The rare syndrome of Blue Rubber Bleb Nevus is known to be causing skin vascular lesions in the form of bluish papules, called blue nevi and the movable rubber-like consistency soft tissue masses. The syndrome frequently involves digestive system besides other visceral organs such as liver, lungs, thyroid gland, spleen and nervous system. We present a case of a 36-year-old female with Blue Rubber Bleb Nevus that involved skin and musculoskeletal system. The rare BRBNS (Blue Rubber Bleb Nevus syndrome) was first reported by Gascoyen in 1860 .Diagnosis is mainly clinically supported by different modalities. Depending on the patient’s presentation and suspicion, the CBC is used to look for anaemia and faecal occult blood could be used to look for bleeding digestive system lesions. Dark urine will necessitate a urine test as bladder lesions could be the cause. Other radiological diagnostics are obtained depending on the specific manifestation, and for instance, musculoskeletal complaints may indicate the need for a simple X-ray or an MRI, while gastrointestinal incidents may require a CT or an endoscopy .Histopathological findings include a vascular network of dilated tortuous blood-filled vessels, lined up with a single layer of endothelial cells, surrounded by a delicate connective tissue .There is no curative treatment. Systemic steroids are used to control intestinal growth. Interferon-alpha is used to treat coagulopathies. Surgical interventions  or electrocauterization and cryosurgery are only used to excise symptomatic lesions and for cosmetic considerations .A 36-year-old female nurse from a remote rural area in Syria without significant medical or family history presented to the Department of Dermatology of Damascus University with skin complaints of ‘deep blue pimples’ and rubbery consistency masses under her skin in different parts of the body.As described by the patient, the lesions started appearing right after birth as pigmented papules disseminated on the back and extremities. The papules grew gradually to form nodules and small tumours that bleed easily when exposed to trauma. Nodules also erupted on mucous membranes and the ones on the tongue and tonsils were surgically removed due to dysphagia and discomfort they caused the patient. The lesions kept appearing until the patient reached 20 years of age. In addition to the papules, the patient noticed the appearance of soft masses of different sizes under the skin, some of which was tender.Physical examination of the patient revealed a pigmented brown-blue patch on the back , severalThe patient did not suffer from any anaemia that was due to the CBC laboratory examination.Apart from the scars of previous surgical excisions, the rest of the physical examination was normal including the lymph nodes, hair and nails.Additionally, the patient reported musculoskeletal complaints of bone and joint pain for which an MRI was ordered only to disclose multiple tumours occupying the muscles of the forearm .At the age of 6, a biopsy was performed on one of the excised lesions by a dermatologist . HistoloThe patient was offered cryosurgery or electrocauterization to remove bothersome lesions as she wishes.To differentiate this diagnosis from similar diseases and syndromes, we compared the specifications of our case with six other entities: Diffuse Neonatal Hemangiomatosis, Familial glomangiomatosis, Maffucci syndrome, Kaposi sarcoma and Klippel–Trenaunay–Weber syndrome .Absent at birth and developing during the first weeks of life, the Diffuse Neonatal Hemangiomatosis is characterized by multifocal tumours up to 20 mm in size that grows rapidly and regresses spontaneously without sequela when confined only to the skin. The tumours could form in the liver, lungs or nervous system, causing symptoms of obstructive jaundice, heart and lung failure, or intracerebral haemorrhage. The mortality rate is high in untreated individuals. Only large symptomatic lesions should be excised. Histopathology reveals endothelial cell hyperplasia in a lobular form, mast cells and a prominent basement membrane. Upon regression, lipofibromatous tissue is seen with mast cells . Our patWhile being not congenital, Familial Glomangiomatosis manifests with hard (not rubbery) dark blue papules and nodules clustered in groups with less mucosal involvement. The lesions usually start in young children and are less likely to cause symptoms. Some patients report pain episodes elicited by menstruation and pregnancy. The biopsy findings could catch the diagnosis as they show venous malformation of glomus cells surrounding irregular veins .The genetic syndrome of Maffuci generally shows a manifestation at the age of 4–5 years old with cartilage tumours and vascular anomalies including spindle cell endothelial tumours. The nodules are tender and asymmetric, localized more on the distal extremities. The hemangiomas could be superficial or deep. The probability for chondrosarcoma is possible around the age of 40 . Our patIn contrast to our patient who had several lesions, Klippel–Trenaunay–Weber presents with one large vascular nevus, mostly on the thigh, knee or leg, with overlying lymphatic bullae that could bleed after puberty. The syndrome also has bone and connective tissue hypertrophy with varicose veins that may or may not accompany deep venous anomalies. Doppler ultrasound is used to diagnose this syndrome along with MRI .The nodular type of Kaposi Sarcoma can manifest in older males as lesions mainly on lower extremities and rarely on the face or torso. The lesions can be symmetric with accompanying lymphedema. Hyperkeratotic overlying skin can be seen with a chance of malignancy in the advanced lesions. The biopsy shows bland spindle cells forming slit-like spaces occupied by red blood cells .Our case represents one of the rarest vascular syndromes where only skin, mucous membranes and musculoskeletal systems were involved. The importance of the biopsy is not to be forgotten in combination with the clinical assessment being put in the picture of the whole presentation, such as the age and the progression. Patient education and life-long follow-up are essential to detect probable more dangerous manifestations which might be life-threatening such as gastrointestinal and neurological manifestations.


Colorectal lymphomas are very rare and are usually found in male patients between the fifth and seventh decade of life. Our patient was a 30-year-old male who presented with the complaint of epigastric pain and abdominal mass for the past three months. Physical examination was remarkable for soft solid abdominal mass extending from the right side of the abdomen toward the left side. Computed tomography showed a large lobulated mass in the right lower quadrant involving multiple loops of bowel. The mass measured 15.1 x 12.5 cm in transverse dimension and 16.2 cm in craniocaudal dimension. Colonoscopy showed a villous and fungating mass occupying the whole cecum and extending into the ascending colon. Pathology from the colonoscopy mass revealed diffuse large B-cell lymphoma, which was CD20 (cluster of differentiation 20) positive. He was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapy regimen and is following at the oncology outpatient department. A combination of surgical resection and chemotherapy is used in treating patients with colorectal lymphoma. Lymphomas are categorized into two groups: non-Hodgkin's lymphoma and Hodgkin's lymphoma. Non-Hodgkin's lymphoma is the seventh most common type of cancer in the United States, and as of August 30, 2022, accounted for 4.2% of all new cancer cases diagnosed in the US in 2022 . The gasOur patient was a 30-year-old Hispanic male who presented to the emergency department with a complaint of epigastric pain and abdominal mass for the past three months. His epigastric pain was sharp in character, non-radiating, mild in intensity, was not associated with eating or fasting, and had progressively increased with time. His abdominal mass had progressively increased with time and he was unable to lie in a prone position. He also reported nausea and early satiety. He stated that he has lost 30 pounds over three months. He stated that the main reason he was concerned now was because of the inability to lie on his bed in the prone position. He denied dysphagia, vomiting, hematemesis, diarrhea, constipation, change in stool consistency and caliber, hematochezia, and melena.He denied any significant past medical and surgical history. His family history was significant for stomach cancer in two of his maternal aunt. He denied smoking and using any recreational drugs.In the emergency department, he was found to have a temperature of 98.4°F, heart rate of 84 beats per minute, respiratory rate of 16 breaths per minute, blood pressure of 121/66 mmHg, and was saturating 100% on room air. Abdominal examination was significant for a soft solid mass palpated, which was extending from the right side of the abdomen toward the left side. There was no abdominal tenderness or rigidity. He was found to have normal bowel sounds. He had no cervical and inguinal lymphadenopathy. He had bilateral vesicular breathing on lung auscultation and had normal heart sounds. Neurological examination was unremarkable. He had no cervical, axillary, or inguinal lymphadenopathy. His initial laboratory findings have been presented in Table The patient underwent computed tomography (CT) of the abdomen and pelvis with intravenous contrast, which showed a large lobulated mass in the right lower quadrant involving multiple loops of bowel. The mass measured 15.1 x 12.5 cm in transverse dimension and 16.2 cm in craniocaudal dimension. This has been shown in Figure He was given pain medication and antiemetics in the emergency department. He was admitted to the floor for further workup. Colonoscopy showed a villous and fungating mass occupying the whole cecum and extending into the ascending colon. The mass was biopsied and sent for analysis. The appendix and ileocecal valve could not be identified. This has been shown in Figure He underwent esophagogastroduodenoscopy (EGD) for his epigastric pain, which was significant for erythema of the greater curvature of the gastric body. His cancer antigen 19-9 (CA 19-9) and carcinoembryonic antigen (CEA) were