastrointestinal spread from MM is relatively common, with the SB representing the most common site of involvement , 15. HowThe clinical presentation of SB MM is usually non-specific, including a constellation of symptoms associated with GI tract pathology, including abdominal pain, unexplained weight loss, iron-deficiency anaemia, change in bowel habit and GI bleeding, though a large proportion may be asymptomatic. Metastases are more frequent in the SB than primary tumours and may occur by haematogenous spread, local extension or intraperitoneal seeding . MelanomThe diagnosis of SB MM remains challenging due to its vague clinical presentation and limited endoscopic access. Most lesions are identified during surveillance imaging, or incidentally in patients having routine or urgent scans for other reasons. As imaging patterns of SB involvement can be diverse and mimic several other conditions, it is important to keep MM as a possible differential in patients with a prior history of melanoma, even when unusual features, like aneurysmal SB dilatation, are present. In all 3 cases, the initial radiological diagnosis was given as lymphoma, even when secondary imaging clues were present which could have helped raise concern for MM. In one of the cases, the presence of a small number of localised large necrotic lymph nodes close to the main lesion could have prompted the radiologist to consider a different diagnosis besides lymphoma, as necrotic lymph nodes are uncommon in untreated lymphoma . EquallyInterpretation of staging CT studies of patients with MM can be challenging given the need for a thorough review of the scans and unpredictable metastatic patterns with this disease. Nevertheless, the bowel is an important review area in these patients, particularly in the case of primary cutaneous melanoma arising in the head and neck region, trunk and lower extremity . On the In the case of a suspected SB lesion and/or in the presence of symptoms, video-capsule is still the preferred method for non-stenotic SB lesions . HoweverModern therapeutic interventions for MM have radically expanded in the last decade and significantly improved overall survival of patients, some able to enter long term remission . AlthougRecognition of unusual radiological signs of SB spread from MM, like aneurysmal SB dilatation, is important to expedite diagnosis, provide appropriate treatment, and consequently improve quality of life and likely survival of these patients. As the most common cancer type metastasising to the SB and as a known imaging mimicker, MM should remain in any radiologist’s differential diagnosis for SB lesions. Not uncommonly, there are other imaging features which can help make the correct diagnosis.Additional file 1.


Dermatofibrosarcoma protuberans is a low-grade cutaneous sarcoma typically located on the trunk or proximal extremities. Less common locations include the head, face, and neck area. This tumour is slow growing with variable clinical appearance. It is known for its locally invasive nature and low metastatic propensity. Because imaging findings are rather nonspecific, biopsy is needed for definite diagnosis. This case describes an unusually large example of dermatofibrosarcoma protuberans in the less common preauricular region. Dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive sarcoma of the skin that ranges in presentation from a small firm subcutaneous nodule over a coalescent indurated plaque to a giant exophytic nodular mass, depending on the growth duration . The meaA 36-year-old African man presented to the emergency department (ED) with a large right-sided facial mass causing massive facial disfigurement. The patient's right ear was displaced superiorly while his mouth was deviated to the left . The fouContrast-enhanced computed tomography (CT) of the head and neck showed an exophytic and lobulated mass  centred at the right preauricular region. It appeared as a moderately hypoattenuated lesion with moderate contrast enhancement without calcifications. In addition, a mass effect was shown on the surrounding tissues with bone scalloping of the right posterior mandibular ramus, the external auditive canal, and the mastoid, all of which are indicative of a slow-growing process. CT angiography showed multiple branches of the external carotid artery circumferentially encased by tumoral tissue. The right upper lobe showed findings compatible with an active open tuberculosis (TB), which most likely was causing the fever in this patient.On magnetic resonance imaging (MRI), the tumour appeared hypointense on T1-weighted imaging and moderately hyperintense on T2-weighted imaging and showed marked homogeneous contrast enhancement with peripheral zones of necrosis Figures . The adjPositron emission tomography with CT showed F-18 fluorodeoxyglucose accumulation in the right facial tumour and in the right upper lobe (due to TB). However, there were no signs of metastasis.Based