Introduction: Laryngeal neuroendocrine neoplasms (NENs) are a rare group of NENs of the neck, which commonly show immunostaining for calcitonin. Laryngeal NENs with calcitonin hypersecretion and lymph node metastases represent a diagnostic and therapeutic challenge, which should be included in the differential diagnosis of medullary thyroid carcinoma (MTC). We report a complex case of laryngeal NEN with calcitonin hypersecretion and a review of the literature.Case Presentation: A 59-year-old man presented with dysphagia, dyspnea, and lateral cervical mass; he was a smoker. At first imaging, a laryngeal lesion with lateral cervical lymphadenopathies was found, and it resulted as a moderately differentiated neuroendocrine tumor (G2), Ki67 = 5%, positive for calcitonin. Increased levels of serum calcitonin (50 pg/ml) were found. The patient started somatostatin analogs for lesions positivity to somatostatin receptor-based imaging. After 5 months, the disease progressed at 18F-fluorodeoxyglucose (18F-FDG) PET-CT, and also new painful cutaneous lesions occurred. Considering high serum levels of calcitonin, differential diagnosis with MTC was required. Patient performed a thyroid color Doppler ultrasound, nodule fine needle aspiration, calcitonin dosage in fine needle washout fluid, and a calcium gluconate stimulation test. After multidisciplinary evaluation, we decided to perform a total thyroidectomy associated with lateral cervical lymphadenectomy and resection of skin metastases. No MTC was found. Two of the five resected lymph nodes, left upper parathyroid, and skin lesions were metastases of NEN G2, positive for calcitonin. After 2 months, new painful skin lesions occurred, and a target therapy with everolimus 10 mg/day was started. After 6 months of therapy, partial metabolic response with a reduction of 53.7% of radiotracer uptake at primary tumor was detected together with an improvement of patient's quality of life.Conclusions: The present case is the seventh described in the literature of laryngeal NEN associated with elevated serum calcitonin levels and the first case with parathyroid metastasis, suggesting the importance of a correct differential diagnosis between MTC and calcitonin-secreting laryngeal NEN, using an integrated approach of biochemistry and advanced imaging. This is also the first time that somatostatin analogs and then everolimus were used in this setting, resulting in clinical and partial metabolic response. Laryngeal neuroendocrine neoplasms (NENs) are a rare group of NENs of the neck, divided into epithelial (carcinomas), and neural-type tumors (paraganglioma) . PrimaryA 59-year-old man attended the endocrinology outpatient clinic for the occurrence of dysphagia, dyspnea, and a left lateral cervical mass, which appeared 2 years before. He had a history of smoking one pack of cigarettes per day since age 18. In the last 7 years, he had a history of hypertension and hepatitis C treated with sofosbuvir and velpatasvir with negativization of viremia 4 years before. The patient had no familial history for neoplasms or endocrine diseases. On clinical examination, left lateral cervical mass was fixed and firm, and no other relevant alterations were found.In the diagnostic workup, contrast-enhanced computed tomography (CT) was performed and showed the presence of a vascularized lesion of the supraglottic left emilarynx , which invaded the left epiglottic vallecula, aryepiglottic fold, piriform sinus, and free wall of the epiglottis. CT scan revealed also thickening of the left vocal cord and adjacent soft tissue and the presence of multiple lateral cervical lymphadenopathies, the most evident on the left side , and one left supraclavear lymphadenopathy .At fibrolaryngoscopy, the laryngeal lesion was biopsied, revealing a moderately differentiated neuroendocrine tumor (grade G2), Ki67 index of 5%. Immunohistochemistry was positive for synaptophysin, neuron-specific enolase, chromogranin A, pan-cytokeratin, cytokeratin 19, and calcitonin. Circulating neuroendocrine markers (neuron-specific enolase and chromogranin A) were within the normal range, except for calcitonin .68Ga-DOTATOC PET-CT showed accumulation of radiotracer in the left epiglottic region (18F-FDG) PET-CT showed uptake in the left epiglottic region , but the size and the morphology remained unchanged. On the contrary, the left lateral cervical mass  was incremented in dimensions (21 vs. 18 mm). After 2 months, two pericentimetric cutaneous lesions appeared in abdominal and zygomatic regions, confirmed as cutaneous metastases of well-differentiated neuroendocrine tumor at biopsy.A c region . A somat18F-FDG PET-CT  and a new focus of uptake in another left lateral cervical lymph node . Clinically, dysphagia worsened, limiting the possibility to eat solid food, and pain became more frequent