unremarkable. His peripheral smear was unremarkable. His sputum acid-fast bacilli (AFB) culture was negative. He tested negative for human immunodeficiency virus (HIV). He underwent a CT of the chest and head, which was unremarkable.Pathology from the colonoscopy mass revealed diffuse large B-cell lymphoma, which was CD20 (cluster of differentiation 20) positive. Ki-67 showed a high proliferative index (-80%) and Epstein-Barr virus-encoded RNA-1 in situ hybridization (EBER(ISH)) stain was negative. This has been presented in Figures Helicobacter pylori were seen. He was diagnosed with diffuse large B-cell cecal lymphoma. He was started on rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP) chemotherapeutic regimen. He underwent two cycles of chemotherapy and had a significant decrease in abdominal mass size on physical examination. The patient is currently followed in the oncology clinic for repeat CT scans, positron emission tomography (PET), and chemotherapy.Biopsy from the greater curvature of the gastric body showed mild gastritis with focal activity and no H. pylori infection, autoimmune diseases, immunodeficiency, immunosuppression, celiac disease, and inflammatory bowel disease are a few conditions that increase the risk of getting gastrointestinal lymphoma. H. pylori infection has been associated with the development of mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach [H. pylori infection in the development of colon lymphoma has not been established [H. pylori infection and he tested negative for HIV. stomach . Currentablished . Our patDifferential diagnoses of large cecal masses in a young patient include adenocarcinoma, lymphoma, gastrointestinal stromal tumors, and tuberculosis. Patients with large cecal masses usually present with the sign of obstruction but our patient was able to tolerate food and had no clinical sign of obstruction despite the mass being 15.1 x 12.5 x 16.2 cm in size.CT of the abdomen and double contrast barium enema are the two major radiological modalities in diagnosing colon lymphoma. Radiological findings of colorectal lymphoma on CT scan include polypoid mass, circumferential infiltrative lesion, large cavitary lesion, or ulcerative lesion ,15. PolyA combination of surgical resection and chemotherapy is used in treating patients with colorectal lymphoma. The CHOP  chemotherapeutic regimen is the treatment of choice in patients with colorectal lymphoma. Our patient had a good response to chemotherapy so surgical intervention was not considered. The addition of rituximab to the CHOP chemotherapeutic regimen has been shown to result in a higher response rate and also improves the progression-free, event-free, disease-free, and overall survival rates ,18. The Based on our literature review of published cases of cecal lymphoma in patients older than 18 years of age but less than 45 years of age, in most of the case reports, the HIV status was unknown or positive. Our patient did not have vomiting and was HIV-negative.Primary colonic lymphoma is a very rare malignancy and is most commonly seen in elderly male patients. Our case highlights the importance of having awareness of colonic lymphoma in young patients who do not have risk factors predisposing to colonic lymphoma. Early diagnosis and prompt treatment are fundamental in improving the overall prognosis for the patient.


Schwannomas are tumors of neoplastic Schwann cells generally found in peripheral nerves in the head, neck, and extremities. They do not demonstrate hormonal abnormalities, and initial symptoms are typically secondary to adjacent organ compression. These tumors are rarely found in the retroperitoneum. We present a rare finding of an adrenal schwannoma in a 75-year-old female who presented to the emergency department with right flank pain. Imaging incidentally demonstrated a 4.8 cm left adrenal mass. Ultimately, she underwent a left robotic adrenalectomy, and immunohistochemical testing confirmed the presence of an adrenal schwannoma. It is imperative to undergo adrenalectomy and immunohistochemical testing to confirm the diagnosis and rule out malignancy. An adrenal incidentaloma is an unexpected tumor on the adrenal gland discovered by imaging during an investigation for conditions not thought to be adrenal pathology. Adrenal incidentalomas are at increased risk of indicating malignancies when they are larger than 4 cm in diameter, appear bilaterally, have decreased washout on magnetic resonance imaging (MRI) or computed tomography (CT), and demonstrate irregular borders on imaging. While there are numerous differentials associated with some of these characteristics, adrenal schwannomas can be considered. Adrenal schwannomas are extremely rare and difficult to diagnose. Schwannomas are more commonly described in other parts of the body including the head, neck, and extremities . It is uA 75-year-old female with a past medical history of hypertension, hyperlipidemia, and gout presented to an outside facility with a chief complaint of right flank pain that started two weeks ago. She described the pain to be a moderate aching pain radiating to the right groin. CT imaging was performed and showed a 9 mm stone in the right ureter and an incidental left adrenal mass measuring 4.8 cm with no evidence of washout. The patient subsequently underwent ureteral stent placement for ureterolithiasis and was then referred to our facility for further work-up of the left adrenal mass. Upon evaluation at our facility, she admitted to having residual intermittent gross hematuria, dysuria, and right flank pain since her stent placement. A review of systems was negative. She was afebrile, and her vital signs were unremarkable. Her physical exam findings were benign including no purple striae, buffalo hump, or other cushingoid features. The pain had gradually decreased and disappeared after the stent was removed.MRI of the abdomen with and without contrast was ordered and was significant for a 2.9 cm x 2.5 cm x 4.8 cm left adrenal mass, as shown in Figure There was no evidence of washout on contrast-enhanced phases. Tests for serum electrolytes, serum aldosterone to renin ratio, a 24-hour urine collection for catecholamines and metanephrines, and a low-dose dexamethasone test were performed and unremarkable.Due to the size of the mass being over 4cm in diameter and the lack of contrast washout, there was a concern for a malignant adrenal mass or metastatic disease. She was hospitalized for a robotic left adrenalectomy, which was performed without complications. The histopathology of the specimen identified a spindle cell tumor which was close to histologically unremarkable adrenal gland tissue. Spindle cells showed no necrosis and no increased mitotic activity. Segments of nerves and ganglion cells were noted adjacent to the tumor mass, and some of the tumor cells showed nuclear pleomorphism. Immunohistochemical staining performed on the adrenal mass showed S100 protein positivity and negativity for smooth muscle actin antibody, desmin, anticytokeratin monoclonal antibodies (AE1 and AE3), synaptophysin, chromogranin, and Melan A. The patient was discharged from the hospital two days later and she continued with urology for outpatient follow-up.Adrenal schwannomas are an extremely rare form of an adrenal tumor, and very few cases have been documented in the medical literature. This case highlights the significance of considering this diagnosis when assessing an adrenal mass as well as adding to the medical literature of such cases.Adrenal incidentalomas larger than 4 cm with no evidence of MRI washout, as seen with our patient, are at increased risk of malignancy ,4. This Our patient presented with a 4.8 cm left adrenal incidentaloma. MRI with and without contrast demonstrated a well-circumcised mass without washout on CT scans or MRI. Labs for serum electrolytes, serum aldosterone to renin ratio, a 24-hour urine collection for catecholamines and metanephrines, and a low-dose dexamethasone test were within the normal range. Immunohistochemical staining was positive for S100 and negative for smooth muscle actin, desmin, AE1/3, synaptophysin, chromogranin, and Melan A, indicating a potential diagnosis for a schwannoma.A schwannoma is an encapsulated, benign, slow-growing neoplasm that generally occurs between the third and sixth decades of life. The main component of schwannomas arises from neural crest cells. Therefore, with the exception of cranial nerves I and II (which lack Schwann cells), schwannomas can occur in every other organ or nerve trunk . These nThe general patient population with a schwannoma tends to be asymptomatic. One case series of 33 cases demonstrated the median age at the time of diagnosis to be 49 years and with a female predominance. This case series also highlighted that only 13 patients were symptomatic. The other schwannomas were found incidentally . The retDue to their nonfunctional status, most patients with adrenal schwannomas do not exhibit biochemical or clinical evidence of hormonal abnormalities. Nevertheless, concern for malignancy due to size and lack of imaging washout required labs to rule out the presence of other adrenal masses such as pheochromocytoma, myelolipomas, neuroblastomas, ganglioneuroma, cysts, and metastatic disease . Labs foOn CT scans, schwannomas generally appear as a round or oval mass, which is well-demarcated. There have been cases where imaging has demonstrated cystic degeneration and/or calcifications . On MRI,A schwannoma cannot be diagnosed until it is surgically removed. The general approach is laparoscopic, similar to the one we utilized in this case. The removal is also therapeutic as schwannomas are not responsive to either radiotherapy or chemotherapy . Only suIn conclusion, adrenal schwannomas are rare tumors that are mostly found incidentally. They are difficult to diagnose and although the workup can confirm that the mass is a non-secreting adrenal mass, it is usually non-diagnostic. As confirmed by the literature and by our experience, prompt surgical excision followed by histological and immunohistochemical evaluation of the mass is crucial for accurate diagnosis and to predict the outcome. Hence, adrenal schwannomas should always be included in the differential diagnosis of solid nonfunctioning adrenal tumors.