on the imaging findings and the location of the tumour, we posed a differential diagnosis consisting of chronic tuberculous lymphadenitis, plexiform neurofibroma, liposarcoma, DFSP, and giant pleomorphic adenoma. Histopathological examination showed an unencapsulated but well-defined nodule consisting of monomorphic spindle cells in a storiform pattern. Immunohistochemistry for CD34 was strongly positive. These findings revealed the diagnosis of DFSP.Given the tumour's massive size and proximity to important structures, the patient underwent a debulking excisional surgery, a mass of 2.8 kg tumorous tissue was removed in total . The tumA second reconstruction using a split-thickness skin graft from the right thigh was used to cover the remaining defect after two weeks of granulation of the original wound bed . The resOur case describes a 28 cm diameter DFSP in the head-and neck area of a 36-year-old black male from African origin. Incidence rates have been described as nearly twice as high in black people compared to white people . Its mosDFSP is a slow-growing tumour that usually starts as an asymptomatic firm subcutaneous nodule or a coalescent indurated cutaneous plaque but can progressively turn into a more lobulated, exophytic mass. DFSP can arise in healthy skin or in areas exposed to repeated trauma, vaccination, radiation, or scarring. At later stages, ulceration and bleeding of the tumorous tissue are possible, and the invasion of adjacent fascia, muscles, and bone has been described. The latter was also observed in our case. Although DFSP is typically locally aggressive, metastasis is rare and occurs in only less than 5% of the cases, typically spreading to the lungs , 3.Imaging findings of DFSP are rather nonspecific. According to Millare et al., CT demonstrates an isoattentuated to hypoattenuated lesion without calcifications and variable contrast enhancement depending on its size. Due to the slow-growing nature of the tumour, the adjacent underlying bone will remodel rather than get aggressively destroyed. For preoperative reasons, radiologists should mention any invasion of the deep head and neck structures in the radiological report. MRI findings include a hypointense signal on T1-weighted imaging and a hyperintense signal on T2-weighted imaging with uniform contrast enhancement on T1-weighted imaging after administration of gadolinium, similar to that seen in our patient [The main differential diagnosis of DFSP consists of dermatofibroma, leiomyosarcoma, Merkel cell carcinoma, keloid, or other soft tissue tumour. Due to the atypical presentation of our case and his positive TB status, our differentials also included chronic tuberculous lymphadenitis, plexiform neurofibroma, liposarcoma, and giant pleomorphic adenoma. A more heterogeneous aspect of the tumour with central cystic changes would be expected in chronic tuberculous lymphadenitis. The absence of the diagnosis of NF-1 in our case makes the diagnosis of plexiform neurofibroma less likely, though not impossible . Lastly,DFSP is a histopathological diagnosis and cannot be made solely on the base of clinical appearance and imaging. Histopathological examination typically shows spindle cells which are irregularly organized in linked fascicles with a storiform arrangement. Furthermore, the spindle tumour cells are positive for CD34 and vimentin on immunohistochemistry .The gold standard treatment of DFSP should aim for complete surgical excision of the lesion by means of wide local excision or MOHS. However, these treatment options were not feasible in our massive and complicated case. First, the head and neck region does not easily allow for wide local excision due to the presence of important surrounding structures such as the carotid artery or base of skull. Second, the enormous size of our patient's tumour warrants debulking rather than curative surgery and therefore largely excludes the option of MOHS. In brief, the challenge in our case's surgical planning was to find a balance between maximal tumour tissue resection and achieving a structural and aesthetical acceptable result despite significant perioperative soft tissue loss. Curative radiation therapy should be offered in the case of unclear surgical margins, as in our case. Adjuvant radiotherapy should also be offered in cases of free surgical margins to prevent early local disease recurrence , 8, 9. IDFSP in the head and neck region is characterised by a slow-growing pattern, locally aggressive in nature with high recurrence rates, and low malignant potential. DFSP is usually hyperintense at T2-weighted imaging with marked enhancement at T1-weighted imaging. The current case stands out because of its massive size and unusual off-midline location at the preauricular area. Radiologists should be familiar with the imaging appearance of this entity and include DFSP in the differential diagnosis of head and neck cutaneous tumours with similar characteristics.