and severe, requiring oral morphine therapy.After 9 months of treatment, an G PET-CT  showed iConsidering high levels of serum calcitonin, differential diagnosis between MTC and calcitonin-secreting laryngeal NEN was required. A thyroid color Doppler ultrasound was performed, and it showed an irregular hypoechoic nodule (11 × 7 mm) with calcifications in the paraisthmic portion of the left thyroid lobe and also confirmed the presence of multiple lateral cervical lymphadenopathies . The nodMultidisciplinary evaluation by the Neuroendocrine Tumor task force (NETTARE) and thyroid cancer multidisciplinary unit of our University “Sapienza”–Policlinico Umberto I Hospital proposed total thyroidectomy for cytological diagnosis of TIR3B, associated to cervical lymphadenectomy and cutaneous metastases resection aimed to tumor debulking and pain relief.The histological examination revealed benign hyperplastic nodules in the thyroid gland, two of the five resected lymph nodes and cutaneous fragments as metastasis of a neuroendocrine tumor G2, positive for calcitonin, as shown in 18F-FDG PET-CT showed a reduction of 53.8% of the radiotracer uptake in the left emilarynx lesion  and borderline uptake at sternal body  and at left iliac fossa .After surgical intervention, the patient reported rapid pain relief, and oral morphine therapy was withdrawn. Unfortunately, after 2 months, disease progressed under SSA therapy with the appearance of three new cutaneous metastases. Therefore, we decided to start everolimus, with a starting dose of 10 mg/day. After 3 weeks of therapy, the patient developed oral mucositis (grade G3), characterized by pain and nutritional impairment, which required treatment withdrawal. After appropriate antifungal therapy, everolimus treatment was restarted at the half dose of 5 mg, then increased to 10 mg with good tolerance and safety profile. After 6 months from the beginning of the target therapy, We performed calcitonin stimulation test obtaining multiple blood samples to evaluate calcitonin levels before and after 1, 2, 3, 5, 10, and 15 min after calcium gluconate infusion (25 mg/kg) . Serum cAll the tissue specimens  were fixed with 10% formalin and embedded in paraffin. Hematoxylin–eosin (H&E)-stained sections (3 μm) were used for light microscopic examination. Immunohistochemical stains were performed by BOND-III automated IHC stainer  with the following BOND ready-to-use antibodies : pan cytokeratins CKAE1/AE3, chromogranin A, synaptophysin, CD56, CT, TTF-1, CEA, PAX8, and Ki67, using HRP-DAB detection system. Immunostaining slide with Ki67 was captured with Aperio scanner , and the nuclear positivity (in 500 tumor cells) was quantified manually by a pathologist on digital images and expressed as percentage.We report a complex case of laryngeal NEN with many peculiar diagnostic and therapeutic features. The importance and peculiarity of the present case are represented by the following points:  the rariLaryngeal NENs typically show neuroendocrine histological and immunohistochemical features including expression of chromogranin A, synaptophysin, and cytokeratins and can stain positive for calcitonin. Rare cases of MTC can extend into the larynx and must be distinguished from primary laryngeal NEN . EmbryolLaryngeal NENs commonly affect men more often than women 3:1), usually in the fifth to seventh decade of life, often with a history of smoking; the most frequent location is the supraglottis :1, usual. PatientThe finding of calcitonin levels higher than the normal range may require a confirmatory stimulation test, which can be useful to identify the possible coexistence of non-thyroidal NENs. In fact, calcitonin-secreting NENs can be distinguished from a C-cell disease by the absence of response to the stimulation test ; howeverIn the literature, the most cases of moderately differentiated laryngeal NENs were treated by radical surgery and chemoradiotherapy , 25. In The histological examination revealed benign hyperplastic nodules in the thyroid gland; no MTC was found, and confirmed lymph nodes and cutaneous metastases of NET G2, positive for calcitonin, and interestingly, showed a metastasis in the left superior parathyroid gland.In view of the positive uptake of Gallium peptides, indicating a neoplasm with high expression of somatostatin receptors, a first-line SSA therapy was started. No other cases of laryngeal NEN treated with SSA are reported, so it represents an unexplored medical treatment . In our Laryngeal NENs are rare neoplasms, which often represent a diagnostic and therapeutic challenge. We report a complex and peculiar case of a 59-year-old man, with a long history of smoking, affected by laryngeal NEN metastatic to nodes, skin, and parathyroid with elevated serum calcitonin levels. This case underlines the importance of a correct