Myositis ossificans (MO) and myositis ossificans-like lesions have been rarely described within the veterinary literature, and are even less common in the maxillofacial region. When MO affects the muscles of mastication, it can result in complete or partial inability to open the mouth. As with other conditions resulting in decreased or restricted mandibular range of motion, severe and potentially fatal sequelae such as difficulty with prehension, swallowing, and air exchange are possible. Diagnostic imaging is essential in achieving an accurate diagnosis and in formulating an appropriate treatment plan. In this “method” manuscript, we provide a detailed description of our approach to diagnosis and surgical management of MO-like lesions of the pterygoid muscles and describe our experience with two young French bulldogs. Myositis ossificans (MO) is a term used to describe heterotopic bone formation within a muscle. In people, the disease is rare, and even more so in the maxillofacial region. The human medical literature has classically described MO as being either myositis ossificans progressiva  or myositis ossificans traumatica . The two are believed to have distinct etiologies and outcomes with MOP having a genetic underpinning and invariably progressing, whereas the mechanism of MOT is unknown but often suspected to occur secondary to trauma. In both processes, attempted excision of the heterotopic bone is often followed by recurrence –4.Myositis ossificans is very rarely reported in dogs, and mostly in the limb muscles , 6. One As with other conditions resulting in decreased mandibular range of motion, severe and potentially fatal sequelae are possible. Difficulty with prehension, swallowing, and air exchange are known life-threatening complications, in addition to decreased quality of life secondary to an inability to groom and play. For this reason, surgical intervention is imperative if euthanasia is to be avoided. However, based on the human medical literature, attempted excision of these osseous proliferations typically recur. In addition, excision of bridging osseous lesions from the base of the skull to the mandible carries the risk of catastrophic hemorrhage. Therefore, an alternative surgical technique which provides a functional outcome without the risk of recurrence or significant hemorrhage is needed.In this report, the diagnostic imaging, clinical, and histopathologic features of two cases with MO-like lesions of the pterygoid muscles are described. In addition, a surgical approach of bilateral segmental mandibulectomies resulting in functional long-term outcome will be detailed and discussed.Two dogs presented for evaluation of a progressive inability to open the mouth at a young age and that did not exhibit severe atrophy, swelling, or pain associated with the muscles of mastication, suggestive of masticatory myositis, received a cone beam CT  or CT  scan. Each case was evaluated on medical grade monitors. Findings of the CT/CBCT include bilateral smoothly margined, dense osseous proliferation spanning the region of the medial pole of the condylar process and tympanic bulla to the medial aspect of the body of the mandible .Depending on the severity of the dog's inability to open the mouth, modified anesthetic approaches are taken. In dogs with minimal or no opening of the mouth, a temporary tracheostomy is anticipated. Alternatively, in dogs with a small amount of remaining range of motion, flexible or rigid endoscopy is utilized to assist with orotracheal intubation. In all cases, preparations are made for a temporary tracheostomy if the endoscopy-assisted intubation is unsuccessful. Patients are pre-operatively blood-typed and cross-matched in the event of significant hemorrhage. Inferior alveolar nerve blocks may not be possible if there is obliteration of the mandibular foramina by proliferative osseous tissue. Post-operatively, patients are monitored in the intensive care unit for at least 12 h.Dogs are clipped along the entirety of the ventral and lateral aspect of both mandibles and aseptically prepared for surgery. The dogs are placed in dorsal recumbency with the neck gently extended. A skin incision is made using a #15 blade along the ventromedial border of each mandible, extending from the caudal aspect of the mandibular symphysis to just rostral to the angular process . Then th®, Mectron, Carasco, Genova, Italy), shallow outlines of the bone cuts are made. The needles are then withdrawn and removed from the surgical site. The caudal osteotomies are made sequentially in both mandibles in case there is a need to open the mouth emergently. Osteotomies are then performed rostrally as well. Once both osteotomies are complete for each segment  and the inferior alveolar neurovascular bundles are ligated rostrally and caudally, the vessels and nerves are transected. Bone holding forceps are then used to place traction on the segments so that a #15 scalpel blade can be used to remove any remaining soft tissue attachments, including the oral mucosa and gingival attachment surrounding each mandibular first and/or second molar teeth. The segments are then removed through the extraoral incision  scan was elected. Given concerns for aspiration pneumonia and likely brachycephalic obstructive airway syndrome (BOAS), a sedated CBCT was ruled out. The dog was premedicated with maropitant (1 mg/kg SQ) the evening prior to anesthesia. Prior to anesthesia induction, it was sedated with butorphanol IM (0.3 mg/kg) and dexmedetomidime (4.85 mcg/kg IM). After an IV catheter was placed, the patient was induced with ketamine (4 mg/kg IV) and midazolam (0.2 mg/kg IV). Using a flexible endoscope for guidance, the dog was orotracheally intubated, although preparations for temporary tracheostomy were made prior to induction. Complete blood count and serum biochemistry did not reveal significant abnormalities.Anesthetized interincisal distance was noted to be 10 mm, consistent with the awake exam. CBCT revealed bilateral bridging osseous proliferation spanning from the region surrounding the tympanic bulla to the medial aspect of the mandible in the region of the mandibular foramen . The ossA diagnosis of bilateral pseudoankylosis was made, suspected to be due to myositis ossificans-like lesions of the pterygoid muscles. A replica of the skull was 3D printed for surgical planning. After conversations with the owner regarding risks and benefits of various treatment options including excision of the osseous proliferation, medical management, and salvage bilateral segmental mandibulectomy, the latter option was elected. The dog was anesthetized using the protocol reported earlier with the addition of an intraoperative continuous rate infusion of sufentanil (0.01 mcg/kg/min). No locoregional anesthesia was performed. Ampicillin was administered once at the start of the procedure (20 mg/kg IV). Bilateral segmental mandibulectomy was performed as described above using an extraoral approach followed by intraoral closure and placement of elastic training . The remThe elastic chains were maintained for ~4–5 weeks after surgery. The client reported moderate difficulty in changing and maintaining the chains but found that it was possible to replace them when the dog was sleeping. The dog's ability to eat improved rapidly, and the client reported less stertor, more energy, and a more playful demeanor after recovering from surgery.2 months after the initial procedure, the dog returned for a periodontal treatment. An oral exam revealed mild dehiscence in both sites, but granulation tissue filled the mandibulectomy defects, with no bone exposure. The right mandibular fourth premolar tooth had significant gingival recession along the distal aspect. Buccoversion of all right mandibular dentition was still present. The buttons were removed and a periodontal treatment was performed. The right mandibular fourth premolar tooth was extracted, the granulation tissue was debrided, and the oral mucosa over the mandibulectomy area was closed routinely. The dog recovered well and continued to improve at home.3 years after the initial surgery , the dogA 7-month-old, male intact, French bulldog weighing 8.2 kg was presented because of reduced ability to open the mouth. The dog had been adopted 5 months earlier and the new owner quickly noticed difficulties opening the mouth. A latero-lateral thoracic radiographic examination obtained a week before presentation showed normal cardiac and pulmonary structures. The dog was anesthetized to perform an endoscopic examination to stage his BOAS, which showed mild elongation of the soft palate, mild evertion of the laryngeal saccules, mild laryngeal collapse, and mild reflux esophagitis of the proximal esophagus. Reduced interincisal distance was identified during the procedure, and the dog was referred. At the time of presentation, the dog was reported to have a good appetite, to be able to eat and drink, but with frequent episodes of retching after drinking as well as seldom regurgitation and vomiting. The dog was also reported to have dyspnea, stridor, inspiratory efforts, exercise intolerance, and was reportedly unable to play with toys using his mouth but was bright, alert, and responsive with a body condition score of 5/9. A left sided grade I/VI heart murmur, mild stenotic nostrils and moderate stertor and stridor were present. An echographic examination diagnosed a mild mitral insufficiency that appeared hemodynamically insignificant.A slight atrophy of the left muscles of mastication was present. Retropulsion of the eye globes was symmetrical and considered normal. No pain could be elicited on palpation of maxillofacial structures or on attempted opening of the mouth. Despite the brachycephalic conformation of the skull, a mild (8 mm) class 2 malocclusion was present, and the mandibles also appeared slightly rotated to the left side. Maximum interincisal distance appeared to be 10 mm. A permanent dentition with absence of left mandibular second and third molar teeth and right mandibular third molar tooth, and crowding and rotation of multiple teeth were evident. During examination, the dog regurgitated an abundant amount of clear fluid. Based on physical exam and history, TMJ ankylosis or pseudoankylosis were suspected.A complete blood count and serum biochemistry were within normal limits, and 3 weeks later the dog was anesthetized to perform a CT scan with both pre- and post-contrast images available for evaluation. The