Hemoculture tests and urine analysis were negative for infection. A contrast chest computed tomography revealed a mass measuring 5.5 × 4.5 cm, occupying the left atrium cavity. The patient underwent surgical excision of the mass, however, surgical margin of the resected tumor could not be evaluated, due to the multifragmented nature of the resection specimen. Postoperative CRP and leukocyte levels normalized, highlighting the relationship between the tumor and the inflammatory status. Early diagnosis is crucial for a proper management and favorable outcome, enabling patients to undergo chemotherapy and achieve complete surgical resection.Cardiac undifferentiated pleomorphic sarcoma (UPS) associated with fever and inflammatory response is an extremely rare condition. Herein, we report a rare case of cardiac UPS with unusual clinical presentation and inflammatory response. A 67-year-old male complaining of progressive dyspnea and intermittent fever of unknown cause was referred to our hospital for surgical resection of a left atrial mass. Laboratory analysis showed leukocytosis (26 × 10 Cardiac sarcomas are rare clinical entities, with high metastatic potential and aggressive clinical behavior associated with a poor survival rate .Cardiac undifferentiated pleomorphic sarcoma (UPS) associated with fever and inflammatory response is an extremely rare condition .Given the rarity of cardiac UPS, the locally advanced tumor at the time of diagnosis as well as the nonspecific clinical presentation, available data about cardiac UPS are limited.Cardiac UPS typically develop in the left atrium (LA) and invade the atrial and ventricular walls with an infiltrative growth pattern, resulting in mitral valve dysfunction and subsequent left-sided heart failure .The efficacy of current treatment is unclear. The protocol includes complete surgical resection if possible, followed by adjuvant chemotherapy and/or radiation therapy, to reduce the risk of recurrence .This study reports a rare case of cardiac undifferentiated pleomorphic sarcoma with unusual clinical presentation and inflammatory response.A 67-year-old male patient with a history of progressively worsening dyspnea, fatigue, and intermittent fever of unknown cause, was late referred to a cardiologist.The patient had a previous history of atrial fibrillation, stroke, and chronic kidney disease.3/μL) with a neutrophil-lymphocyte ratio of 20 and high C-reactive protein (CRP) levels (155.4 mg/L), and a normal value of procalcitonin (<0.5 ng/mL). Large spectrum antibiotic therapy was initiated as bacterial infection was suspected, however, the patient’s clinical condition did not improve.The physical examination was notable for a systo-diastolic murmur throughout entire precordium. Electrocardiogram showed atrial fibrillation. Laboratory analysis showed leukocytosis  revealed a mass measuring 5.5 × 4.5 cm, occupying the left atrium cavity , as wellThe patient underwent surgical excision of the left atrial mass. During inspection, a large lobulated mass with a smooth surface and malignant aspect was found arising from the infero-posterior mitral annulus and extending to the posterior mitral leaflet. Wide surgical excision and mitral valve replacement were performed. Moreover, due to severe tricuspid regurgitation, tricuspid annuloplasty was performed as well. The tumor was sent for diagnosis confirmation at the pathology department.The histological diagnosis was high-grade undifferentiated pleomorphic sarcoma.Microscopically, the tumor was composed mainly of atypical fusiform cells with a storiform pattern and rare epithelioid cells, areas of myxoid differentiation and nuclear atypia . Less thAccording to the American Joint Committee on Cancer (AJCC) and International Union Against Cancer (UICC) criteria, the tumor has been assigned a histological grade 2 (high grade).Immunohistochemical (IHC) examination showed vimentin and smooth muscle actin positivity.The tumor cells were negative for desmin, melan-A cytokeratin A1/AE3, CD34, HMB45, S100. The Ki-67 proliferation index ranged between 50 and 60%.The surgical margin of the resected tumor could not be evaluated due to the multifragmented nature of the resection specimen.Postoperative transthoracic echocardiography revealed a normal function of biological mitral prosthesis, mild tricuspid regurgitation and no further visualization of the mass.3) and CRP level of 3.41 mg/dL. The patient recovery was uneventful, except for a pericardial effusion which was surgically drained. Postoperative laboratory tests showed normal leucocytes  associated with neoplastic fever and leukocytosis is not extensively reported in the literature.Previous studies have demonstrated that fever related to subsequent cancer occurs in approximately 2.3% of patients, and only a small proportion of these patients were diagnosed with sarcoma .Neoplastic fever, which refers to fever caused by the malignancy itself, is extremely rare in patients with sarcomas. The diagnosis of neoplastic fever can be a dilemma in patients with cancer, and it should be considered after exclusion of other causes of fever, such as infection, leukemia or autoimmune disease ,8.The recognition of cardiac sarcomas can be difficult. Clinical presentation is nonspecific and may include symptoms related to obstruction, such as progressive dyspnea, pulmonary edema, chest pain, or embolic phenomena ,10.Due to delayed diagnosis and therapeutic difficulty, the management of sarcomas represent a challenge, and the long-term outcome is very poor, with median survival of less than one year .The reported patient presented a mass that filled approximately 75% of the left atrium, and most of the symptoms were related to valve obstruction, except for fever.Some