differential diagnosis between intrathyroidal or metastatic  MTC and calcitonin-secreting laryngeal NEN, which should include the measurement of calcitonin in the washout fluid of FNA and a calcium gluconate stimulation test. The management of this case required an integrated approach of biochemistry  and advanced imaging , allowing an absolute diagnostic completeness. The review of the literature showed only other six cases of calcitonin-secreting laryngeal NEN, often metastasizing to nodes and with painful cutaneous metastases, usually treated by radical surgery and/or chemotherapy. This is the first time, to the best of our knowledge, that the SSA treatment and then a targeted therapy with the mTOR inhibitor everolimus were used in laryngeal NEN, resulting in clinical and partial metabolic response with a reduction of 53.7% of radiotracer uptake at primary tumor. However, further studies are needed to confirm the suitability of this therapy and long-term outcomes.The original contributions presented in the study are included in the article/supplementary materials, further inquiries can be directed to the corresponding author/s.Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.TF is the first author for this case report and she contributed to the concept and design for the study. EG is the corresponding author supervising this work. AF, GP, FS, and RM contributed to the manuscript preparation. EA contributed to the Methods section in relation to laboratory dosage techniques. CG and RCa contributed to the tissue specimens  analyses, immunohistochemical stains, and related photos, as well as to the drafting of anatomopathological methods. All authors contributed to manuscript revision and read and approved the submitted version.The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.


Intraneural lipomas are rare soft-tissue tumors that can occur particularly within the median nerve. Even fewer cases have been reported of their occurrence within the ulnar nerve. These masses can cause compression neuropathies. In this report, we present the first documented case of an intraneural lipoma of an ulnar digital nerve. Intraneural lipomas and lipofibroma (librofibromatous harmatomas) are rare soft-tissue masses of peripheral nerves. These masses are benign adipose tumors that most commonly occur in the forearm and wrist. The first case of lipofibroma was reported in 1953 by Mason . These mIntraneural lipomas may be amenable to complete excision without sacrificing the offending peripheral nerve. Intraneural lipomas have a female predominance and, although rare, most commonly occur within the median nerve in the forearm and wrist . Some caA 25-year-old, right-hand-dominant female presented with a progressively enlarging mass along the ulnar border of her right ring finger. The mass extended proximally from her proximal phalanx to her middle phalanx, ~2 x 2 cm in size. The mass was soft, mobile, with no overlying skin changes. No pain, tenderness, paresthesias, or bruit were noted. There was no history of trauma. MRI was obtained and demonstrated a lobulated lipoma on the volar and ulnar aspect of the right ring finger extending from the proximal phalanx to the distal aspect of the middle phalanx Figure .Surgical excision was recommended, which the patient deferred at that time. The mass continued to enlarge over the next two years with the onset of worsening pain and paresthesias. Repeat MRI confirmed the diagnosis of intraneural lipoma of the ulnar digital nerve of the patient’s right ring finger. The patient agreed to surgical intervention.Upon exploration, the mass appeared to be a large, multilobulated, serpiginous, fibrofatty tumor consistent with an intraneural lipoma along the entire volar ulnar surface of the ring finger Figure .The lipoma was intimately associated and intertwined with the ulnar digital nerve, extending from the level of the A1 pulley proximally to the level of the distal interphalangeal joint distally. The ulnar digital artery was displaced by the mass. Meticulous dissection was undertaken to enucleate the intraneural lipoma, safeguarding against any injury to the ulnar digital nerve. The resection yielded a 9-cm multilobulated, serpiginous mass with no residual lesion Figure .After meticulous dissection to completely excise the intraneural lipoma, the ulnar digital nerve remained intact with noted areas of the previous compression Figure .Gross pathological examination of the specimen revealed a large, encapsulated mass comprised of fibroadipose tissue consistent with an intraneural lipoma. Postoperatively, the patient experienced some mild paresthesias along the ulnar aspect of her right ring finger, which did resolve over time. No strength or range-of-motion deficits were observed. Full recovery was achieved by the patient.Adipose tumors