ventral neck was clipped and prepared for a possible tracheostomy. After an IV catheter was placed, anesthesia was slowly induced with fentanyl (6 mcg/kg), propofol (3 mg/kg), and midazolam (0.2 mg/kg) IV, without premedication. Although a rigid endoscope was available to facilitate the procedure, endotracheal intubation was first attempted and performed blindly with a #3 cuffed endotracheal tube, which was considered slightly undersized for the patient but still effective in obliterating the tracheal lumen. Anesthesia was maintained with oxygen and isofluorane.Interincisal distance measured under anesthesia was confirmed to be 10 mm. The CT examination showed symmetrical, multilobular, tubular mineralizations of the right and left lateral pterygoid muscles, originating dorsally between the lateral wall of the tympanic bullae and the mandibular condylar process, and extending to the medial surface of the mandibular ramuses, just caudo-ventrally to the mandibular foramina , which aA bilateral segmental mandibulectomy was performed a week later. The dog was anesthetized and intubated using the protocol reported earlier with the addition of an intraoperative administration of maropitant (1 mg/kg), and a continuous rate infusion of fentanyl (10 mcg/kg/h) and propofol (0.2 mg/kg/min). A lidocaine bolus (1 mg/kg IV) was administerd 10 min after induction. No locoregional anesthesia was performed. Cefazolin was administered once at the start of the procedure (20 mg/kg IV).Bilateral segmental mandibulectomy was performed as described above, making the ostectomy cuts through the distal root of right and left mandibular first molar teeth, and distal to the mandibular second molar teeth. This resulted in a removal of segments which were 10 mm on each side. Because of slight sublingual swelling, dexamethasone was administered intraoperatively (0.25 mg/kg IV). Once the mouth could be opened, the endotracheal tube was removed and replaced with a larger (#3.5) cuffed endotracheal tube. The right and left first molar teeth were extracted and a post-operative intraoral radiographic examination of the surgical area was performed. However, because of anatomical conformation and muscle mineralization, placement of the radiographic plates lateral to the caudal mandibular segments was limited. The surgical sites were then sutured routinely. A postoperative CT scan was performed. Postoperative interincisal distance was 77 mm, and the elastic chains were positioned to allow a 30 mm mouth opening .The dog recovered uneventfully, and was monitored in the intensive care unit for the following 24 h. It was discharged the following day, with a prescription for cephalexin (120 mg PO q12 h for 5 days), meloxicam (0.8 mg PO q24 h for 7 days), tramadol (25 mg PO q8 h for 7 days), and 0.1% chlorhexidine gel orally 2–3 times daily for 4 weeks. A few extra elastic chains were provided to the owners, with the instruction to change them about once weekly or sooner if necessary. It was recommended to offer small meatballs or dry food slightly moistened with water, and to allow eating and drinking from an elevated bowl and/or from the faucet. Furthermore, the owner was instructed to restrict the dog's physical activity as much as possible, but to favor chewing activity and perform manual physiotherapy gently opening and closing the dog's mouth several times a day, to decrease the chances for indirect bone healing in the ostectomy site.At the 3-weeks  and 9-weThe elastic chains were kept in place for about 7 months. Successive follow-ups were performed by telephone, until the 8-months re-check. During this time the diet was modified to dry kibble, without any significant problems. The dog was reported to be active and able to play with corks and rubber balls using his mouth. Open mouth and mild central lingual protrusion were still present. Maximum interincisal distance was measured to be 44 mm in an awake state, and complete manual closure of the mouth was still impossible. It was then recommended a further re-check in about 10 months, to perform a CT re-examination to evaluate the healing of the surgical sites and any progression of the muscular lesions. At the time of publication, 15 months after surgery, the dog was reported to be thriving at home with no ongoing concerns.To the authors' knowledge, this is the first report on the surgical management of bilateral pterygoid myositis ossificans-like lesions in dogs. This report demonstrates several clinically relevant aspects in cases of MO-like lesions with subsequent treatment with bilateral segmental mandibulectomy. First, diagnostic imaging features of the MO-like lesions are largely consistent with reports of myositis ossificans in people. Second, salvage treatment by means of bilateral segmental mandibulectomy resulted in an acceptable long-term outcome as demonstrated in both case examples. Third, both dogs were young French bulldogs with unknown early history, indicating that both genetic and traumatic etiologies may be considered. Fourth, diagnostic imaging by means of CT or CBCT was essential for diagnosis, surgical planning, and follow-up. 3D printing is also a very useful addition in surgical planning. Finally, special considerations for perioperative and post-operative anesthetic monitoring and care are required for these patients.In the cases presented here, the combination of clinical signs and diagnostic imaging, were consistent with reports of MO in humans. Histopathology confirmed that the biopsy obtained from the pterygoid muscle location in case 2 is consistent with bone tissue composed of woven (immature) and lamellar (mature) bone. The latter indicates a metaplastic process of bone formation, also known as heterotopic ossification. As has been found in humans, the predominant clinical sign in our patients was inability to open the mouth. Neither of the patients had swelling, overt pain, or were febrile as may be expected in other diseases processes that lead to inability to open the mouth. In people, swelling and pain are only found occasionally , and theFrom a surgical standpoint, excision of the osseous proliferation would have been ideal because it would have, theoretically, allowed for a completely normal occlusion and return to function in the future. However, human data suggests that recurrence is likely, possibly because the surgery itself is a traumatic event and may incite further bone formation . An alteIn humans, MO progressiva is known to have a genetic component, whereas MO traumatica/circumscripta is thought to have a traumatic basis. Interestingly, both of our patients were young French bulldogs, which raises the possibility of a genetic predisposition, although this may be a coincidence as the patient in a previous report  was an AIn both cases, advanced diagnostic imaging was essential for obtaining a presumptive diagnosis and formulating a treatment plan. As has been discussed elsewhere , there aFinally, the perianesthetic and perioperative periods required special consideration for both of our patients. The primary challenge in both cases was securing the airway, given that both had very limited interincisal distance. One required endoscope-guided orotracheal intubation, but in both cases emergency tracheotomy was anticipated. Once the surgery was completed, the mouth could be opened but given the brachycephalic conformation, the possibility of significant swelling leading to airway obstruction was still present. Therefore, careful monitoring in the ICU with or without oxygen supplementation for at least 12 h is recommended. In addition, intraoperative hemorrhage was a possibility. In general, when the inferior alveolar artery is inadvertently lacerated or transected prior to completion of the ostectomies, placing digital pressure over the mandibular foramen while ligating the artery typically helps control hemorrhage. However, in case 1, the mandibular foramen was occluded by bone, making digital compression impossible if needed. Therefore, it is advisable to ensure that appropriate blood products are easily available prior to undertaking this surgery. Additionally, the use of piezosurgical equipment, which is considered safer when working in proximity to important neurovascular structures , is evenMyositis ossificans-like lesions of the muscles of mastication in dogs are rare but debilitating and potentially life-threatening disease processes. Given that these lesions have the same general and vague presenting complaint of ‘inability to open the mouth' as other disease processes, it is imperative that diagnostic imaging is obtained to achieve a presumptive diagnosis. Given the likelihood of recurrence following attempted surgical excision of MO-like lesions, an alternative surgical procedure, such as the one presented here, is needed. The complexity of these cases requires thorough planning in the perioperative period as it pertains to anesthesia, surgery, and post-operative care. However, this report demonstrates that salvage bilateral segmental mandibulectomy for patients with MO-like lesions of the pterygoid muscles may result in acceptable clinical outcome for patients with an otherwise life-threatening disease process.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.Ethical review and approval was not required for the animal study because the study is retrospective in nature and included clinical cases, hence, it is exempt from IACUC requirements. The standard written informed consent was required for all procedures performed at the William R. Pritchard Veterinary Medical Teaching Hospital of the University of California, Davis was obtained from the owners. The standard written informed consent required for all procedures performed at both facilities.MD: surgical concept and design, manuscript writing, and provision of study cases. MG: manuscript writing, provision of study materials and cases, and review of the manuscript for important intellectual input. GL: manuscript writing and review of the manuscript for important intellectual input. NV: manuscript writing, interpretation of pathology and electron microscopy, and review of the manuscript for important intellectual input. BA: study concept, surgical concept and design, manuscript writing, provision of study materials and cases, and review of the manuscript for important intellectual input. All authors contributed to the article and approved the final version.The remaining authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.