studies suggested that neoplastic fever is associated with a more advanced disease and worse survival outcome . In a stThe inflammatory status, such as high CRP levels, is associated with decreased survival rates in patients with many types of malignancies ,14,15,16Surgical excision is considered the gold standard treatment for sarcomas. Complete surgical resection is associated with improved survival. However, achieving negative margins is difficult, due to the delayed diagnosis and extent of myocardial involvementPartial resection of the tumor may have an impact on symptom relief and quality of life; nevertheless, it is associated with poor prognosis, especially in patients with neoplastic fever and inflammatory response ,19. SimiThe utility of chemotherapy and radiation in UPS remains undefined.Given the rarity of these tumors and lack of clinical trials, treatment modalities are extrapolative and chemotherapy regimen is derived from extra-cardiac sarcomas ,21,22.Combined neo-adjuvant chemotherapy and radiation may improve the result of surgical resection and reduce the risk of mass recurrence ,24.Several studies have shown that neo-adjuvant chemotherapy is associated with significant improvement in the rate of negative margins and overall survival ,26.In this case, surgical resection with negative margins was not possible due to the infiltrative pattern of the mass and delayed diagnosis. Chemotherapy and radiation treatment were palliative due to the presence of metastases at the time of diagnosis.Cardiac sarcomas are an extremely rare condition and are associated with poor prognosis. Due to the difficulty in obtaining negative margins, survival after surgical resection is limited. Therefore, neo-adjuvant chemotherapy should be considered to aid surgical treatment.Consequently, early diagnosis is crucial for a proper management and favorable outcome, enabling patients to undergo chemotherapy and achieve complete surgical resection.In summary, we presented an atypical presentation of a rare UPS localization with inflammatory response and recurrent fever, which was associated with a poor prognosis.


A 66-year-old man was treated for a moderately differentiated T3 N1 M0 adenocarcinoma of the rectum in 2015 with preoperative short course radiotherapy, anterior resection and then adjuvant chemotherapy with oxaliplatin and capecitabine. Following ileostomy reversal, he complained of intense, unremitting anorectal pain. After repeated scans, computed tomography (CT) showed findings suggestive of a longstanding anastomotic leak. Subsequent, magnetic resonance imaging (MRI) revealed osteomyelitis of the sacrum, with the development of sacral osteomyelitis in this context unusual. Our case highlights the importance of appropriate radiological imaging and that clinicians should consider osteomyelitis as a differential diagnosis in patients presenting with severe anorectal pain after treatment for rectal cancer. A 66-year-old man with a diagnosis of moderately differentiated T3 N1 M0 adenocarcinoma of the rectum in 2015 underwent short course radiotherapy prior to anterior resection that was followed by adjuvant chemotherapy with oxaliplatin and capecitabine, which were stopped prematurely due to grade 1 peripheral neuropathy. His temporary ileostomy was reversed 1 year later. Following reversal, he was troubled with on-going difficulties of bowel function including increased frequency, urgency with incontinence and tenesmus. Most troublesome of all was progressive rectal pain. He was referred to our Consequences of Colorectal Cancer Clinic, 4 years later.Flexible sigmoidoscopy showed no significant changes other than a previous anterior resection. His blood tests during this time showed normal leukocyte counts and a mildly elevated C-reactive protein that ranged from 16 to 29. Three CT scans of his abdomen and pelvis were arranged before referral to us. The initial CT in July 2017 showed presacral soft tissue thickening with air pockets that were in keeping with typical postsurgical changes. However, unusually, this presacral collection became more prominent in subsequent CT scans. By the time of his third CT in late 2018, a fistula between the rectum and the presacral collection at the anastomotic site could be identified , suggestDue to his on-going symptoms and for better delineation of the anatomic structures, MRI of the sacrum and coccyx with Short-TI Inversion Recovery sequences was requested. This showed hyperintense signalling in the lower sacral vertebrae representing changes consistent with chronic osteomyelitis . The preLong-term significant defaecatory dysfunction following anterior resection is estimated to occur in between 37 and 90% of patients . When anSacral osteomyelitis is an extremely rare complication of treatment for pelvic cancer. When it occurs, it is usually associated with fistulization from the rectum as in our patient or in one other case we have found  or from MRI imaging should be utilized to assess patients with ongoing anorectal pain following treatment for pelvic cancer. In those who have developed sacral osteomyelitis, MRI demonstrates changes in the water content of bone marrow while also providing excellent structural definition . It alsoTreatment of sacral osteomyelitis is often multi-modal and can involve a combination of targeted antibiotic therapy, radiological drainage and/or surgery . It is tIn conclusion, clinicians should have a low index of suspicion of an anastomotic leak and associated sacral osteomyelitis when treated rectal cancer patients present late, often after many years, with anorectal pain. Our case has highlighted the importance of this and how imaging can be utilized to diagnose the condition and plan for treatment.