are the most common soft-tissue tumor; yet, in the hand, lipomas account for less than 5% of benign tumors ,10. AddiIntraneural lipomas and lipofibroma (librofibromatous harmatomas) are rare benign adipose tumors of peripheral nerves that most commonly occur in the forearm and wrist. The first lipofibroma was reported in 1953 by Mason . ClinicaMRI is diagnostic and helpful in distinguishing between the two entities . This imIntraneural lipomas, although rare, most commonly occur within the median nerve in the forearm and wrist . Some caIn this report, we presented the first documented case of an intraneural lipoma of an ulnar digital nerve and its successful complete excision. Benign fibroadipose tumors of the upper extremity are extremely rare. Progressive compression neuropathy symptoms may be noted given the tumor’s location and size. Surgical excision should be considered in patients with large masses, especially if symptomatic. MRI can help differentiate between an encapsulated, intraneural lipoma, which is amenable to complete excision, and an infiltrating lipofibroma, which may only be completely removed with nerve resection/sacrifice.


The cervical thymic cyst (CTC) is a rare, benign neck mass that most commonly presents in the pediatric population. These entities can occur anywhere along the normal path of descent of the thymus from the mandible to the sternal notch, and extension into the mediastinum has been observed. The presentation of these masses is often characterized by a painless, enlarging neck mass in a child during the first decade of life. Although most patients are asymptomatic, abutment of the cyst against local structures has led to a variety of presentations including respiratory distress. These rare lesions are noted to have a male predominance and most commonly present on the left side of the neck. We present the rare case of a 19-year-old male who presented with a left-sided painless, cystic neck mass. He underwent a computed tomography scan of the neck which showed a large cystic mass in the left neck deep to the sternocleidomastoid muscle. Preoperatively, the diagnosis of an infected third branchial cyst was favored. The lesion was completely excised in the operating room. Final pathology was consistent with a CTC. The CTC is an uncommon benign process that often presents as an asymptomatic cystic neck mass. Knowledge of the clinical presentation, diagnostic process, and treatment of these rare lesions is essential for the Otolaryngologist. Cystic neck masses represent a diverse clinical entity ranging from congenital cysts to metastatic malignant disease. Out of the broad range of benign cystic neck masses, the majority represent infected congenital neck masses such as branchial cleft or thyroglossal duct cysts. The cervical thymic cyst is an extremely rare cystic neck mass , 2. Of tA 19-year-old male presented with a large left-sided neck mass that appeared suddenly during an upper respiratory infection (URI). Apart from noticing the mass, the patient was asymptomatic and denied pain or dyspnea. He did not have a history of prior neck masses, trauma, recent travel, smoking, or animal exposure. A computed tomography scan of the neck with contrast was completed and revealed a 4 cm × 8 cm × 3 cm cystic mass in the left neck occupying nodal levels 2–4 deep to the sternocleidomastoid muscle . The radA CTC is an uncommon clinical entity, and there are few small case series describing these lesions , 2. A reThe origin of thymic cysts is not fully understood. The presence of asymptomatic thymic tissue in the neck is relatively common; however, a CTC is exceedingly rare. A thymic cyst can occur anywhere along the normal path of descent of the thymus from the mandible to the sternal notch. Various theories exist to explain the pathogenesis of these cysts. These entities may originate from degeneration of Hassall's bodies or possibly from remnants of the thymopharyngeal duct .Of the cervical thymic cysts reported, the most common presenting symptom is painless swelling in a child during the first decade of life. Upper airway symptoms such as dyspnea, dysphagia, hoarseness, and stridor have been reported in 6–13% of presentations . PreoperThe CTC is a rare benign cystic neck mass that most commonly presents in children during the first decade of life. Treatment of these cysts is with complete surgical excision, and the rate of recurrence is extremely low. Definitive diagnosis of these lesions depends on histologic examination of the excised specimen and can rarely be accurately diagnosed by preoperative imaging. We present the uncommon presentation of an adult male found to have a cervical thymic cyst. Although rare in clinical practice, the CTC should be kept in the differential diagnosis for cystic neck masses in both children and adults.