Thrombosis of the celiac artery trunk is a rare cause of acute abdominal pain which is associated with high mortality and morbidity if not diagnosed and treated early. We present an unusual case of celiac trunk artery thrombosis associated with aortic thrombosis and partial splenic infarction in a 52-year-old female who presented with acute abdominal pain. Hematological investigations failed to predict any predisposing factors. This case demonstrates the importance of considering such clinical presentation in the differential diagnosis of acute abdomen Thrombosis of the celiac trunk is a rare cause of acute abdominal pain. Successful treatment depends on early diagnoses and early establishment of the blood flow by surgical or endovascular intervention. We present a 52-year-old lady who was admitted to our emergency department with acute abdominal pain and signs of peritonitis and was diagnosed with a CT scan to have non-occlusive celiac trunk thrombosis associated with aortic thrombosis splenic infarction.A 52-year-old previously healthy Saudi woman with no history of medical disease or surgical operations presented at our hospital with a sudden onset of upper abdominal pain for four days. She described the pain as a dull ache localized initially to the epigastrium, but it became severe and diffused all over the abdomen on the admission day. The pain was aggravated by food and fluid intake and relieved by hunger and was associated with frequent vomiting of bile-stained vomitus. The bowel habits were regular, and there was no blood in the stool. She was not on medication other than acetaminophen for relief of the current episode of abdominal pain. She was not on birth control pills, hormone replacement therapy, tamoxifen, or other drugs that may increase the risk of thrombosis. On clinical examination, she looked ill, pale, in pain, and not febrile Pulse was 106/minute and BP104/56. Abdominal examination revealed mild distension with generalized rebound tenderness, especially on the left upper quadrant of the abdomen. Bowel sounds were normal. The laboratory tests showed hemoglobin of 11.3 g% white blood cell 8,300/, platelet 519,000/mm3, the international normalized ratio of 1.45, and activated partial thromboplastin time of 36.30s. The liver function test, urea, creatinine and electrolytes, and serum lipase and amylase were within the normal ranges, as shown in Table She underwent a computer tomography angiography (CTA), which showed evidence of abdominal aortic anterior mural thrombus measuring 27x11x8.5 mm in diameter . The hematologist later saw her later and was shifted to enoxaparin and aspirin. She tolerates oral feeding without having abdominal pain. She was discharged 10 days after her admission.The celiac trunk, also known as the celiac artery, is a short vessel that arises from the aorta and passes below the median arcuate ligament, just as the aorta enters the abdomen at the level of the T12 vertebra. The celiac trunk is classically divided into three major branches: left gastric, common hepatic, and splenic artery. It supplies blood to the foregut, namely the distal esophagus, stomach, second part of the duodenum, liver, pancreas, gallbladder, and spleen [Thrombosis of the celiac trunk is an uncommon etiology of acute mesenteric ischemia. Early diagnosis with immediate angiography and revascularization is lifesaving . The disAcute occlusion of the celiac trunk results in ischemic necrosis affecting mainly organs supplied by end arteries. The clinical presentations and the outcomes of celiac trunk thrombosis are variable. The stomach and spleen are more susceptible to ischemia and infarction, and dual blood supply and superior collateral flow ensure that the liver is resilient to the effects of thrombosis. Ali-Akbarian M et al. reported a case of celiac trunk thrombosis associated with a completely gangrenous stomach, the first and second portion of the duodenum, omentum, and diffuse ischemic changes and infarction of the liver and spleen . In our Splenic infarction is common with celiac trunk thrombosis because the splenic artery is the main blood supply of the spleen. Patients may have sudden onset abdominal pain that usually resolves gradually, without clinical sequelae, and surgery is required when complications arise . An uncoThrombosis of the celiac trunk is a rare cause of acute abdominal pain. The clinical presentations and the outcomes of celiac trunk thrombosis are variable. Diagnosis depends on a high index of suspicion, and CTA is the gold standard to confirm the diagnosis. Successful treatment depends on the early establishment of the blood flow by anticoagulant, surgery, or endovascular intervention.


Neurological vertigo is a common symptom in children and adults presenting to the emergency department (ED) and its evaluation may be challenging, requiring often the intervention of different medical specialties. When vertigo is associated with other specific symptoms or signs, a differential diagnosis may be easier. Conversely, if the patient exhibits isolated vertigo, the diagnostic approach becomes complex and only through a detailed history, a complete physical examination and specific tests the clinician can reach the correct diagnosis. Approach to vertigo in ED is considerably different in children and adults due to the differences in incidence and prevalence of the various causes. The aim of this systematic review is to describe the etiopathologies of neurological vertigo in childhood and adulthood, highlighting the characteristics and the investigations that may lead clinicians to a proper diagnosis. Finally, this review aims to develop an algorithm that could represent a valid diagnostic support for emergency physicians in approaching patients with isolated vertigo, both in pediatric and adult age. Vertigo can be defined as a disorder of space sensitivity classically described as an unpleasant illusion of motion of the patient or the environment –3. It isAlthough vertigo is usually a symptom of peripheral causes as opposed to dizziness, neurological conditions may often presented with vertigo and the spectrum of differential diagnoses is broad and different for adult and pediatric population. The main causes of adulthood neurological vertigo, primarily represented by acute vascular injuries, are uncommon or totally absent in childhood , 5. More2, CATCH2, STANDING scores) [A proper diagnostic evaluation essentially includes a stepwise detailed history, a careful physical examination and further tests based on clinical indications . A metic scores) . Lastly, scores) .To date few studies have tried to validate specific diagnostic approaches for neurological vertigo and, in particular for patients with vertigo as exclusive clinical manifestation, there are no standardized diagnostic algorithms . The aimWe carried out a systematic review of the literature using PubMed/NCBI database, through a comprehensive MEDLINE search, in order to identify all the available studies describing the isolated neurological vertigo. The following search words were used: “isolated neurological vertigo” and “isolated vertigo children”. The search was conducted between May 19th, 2020 and July 10th, 2020 and it collected studies published between March 1989 and July 2020. We included all original articles  written in English, in which subjects presented with isolated vertigo. Our research included not only diseases characterized by vertigo as the only clinical manifestation, but also all the potential causes that, even if usually associated with other symptoms, may initially present with vertigo as exclusive complaint or could be associated to signs highlighted only during the clinical examination. We included studies involving both pediatric and adult population. All the reviewers worked independently in selecting the studies and extracting information about epidemiological, clinical and diagnostic features of patients presenting with isolated vertigo. Finally, we proposed two distinct diagnostic algorithms to ascertain the differential diagnosis of isolated neurological vertigo in pediatric  , 14. NeuConversely, in adulthood, isolated vertigo usually represents the first symptom of an acute vascular disease or a brain tumor , 27, 31.Our systematic review revealed that vertigo is a common symptom and an indicator of several diseases in childhood and adulthood. When associated with other symptoms, it is easy to distinguish among differentials. Conversely, when the patient shows isolated vertigo, the diagnostic approach becomes more complex and only through an accurate anamnesis, a complete physical examination and specific tests the clinician can achieve the correct diagnosis. Causes of vertigo in childhood present an age-dependent distribution which may be helpful in narrowing the differential diagnosis. For example, children under five years of age are usually diagnosed with BPVC (71%), followed by VM (19%). These two entities represent the most common causes of vertigo in children under 10 years of age as well, even if with different prevalence (each representing approximately 30% of cases). On the contrary, adolescents are most commonly diagnosed with VM . Also inIt needs to be highlighted that bias can arise at all stages of the review process. Hence, we have made more than an effort to minimize the risk of bias in this review identifying possible concerns with the review process. We tried to follow the ROBIS tool for the assessment of bias in the systematic review. Study eligible criteria have been pre-defined to find relevant literature on vertigo to develop an algorithm. The selection of a single database and English language might have limited the search strategy and this may represent a possible source of bias. However, the search strategy was carried out by two authors (NP and VD) independently, with the second reviewer checking the decisions of the first reviewer. Bias may also have arisen from the process of data extraction which is, by its nature, subjective and open to interpretation. Hence, extraction of data was performed by all the author involved working together to develop the algorithm. We calculated an overall level risk of bias as “low-moderate”, according to the ROBIS tool .Vertigo in pediatric age is a challenge due