Congenital short bowel syndrome (CSBS) is a very rare gastrointestinal anomaly of unknown etiology. We report a case of a six-week-old male with CSBS who presented with features of intestinal obstruction and failure to thrive. The abdominal radiograph was suggestive of a central gasless abdomen, and a provisional diagnosis of malrotation of the gut with volvulus was considered. On exploration, the duodenum was hugely dilated with annular pancreas, which was not obstructing the duodenum. The length of the intestine was 20 cm from the duodenojejunal junction to the ileocecal junction. Duodenal web was ruled out. Total parenteral nutrition (TNP) was started postoperatively. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome; however, the challenges are entirely different in developing countries. Congenital short bowel syndrome (CSBS) is a rare gastrointestinal anomaly characterized by a substantial reduction of small bowel length in the intrauterine period as compared to normal individuals limiting enteral digestion and absorption of nutrients. These patients present within a few days after birth with bilious vomiting, diarrhea, and failure to thrive. The prevalence is less than one in 1,000,000 births, and until recently, less than 65 cases have been reported in English literature . This arA six-week-old male child presented with bilious vomiting for two weeks and failure to thrive. He was the second born from a non-consanguineous marriage, late preterm, appropriate for gestational age baby, and born by normal vaginal delivery with a birth weight of 2.4 kg. The antenatal ultrasonography was within normal limit. The baby cried immediately after birth, passed urine and meconium within 24 hours, and started on breastfeeds. He was detected as COVID-19-positive on the first day of life and was discharged with advice for home quarantine. He was feeding inadequately from then onward, however, and was passing urine and stool.Since the third week of life, he had bilious vomiting and was managed conservatively elsewhere, with nasogastric aspiration and intravenous fluids. At referral, the child weighed 1.9 kg , and his length was 55 cm (within the 25th percentile). He appeared clinically well with no dysmorphic features, rashes, jaundice, or edema. On examination, his abdomen was soft, without any distension or organomegaly.Laboratory studies demonstrated hemoglobin of 17.3 g/dL, white blood cell count was 8,860/cmm with normal differential counts, and platelet count was 456,000/mcL. Serum electrolytes, creatinine, and bicarbonate levels were within normal limits. The serum calcium level was low, for which correction was given. An abdominal radiograph showed fundic gas, a central gasless abdomen, and sparsely scattered minimal air-filled bowel loops toward the left lower quadrant of the abdomen  and started on total parental nutrition. The grave prognosis of the condition was explained to the parents and caregivers and the need for long-term total parental nutrition and the complications associated with it. They were also explained the need for genetic testing of the baby and the parents. However, they were not financially ready to bear the cost of the treatment and unfortunately left against medical advice on postoperative day 4.The exact etiology of CSBS is unknown; however, the most accepted theory is the vascular insult during the embryonic period. CSBS is found to be related to an autosomal recessive mutation in coxsackie and adenovirus receptor-like membrane protein (CLMP) or X-linked recessive mutation in filamin A (FLNA) . Only maNegri et al., recently in a case series on CSBS including 61 patients over 50 years, concluded that the most common presentation was abdominal distension (96.2%) followed by failure to thrive 77.3%) and diarrhea (37%). The associated anomalies reported were bowel adhesion, bands, pyloric hypertrophy, persistent ductus arteriosus, central nervous system malformation, mesenterium commune, and the absence of the appendix [% and diaAnnular pancreas is also a rare congenital anomaly characterized by a complete or partial ring of pancreatic tissue encircling the second part of the duodenum . Non-bilThere is no consensus on the definition of small bowel syndrome in relation to the length of the remaining intestine. The prognosis of CSBS highly depends on the length and function of the remaining segment of the small bowel. Less than 40 cm traditionally requires therapy in most centers for survival . RecentlParenteral nutrition has been a huge support in these patients; however, the rate of complications such as sepsis and liver failure is very high. Bowel-lengthening procedures such as serial transverse enteroplasty (STEP) and plication of dilated duodenum have been used in some centers, and small bowel transplantation is considered the last resort due to overall poor survival . The unaCSBS is a rare condition that presents with bile-stained vomitus, failure to thrive, and diarrhea. A contrast study may give a clue; however, the definite diagnosis is always at laparotomy. Annular pancreas presents as a proximal intestinal obstruction. Imaging studies have a limitation to detect the short bowel syndrome in neonates when there is an associated proximal bowel obstruction, which precludes gas to traverse beyond the obstruction. Hence, emergency physicians will have an on-table surprise to detect the CSBS along with annular pancreas. Early and long-term parenteral nutrition and referral to specialist centers with intestinal rehabilitation programs have improved the overall outcome of this rare condition worldwide. However, the challenges are even more in the developing part of the world.