Pheochromocytomas are rare neuroendocrine tumors that can affect people of all ages and are commonly diagnosed in the 4th and 5th decades of life. Familial pheochromocytomas are diagnosed mostly between the 2nd and 3rd decades of life. They can be benign or metastatic and often present as isolated tumors or along with other neuroendocrine syndromes. We present a case of an elderly man who underwent laparoscopic adrenalectomy for pheochromocytoma at the age of 60 years but developed recurrence of metastatic pheochromocytoma after ten years. We also conducted a literature review to understand the epidemiology and presentation of the tumor and to emphasize that there should be a low threshold of suspicion for timely diagnosis and management of recurrent pheochromocytoma. Pheochromocytomas are very rare functional catecholamine secreting tumors . Eighty-The incidence rate of pheochromocytoma is 0.8 per 100,000 person–years ,4. UsualApproximately half of the patients with pheochromocytomas present with the paroxysmal classic triad, which includes episodic headaches, sweating and tachycardia ,6. AboutIn this report, we describe a case of a 70-year-old male diagnosed with a recurrent metastatic pheochromocytoma. When our patient was 60 years old, he was diagnosed with a right adrenal pheochromocytoma, which was treated by laparoscopic adrenalectomy. We report a rare case of metastatic pheochromocytoma recurrence in a patient 10 years later from the age of 60.A 70-year-old male with a significant past medical history of adrenal pheochromocytoma status post partial right adrenalectomy ten years ago, atrial fibrillation, hypertension, hyperlipidemia, was brought to the hospital with elevated blood pressure readings and palpitations.Ten years earlier, at age 60, this patient was brought to the hospital after he was found unresponsive in his apartment. He was admitted to the ICU (Intensive care unit) and was subsequently diagnosed with a stroke. During this hospital stay, he was found to have a right adrenal nodule which was diagnosed with a pheochromocytoma with positive synaptophysin/chromogranin/S100. He underwent a laparoscopic partial right adrenalectomy and was discharged home once his symptoms subsided. He followed up with his primary care provider regularly and his home blood pressure readings remained around 120/77–130/92 mmHg.Approximately ten years later, he returned to the hospital with palpitations and elevated blood pressure readings with systolic blood pressure of >=200 mmHg. He was diagnosed with another stroke. This time, he developed residual weakness and required the use of a walker due to unsteady gait. He was also diagnosed with atrial fibrillation and was placed on anticoagulants. He was discharged home and regularly saw his primary care physician and attended anticoagulation clinic. He sustained a fall at home one day while walking without a walker and hit his head. There was no loss of consciousness. He went to a clinic four days later where he was noted to be short of breath, pale with extremely high systolic blood pressure of 200 mmHg. Following his admission, he had INR  of 4 and hemoglobin of 7 g/dl. He was given 1 unit of red blood cells (RBC) and 2 units of fresh frozen plasma (FFP). Anemia work up showed Fe 55 µg/dL,% saturation 27%, TIBC 207 μg/dL, ferritin 193 ng/mL, B12 546 ng/mL and folate 17 ng/mL indicating anemia of chronic disease. He also had fever and leukocytosis with WBC of 15 µL and was diagnosed with pneumonia and treated with meropenem and doxycycline. His course was further complicated by atrial fibrillation with a rapid ventricular rate. He was put on alpha and beta-blockers; and transferred to subacute rehabilitation under our care for further management. He remained hypertensive at this time which prompted a workup for secondary causes of hypertension.His