to different etiopathologies, the short-lived manifestations owing to rapid compensation, the inability of children to describe the experienced symptoms and the feasibility of diagnostic tests .In childhood, the most common causes of isolated neurological vertigo are VM and BPVC, although frequencies vary between different studies , 12, 19.In this systematic review we proposed an algorithm to facilitate the approach to children presenting to the ED with isolated vertigo, based primarily on a detailed history and a careful examination.VM is the most common cause of vertigo in children, accounting for 24% of all vertigo causes and is more frequently observed in adolescents . It is cPatients typically present with episodic attacks of spontaneous or positional vertigo that can last between 5 min and 72 h and can be associated with headache, photophobia and phonophobia , 57. TheBPVC is an episodic syndrome with short (seconds to minutes) recurrent attacks of subjective or objective vertigo, which resolves spontaneously. BPVC is a common cause of vertigo in childhood and it precedes the onset of migraine in over 35% of children by many years , 7, 59. Arteriovenous malformations presenting with vertigo symptoms are more frequent in the pediatric population (35%) than in adults (6%) and symptoms are related to the compression of the vestibular nerve or the brainstem nuclei . Among nBrain tumors are not a common cause of vertigo in children. Tumors of the posterior cranial fossa rarely begin with vertigo in the pediatric population (< 1%). More commonly they present with headache, vestibular symptoms or additional neurological deficits due to the compression or involvement of the nearby nuclei and fiber tracts , 7, 63. Vertigo, usually lasting days or weeks, has been reported as initial sign of MS in 20–50% of patients . The acuOrthostatic hypotension determines vertigo in 3–9% of symptomatic children. Affected children become symptomatic within 3 min of moving from posture to sitting or standing from a supine position. It can occur as isolated symptom or associated with autonomic dysfunctions . It is fVN represents between 1 and 16% of cases of pediatric vertigo and it is commonly founded in children older than five years of age and adolescents . It is aThough rare, vestibular paroxysmia can cause multiple short episodes of rotatory or to-and-fro vertigo, up to 30 times or more in a day, that last from seconds to minutes . In someFamilial episodic ataxia type II is a rare autosomal dominant disorder characterized by vertigo that lasts for minutes to hours and ataxia, typically triggered by sport, stress and alcohol . This diTuberous sclerosis is an autosomal dominant disorder characterized by hamartic development in several organs, most notably brain, heart, kidneys, lungs and skin. To date, only one case report described a 9-year-old patient with episodes of vertigo and headache followed by full spontaneous recovery as initial symptom of tuberous sclerosis .Head trauma from falls and whiplash injury are possible cause of vertigo among children, owing to the labyrinthine concussion or the development of perilymphatic fistula , 7. ChilDespite a large number of drugs including vertigo as a possible side effect, iatrogenic forms are rarely reported in children .Anisometropia  and otheFinally, the differential diagnosis of childhood vertigo comprises somatoform vertigo, commonly found in adolescent girls that usually present with isolated episodic or chronic vertigo with normal findings on physical examination . A carefIn adulthood, vertigo can occur as a result of conditions related to impaired cerebral circulation, especially of the vertebrobasilar district  or it caAs per children, we proposed a diagnostic algorithm to guide clinician’s approach to adults with isolated neurological vertigo in ED.Acute isolated vertigo can frequently occur in patients suffering from stroke in the distribution of the vertebrobasilar circulation . The freDifferentiating isolated vascular vertigo from other disorders involving the inner ear is difficult, especially when AICA territory, that supplies both peripheral and central vestibular structures, is involved . PatientVertigo can be the only presenting symptom in patients with posterior inferior cerebellar artery (PICA) infarction  and it iIsolated vertigo can result from dorsal medullary infarction, which can involve vestibular nuclei, nucleus prepositus hypoglossi or inferior cerebellar peduncle . ClinicaIsolated intermittent vertigo can be rarely the consequence of hypoperfusion of the flocculo-nodular lobe due to a persistent trigeminal artery (PTA) or posterior circulation stroke in the context of a PTA . FinallyAlmost all the patients with vascular vertigo show central vestibular signs, such as gaze-evoked nystagmus, normal or contralesional head-impulse test (HIT), skew deviation and central patterns of spontaneous nystagmus . Head-ImBrain tumors account for approximately 2% of all cancers . Even ifMigraine should always be considered among the causes of isolated neurological vertigo because vertigo may be one of the symptoms preceding or manifesting during migraine attack . FurtherUnexpected diagnoses of MS have been made in patients who had an isolated positional vertigo as the only symptom . ResponsEven neuromyelitis optica spectrum disorders (NMOSD) can rarely present with isolated vertigo, especially early in the disease course . VertigoVN is a condition caused by inflammation of the vestibular nerve commonly seen in middle-aged adults  as conseArnold-Chiari malformation is a rare condition in which a displacement of the cerebellar tonsils can affect functions controlled by the cerebellum and brainstem thus causing vertigo.Episodic ataxia type 2 is a rare condition, allelic with hemiplegic migraine and spinocerebellar ataxia, caused by an autosomal dominant mutation in the CACNA1A gene resulting in the dysfunction of voltage-dependent calcium channels . The onsRepetitive vascular compressions in vertebral arteries (bowhunter’s syndrome)  or atherOur systematic review demonstrates that isolated neurological vertigo could be due to different causes in childhood and adulthood. VM and BPVC are the most frequent disorders in children suffering from isolated vertigo presenting the ED; meanwhile the same symptom in adults is more frequently related to impaired distribution of the vertebrobasilar circulation, especially in the older ages. Age may be helpful in narrowing the differential diagnosis. In the majority of the cases, an appropriate diagnosis can be established thorough a careful history collection and a complete clinical examination, as illustrated in the algorithms proposed in the paper that could be an important tool for a prompt differential diagnosis. Indeed it is crucial to be aware of those differentials for pediatric and adult age to be able to make the proper diagnosis and manage those patients appropriately.


Angiolipomas are rare, benign tumors resulting from the proliferation of adipose tissue and blood vessels, most frequently encountered subcutaneously at the upper limbs and trunk level. Due to their rarity, few cases of intraosseous angiolipomas are presented in the literature. The paper reports a 50-year-old female case with intracranial hypertension syndrome, frontal and parietal headache, nausea, and vomiting symptoms increasing in intensity. A CT exam revealed two hypodense expansive intraosseous formations/lesions. The first one was located in the projection of the frontal bone and the second one was placed on the left parietal bone. After further investigations, a two-stage procedure was considered. A frontal craniotomy with excision of the intraosseous tumor was performed in the first stage. In the second stage, a left parietal craniotomy was done with excision of the intraosseous tumor combined with a cranioplasty procedure. The patient had a favorable postoperative evolution with no symptoms or neurological deficits. This is among the few reported cases of intraosseous angiolipoma located at the cranium level and the first case report of two intraosseous angiolipomas situated on the same site. The medical recommendation was a complete surgical excision of the lesion followed by cranioplasty. In the context of rare diseases, a case report represents a valuable resource that supports medical research and education ,2. AngioIntraosseous angiolipomas (IOALs) are among the rarest benign bone tumors, first described by Polte et al. in 1976 . With anThere are only a few cases of IOALs reported in the literature and even fewer IOALs of the cranium.We performed a systematic review of the literature in two of the most prestigious databases (PubMed and Web of Science) using an advanced search function on 1 March 2022. The search was conducted using Boolean search operators and MeSH terms: “((intraosseous angiolipoma) AND ((skull) OR (cranium)))”. PubMed returned seven articles and Web of Science four articles using this method. One article was removed from the PubMed search results since it presented a lipoma and not an angiolipoma of the skull. All articles from Web of Science can be found in PubMed databases. Including our case presentation, only seven cases of intraosseous angiolipoma of the skull are described in the literature. The clinical cases description is summarized in Yu et al.  reported3 located in the right frontal calvarial part, including trabeculated bone and fatty components with no invasion of the soft tissue and brain parenchyma. The surgical treatment for angiolipoma was divided into two steps. Firstly, an image-guided craniectomy was done to cut the calvarial tumor. After that, a titanium mesh cranioplasty was applied. No residual tumor was reported, and after postoperative day 2, the patient’s headache was improved. The histopathological investigation showed that the adipose part was composed through normal lobules, separated by thin fibrous septa.Another interesting case of IOAL was described by Nguyen et al. . In theiAtilgan et al.  presenteA giant intradiploic angiolipoma of the skull was reported by Amirjamshidi et al. . A 41-ye3 right parietal calvarial mass. MRI showed a lesion placed at the right parietal bone center with an extension to the cranial vertex. During the