Angioleiomyoma (ALM) is a benign smooth muscle neoplasm that mainly occurs in lower extremities subcutaneous tissue and generally affects middle-aged adults. This tumor histotype may rarely localize intracranially, although only a few cases have been described in the literature. We report a case of intracranial ALM, whose differential diagnosis has been particularly challenging, and firstly provide a comprehensive radiological and intra-operative evaluation of a such rare entity. This represents also the first report of the use of intraoperative confocal microscopy in ALM and the first documented short-term recurrence. At this regard, a scoping literature review has been conducted with the aim of presenting the major clinical and diagnostic features along with the proposed therapeutic strategies. Angioleiomyoma (ALM), also called angiomyoma or vascular leiomyoma, is defined as a benign and indolent soft tissue neoplasm arising from smooth muscle cells. According to the 2016 central nervous system (CNS) tumors classification by World Health Organization (WHO), ALM is classified as a mesenchymal non-meningothelial brain tumor . Epstein18F-FDG PET-CT) scan was acquired, displaying an increased tracer metabolism in the lesion without evidencing any extracranial pathologic uptake. Moreover, a brain computed tomography (CT) scan showed granular calcifications inside the lesion. Taking into consideration the neuroradiological findings, surgery was thought to be appropriate to both reduce the mass effect and make a definitive histological diagnosis. In the operating room, the patient was positioned supine and, under microscopic guidance, we decided to approach the lesion through a transtemporal tranventricular route in order to gain a wider control on both the lesion and its vascularization. The lesion was encountered in the left tentorial hiatus and appeared as an extraparenchymal, red and capsular mass with an arterialized surface . During resection, the microscope could be switched alternatively from fluorescent to white-light illumination. Intra-operative fluorescein-assisted miniatured confocal laser endomicroscopy  has been used.The standardized surgical protocol of fluorescein-guided technique is based on i.v. SF injection at standard dose of 5mg/kg, by a central or peripheral venous line, immediately upon completion of the induction of general anesthesia . The surintracranial AND angioleiomyoma) AND ). The results were then limited to human subjects. After duplicate removal, title and abstracts were firstly screened and, for the papers deemed appropriate, full text was obtained and reviewed for appropriateness and extraction of data. Article references list was also examined to identify any other relevant study. Only studies dealing with the presence of intracranial ALM were included. Data from the included studies were extracted, organized, and analyzed. The qualitative assessment of the level of evidence of the papers extracted has been evaluated according to Oxford CEBM  statement guidelines, limited to the English language. SCOPUS, PubMed and Cochrane databases were queried using individual keywords and MeSH terms. A purposely defined search string was performed for PubMed, Scopus and Cochrane search: (“Leiomyoma”[Mesh]) AND “Central Nervous System”[Mesh], and for SCOPUS search: TITLE-ABS KEY  for the publication of any potentially identifiable images or data included in this article.EC, FA and PF performed the clinical assessment. ER, EC and GB critically reviewed the literature and drafted the manuscript. All authors were responsible for important intellectual content. All authors contributed to the article and approved the submitted version.