surgery, it was noticed that the tumor was extradural, and it had developed into a cranial cavity, with a biconvex shape. In order to fix the osseous defect, a Synthes PEEK implant was used. In this case, histopathology evidenced mature adipose tissue with thin blood vessels. After the surgery, the patient recovered well, and no signs of cosmetic deformities were noticed.Singh et al.  reportedMorgan et al.  reportedOur current study includes two intraosseous angiolipomas of the cranium, and according to our knowledge, this is the first case with two IOALs reported in the literature. We will highlight our findings and try to present the possible theories of the lesion genesis according to the above-described literature review. The patient diagnostic included the MRI and CT scan investigations and immunohistochemical characteristics.We present the case of a 50-year-old female patient that came to the Emergency Department with intracranial hypertension syndrome, accusing frontal and parietal headache, nausea, and vomiting symptoms from a month before, increasing in intensity.2 and another on the left parietal level measuring 70 × 21 mm2. Both lesions were present with a biconvex imaging appearance, hypodensity, multiple interior calcifications, and a marked thinning of the inner and outer plates, exerting a compressive effect on the cerebral parenchyma.After CT cranial exam, two expansive intraosseous formations were found: one in the projection of the frontal bone measuring 80 × 34 mmThe patient was admitted to the Neurosurgery Clinic for specialized treatment. Under depletive treatment, the symptoms of intracranial hypertension were remitted. A cranial MRI scan confirmed the nature of the compressive extra-axial left frontal and parietal intraosseous lesions on the cerebral parenchyma, as seen in The patient consented to a two-stage procedure consisting of a craniotomy with total excision of the frontal intraosseous tumor in the first stage, followed by a second craniectomy in the left parietal region with total excision of the intraosseous tumor and a titanium cranioplasty intervention. In the first stage, surgery was performed on the frontal lesion. A bilateral frontal craniotomy with excision of the intraosseous tumor and the reconstruction of the area using the intact external plate was performed.In the second stage, 6 weeks later, surgery was performed for the second lesion located in the left parietal region. A left frontoparietal craniectomy was performed with complete ablation of the lesion. A titanium mesh with self-tapping titanium screws adapted for the bone defect was used for the reconstruction of the skull. Due to the large size of the bone defect, it was decided to strengthen the titanium mesh by applying a polymethylmethacrylate (PMMA) cover. Clinical images of the procedure are represented in Specimens were cut and stained with hematoxylin and eosin (H&E) and Masson’s trichrome. Histological evaluation revealed a mixture of mature adipose tissue and thin-walled blood vessels. A general aspect of the tissue can be observed in The microscopic examination presented in The patient presented a favorable postoperative evolution with no symptoms and neurological deficits. The cerebral volumetry and the aesthetic aspect were restored. The patient was seen at regular time intervals of 1-, 6-, 12-, and 24-months remaining symptom-free with no recurrence after a follow-up of 2 years.The case described herein involves two expansive intraosseous angiolipomas of the cranium, one located in the frontal bone and the other in the left-parietal bone. The lesions presented a hypodense aspect with multiple interior calcifications and a marked thinning of the inner and outer plates. Further MRI investigations confirmed the expansive characteristic of intraosseous lesions that exerted a compressive effect on the cerebral parenchyma. Given the presence of two extended IOALs of the cranium, a two-stage procedure was considered.Angiolipomas represent tumoral proliferations formed of various blood vessels  and trabecular bone tissue, with areas of hemorrhagic necrosis sometimes present. The term angiolipoma was firstly described by Howard and Helwig in 1960 . AngioliCurrently, modern imaging techniques manage to narrow the differential diagnosis and offer better insight . A CT scAs we have investigated in the literature, just a few reported cases of IOALs of the cranium were reported . SummariWe report a case of two rare intraosseous angiolipomas of the cranium situated in the frontal and parietal bone, the first such case documented and among the few intraosseous angiolipoma of the cranium reported in the literature. It is important to understand the extent of this disease and how to perform a differential diagnosis and manage the intervention properly. We recommend the main treatment for intraosseous angiolipomas, which consists of complete surgical excision of the lesion. It should be noted that even benign tumors can affect the bone up to destruction and create multiple abnormalities.


There are multiple surgical procedures for resecting non-ampullary duodenal neoplasms (NADNs), and the appropriate method is selected depending on the tumor location and diagnosis. We herein report 3 cases of NADNs that were resected using pancreas-preserving partial duodenectomy (PPD).The first patient, a 73-year-old woman with a circumferential duodenal adenoma in the supra-ampullary duodenum, underwent surgery. After laparotomy, the duodenum proximal to the tumor was confirmed using intraoperative endoscopy and dissected. The duodenum distal to the tumor was dissected under direct visualization, and the specimen was removed. The distal stump of the duodenum was closed, and duodenojejunostomy was performed as described by Billroth II. The tumor was diagnosed as an adenoma 75 mm in size. She was discharged 12 days after surgery without any complications. The second patient, a 48-year-old man, was diagnosed with a neuroendocrine neoplasm (NEN) with a diameter of 14 mm in the supra-ampullary duodenum. Laparoscopic PPD was performed. He was diagnosed with NEN G1 and discharged the 11th day after surgery. The third patient, a 71-year-old man with a 0–Is + IIa lesion in the horizontal duodenum, underwent surgery. After laparotomy, the horizontal duodenum and proximal jejunum were resected, and duodenojejunostomy was performed. The patient was diagnosed with stage I adenocarcinoma and discharged on the 15th day after surgery.PPD is useful for avoiding the morbidity of pancreatoduodenectomy in the management of NADNs without invasion to the ampulla of Vater or pancreas. Pancreatoduodenectomy (PD) is often considered a surgical procedure for duodenal tumors. However, PD is a complex surgical procedure with a high rate of postoperative complications. For non-ampullary duodenal neoplasms (NADNs), PD can carry a higher morbidity (including morbidity from pancreatic fistulas) due to the soft pancreatic texture and the small size of the pancreatic duct, which contributes to reduced survival , 2. PancThe first patient was a 73-year-old woman whose duodenal tumor was detected during preoperative examination for ovarian cancer. Upper gastrointestinal endoscopy revealed a circumferential papillary tumor located from the duodenal bulb to the descending duodenum which was diagnosed with duodenal adenoma Fig. a. One yeA 48-year-old man had a flattened elevated lesion 2 cm in size on the anterior wall of the descending duodenum, and a biopsy revealed a neuroendocrine neoplasm  Fig. a. He undA 71-year-old man had a semi-circumferential 0–Is + IIa lesion in the horizontal duodenum Fig. a, b, andThe treatment strategies for NADAs are unclear. Tumors that are not amenable to endoscopic resection require operative intervention. PD has been the standard operation for invasive lesions that involve the ampulla of Vater or that require lymph node dissection. PPD are usually indicated for select tumors, including gastrointestinal stromal tumors, large adenomatous lesions, T1a adenocarcinoma, and small NEN, that has no risk of LN metastasis. The choice of PPD versus PD is dependent on factors, such as tumor size, location (proximity to the ampulla of Vater), risk of lymph node metastasis, and patient’s overall fitness. However, there are few reports comparing oncological results between PD and PPD, and an ideal surgical approach has not yet been established. Including total duodenectomy, supra- or infra-ampullary partial duodenectomy, and wedge resection of the duodenum, PPD may be one of the curative treatments for non-ampullary duodenal neoplasms depending on the tumor location and diagnosis . When PPPPD has several advantages over PD in terms of operative complexity and organ preservation that leads to a lower incidence of postoperative complications and preservation of pancreatic function. There are some reports that show the usefulness of PPD, but the overall survival was not significantly different, although patients undergoing PD had a higher morbidity –6. An imWe performed partial duodenectomy rather than local resection because of lymph node dissection around the supra-ampullary duodenum for the second patient with NEN. Soga et al.  reportedThe anatomical features of the duodenum, such as the narrow lumen and thin wall, make endoscopic resection of tumors difficult. To prevent complications of ESD, laparoscopic–endoscopic cooperative surgery (LECS), in which the defect of the duodenal wall by endoscopic procedure is closed by laparoscopic suture from the outside of the duodenum, was recently implemented to treat patients with NADA . AlthougWe agree that PPD is an attractive and promising alternative procedure to PD for the treatment of NADNs, especially small NENs, gastrointestinal tumors, large adenomas, and early NADAs. The technical points and pitfalls of this operation have not been sufficiently discussed thus far. Further large studies to examine the safety and curability of PPD are needed in the future.PPD is useful in the management of non-ampullary duodenal neoplasms without invasion to the ampulla of Vater or pancreas, avoiding the morbidity of PD. Appropriate preoperative examinations and treatment strategies should be conducted with consideration for organ preservation and curability.


Thalamic astrocytomas are rare central nervous system tumors that account for 1%-1.5% of all brain tumors. Their Clinical features depend on anatomical involvement. For these tumors, gross total resection is so difficult due to their deep location and also the infiltration of the optic pathway or brain stem. Unilateral adult thalamic locations are rarely described in the literature. Their radiological features often suggest lymphoma. The authors report here a new case of a primary unilateral thalamic pilocytic astrocytoma mimicking lymphoma diagnosed after a stereotactic core biopsy in a 62-year-old male patient with von Recklinghausen's disease and which is responsible for Dejerine–Roussy syndrome. The authors will proceed with a comprehensive review of literature regarding this rare entity. Pilocytic astrocytoma (PA) is a tumor originally described by Harvey Cushing in 1931 The authors report here a case of a primary unilateral thalamic pilocytic astrocytoma mimicking lymphoma in a 62-year-old male with von Recklinghausen's disease which is responsible for Dejerine–Roussy syndrome. The patient underwent a stereotactic core biopsy using the Lars Leksell with uneventful course. The authors will proceed with a comprehensive review of literature regarding this rare entity.A 62-year-old male patient with medical history of von Recklinghausen's disease since childhood not well investigated and acute pancreatitis 8 months before admission without any particular surgical history who was admitted to our neurosurgery department for progressive onset, 2 months before admission, of lower and upper left limbs burning and tingling sensations, widely varying in degree of severity during the day accompanied by hypersensitivity in form of dysaesthesia associated to a moderate gait disorder.Upon neurological examination we discovered hemiparesis and hyperesthesia of his left body with anosognosia and somatoparaphrenia defining the Dejerine–Roussy syndrome. The rest of the body examination revealed several soft tissue cutaneous nodules (neurofibroma), covering the whole body including the head, neck, chest, limbs, dorsal region and even the scalp with multiple hyperpigmented macules (Café-au-lait spots) without freckling in the underarms (axillary) or groin  regions defining the von Recklinghausen's disease (Neurofibromatosis type I) . Deep opCerebral magnetic resonance imaging (MRI)  showed aThe biological assessment of white and red blood cell showed no abnormalities as well as in platelets and C-reactive protein test. Serology for Hepatitis B, C, and human immunodeficiency virus were negative.Our patient underwent a stereotactic core biopsy using the Lars Leksell Stereotactic system with scannographic tracking to allow the brain mapping in a 3-dimensional coordinate system and select the appropriate target coordinates for guiding the biopsy needle. The procedure was performed under local and regional scalp nerve block.Histological examination revealed a glial proliferation with moderate cellularity, sitting in a fibrillary background. The tumor has a biphasic pattern alternating with compact and loose areas A. NeoplaPostoperatively, no complications or worsening of his neurological condition were observed.PA is a tumor originally described by Harvey Cushing in 1931 It is currently not possible to predict the degree of aggressiveness of PA on radiological data alone; several criteria were studied: location, morphology, size, presence of calcifications, and intensity of contrast enhancement Therapeutically, when anatomical conditions allow, PA may and should be removed entirely Chemotherapy is an important part of the treatment of diencephalic and hypothalamic-chiasmatic PAs. Many protocols exist to treat diencephalic  PA, especially described by North American or Japanese teams, most often on small cohorts of patients. The purpose is to achieve tumor control and to avoid or delay radiotherapy (RT). The best indication seems to be progressing PA, for which no reasonable surgical intervention can be considered, or children with a worsening of general condition to undergo surgery and under aged for RT Some authors no longer use RT as first-line , or second-line after incomplete resection PA generally has an excellent prognosis, but recurrent thalamic and hypothalamic forms may lead to the patient's death, most often after a long course marked by multiple recurrences PA is an original, very atypical tumor that abounds in paradoxical features and has a good prognosis. Very little data is available with regard to thalamic and hypothalamic PA. Complete excision, if ever possible, remains the treatment of choice. The high chemo- and radiosensitivity of these tumors is a notion to be balanced, on a case-by-case basis, with the possible morbidity of a surgical procedure. Moreover, the histological grading system used for glial tumors is poorly adapted and proves disappointing for PA. The mitotic index seems to us to be an important data to collect, the only objective factor for these tumors with such unpredictable evolution, and can participate in the decision to prescribe adjuvant treatments.Written informed consent was obtained from the patient for publication of this case report and any accompanying images.


Persistent Mullerian Duct Syndrome (PMDS) is a rare disorder of defective sexual development in males. It is characterized by aberrant synthesis or inadequate action of Mullerian inhibiting factor resulting in derivatives of Mullerian duct, i.e. uterus, fallopian tube and upper vagina, to persist in a phenotypic male with 46XY karyotype. Here, we report a 19-year-old male with bilateral undescended testes. Further evaluation revealed that the patient had both his testes placed intra-abdominally along with a rudimentary uterus. Persistent Mullerian Duct Syndrome (PMDS) is an autosomal recessive disorder where a phenotypic male with normal external genitalia has a concurrent uterus, fallopian tube and/or upper third of vagina. It results from mutations resulting in the inability of Sertoli cells to produce anti-Mullerian hormone (AMH) or a defect in AMH Type II receptor, both of which are involved in male sex differentiation. PMDS is usually an incidental finding in patients with cryptorchidism. Mostly, only one testis is undescended in such patients with the other one present in its typical position; however, both testes can also be undescended.A 19-year-old male was admitted to the department of general surgery at a tertiary care hospital with an empty scrotum. On examination, he had a typical male appearance with well-developed secondary sexual characteristics and a normal distribution of axillary and pubic hair. His penis appeared normal and his testes were not palpable. His abdomen was soft, non-tender, and had no palpable mass or visceromegaly.His ultrasound demonstrated an empty scrotum and the testes were not detected in any ectopic location including the inguinal canal and pelvis bilaterally. Subsequently, a computed tomography (CT) scan with IV contrast was performed, which revealed well-defined enhancing areas in the lower abdomen measuring 2.5 cm × 2 cm on the left and 2.5 cm × 1.6 cm on the right correlating to the undescended testes. Furthermore, a soft tissue density structure likely to be a rudimentary uterus measuring 5.8 cm × 4 cm was seen posterior to the urinary bladder .The patient underwent semen analysis, which showed azoospermia.His hormonal analysis showed normal values of the following:Serum anti-mullerian hormone, i.e. 11.95 ng/ml.Sex hormone binding globulin, i.e. 15.77 nmol/L.Serum testosterone, i.e. 9.16 nmol/L.Estradiol, i.e. 17.43 pg/ml.However, he had increasedLH, i.e. 19.89 IU/L andFSH, i.e. 23.12 IU/ml.A tissue from buccal mucosa was sent for karyotyping, which showed a 46XY configuration of chromosomes.The patient and his family were counseled about the patient’s situation and were informed of the available options. A well-informed consent was obtained from him and his parents for the removal of the rudimentary uterus and both his testes. We used a laparoscopic approach to visualize the presence of testes in the right and left iliac fossas, which were attached to a rudimentary uterus, located posterior to the urinary bladder through the infundibulopelvic ligament . A roundThe post-operative period for this patient was uneventful and, hence, he was discharged on the second post-operative day. He was also started on exogenous testosterone to maintain virilization.Biopsy of his testes revealed benign atrophic changes with no evidence of germ cell neoplasia, granuloma or malignancy. Section of his uterus showed endometrial and endocervical lining without the presence of malignancy.Nilson first described PMDS in 1939 .Although patients with PMDS often present in their pediatric or adolescent years, the oldest age of presentation was 77 years . Its preUltrasonography is the initial diagnostic modality of choice. It tells us about the location of testicles along its path of descent and other sexual organs, hernial sac and contents, uterine wall, etc. However, it is not very specific for the identification of intra-abdominal testicles . CT scanTreatment strategy has remained controversial, especially regarding the removal of Mullerian remnants. There are 15–40% chances of germ cell tumors in patients with PMDS necessitating intervention . This isNone declared.None declared.All data used for this article is publicly available and accessible online.


Subsequently, the patient underwent surgery and the postoperative pathology and immunohistochemistry confirmed MFS. In conclusion, MFS has rarely been reported to arise from the surgical site of squamous cell carcinoma. Our case study demonstrates that MFS should be included in the differential diagnosis of superficial masses in patients with a prior surgical history who present with a soft tissue mass at the surgical site, especially for recently developed rapidly increasing masses. This study aimed to systematically review the clinical features, diagnosis, differential diagnosis, treatment, and prognosis of this disease based on our case and related published literature and to provide clinicians with a broader perspective on the differential diagnosis of soft tissue tumors.Myxofibrosarcoma (MFS) is a rare soft tissue sarcoma that originates in the mesenchymal tissue and occurs mainly in the limbs of elderly patients. Herein, we present the case of a 64-year-old woman who underwent extensive left vulvar resection and bilateral lymphadenectomy for vulvar squamous cell carcinoma 6 months previously. A mass was found again at the original surgical site of the left groin 3 months prior, and its size had increased significantly in the past 1 month, with ulceration and pus. Magnetic resonance imaging (MRI) showed a 10-cm mass in the left groin area; fluoro18-labeled deoxyglucose positron emission tomography/computed tomography ( CT revealed a mass of approximately 3.5 cm × 2.0 cm  in her left vulva, and multiple enlarged lymph nodes were seen in the left groin area , Zunyi Medical College Research Start Fund 2018ZYFY03, and QianKeHe platform talents [2017] (grant numbers:5733-035).The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article, or claim that may be made by its manufacturer, is not guaranteed or endorsed by the publisher.