past medical history also included heart failure, chronic kidney disease, type 2 diabetes mellitus, anemia, anosmia, hearing loss, vitamin D deficiency, colonic polyps, depression, onychomycosis, hearing impairment, myopia and cataracts.Before admission, he was independent for all his activities of his daily living. His family helped him with the instrumental activities of daily living. He had 2–3 falls in the past year.His family history is notable for breast cancer in his sister and liver cancer in another sister. One sister had a kidney and liver transplant. No family history of colorectal cancer, multiple endocrine neoplasia syndrome or other endocrine-related anomalies were reported.His past medication history includes metformin, escitalopram, apixaban, melatonin, metoprolol and simvastatin. He was started on insulin at the hospital for his blood glucose maintenance.68Ga)Ga-DOTA-TATE) imaging showed liver and mesentery metastases.  and hyponatremia (sodium 131). His urine metanephrine was 556 mcg/24 h, normetanephrine was 22,624 mcg/24 h and creatinine was 2.56 mg/dl. His INR was 4.26 which was high. On imaging, there was an abdominal mass on the right upper kidney as well as an abdominal hematoma. Computer tomography (CT) of abdomen showed left lower abdominal wall hematoma and prominent multilobulated soft tissue mass within the right retroperitoneum superior to the right kidney in the region of right adrenal gland involving the liver, vena cava and kidney. Magnetic resonance imaging (MRI) of abdomen and pelvis showed postoperative changes after right adrenalectomy with 7.8 cm heterogenous multilocular enhancing mass in the surgical bed abutting the right posterior wall and close to the right kidney and Inferior vena cava (IVC). The mass and the enlarged regional lymph nodes together caused marked compression of the IVC. Heterogeneity and enhancing focus in the posterior right hepatic lobe indicated the invasion of the right adrenal gland.  Positronastases.  He was dHe was started on the alpha-blockers to control hypertension. After 36 days of admission, he underwent exploratory laparotomy, resection of retroperitoneal masses and an appendectomy. Findings during surgery were omental tumor, tumor at the tip of the appendix, tumor at the right lateral side of the liver; posterior liver/diaphragm mass; and posterior–porta hepatic mass. Pathologic examination of the masses revealed pheochromocytoma and paraganglioma of omental mass, appendix, diaphragm, sub-gall bladder nodule, right portal vein nodule, pericaval and retroperitoneal mass and retro portal mass. Chromogranin and S100 immunostains were positive.After surgery, the patient was transferred to the intensive care unit for monitoring. Three days after his surgery, the patient developed shortness of breath and bradycardia and had a pulseless electrical activity arrest. A code was run for 13 minutes, the airway was secured, and the patient had the return of spontaneous circulation. After one day, pupils became unequal overnight. CT imaging was negative for brain stem hemorrhage and EEG  was unremarkable for any abnormalities. However, ten days after the arrest, patient’s MRI of the head showed diffuse ischemic encephalopathy. He needed continuous ventilatory and circulatory support. The patient showed no improvement in his neurologic status. After 15 days, palliative care was consulted. A goals of care discussion was conducted with family in the wake of poor prognosis and the family opted for hospice style of management. The patient was extubated and provided comfort care and transferred to the hospice unit. He died two weeks later.Pheochromocytomas are rare tumors that were first described by Fränkel in 1886. Although most tumors are benign and are treated with surgical resection, metastatic transformation can occur decades later ,8. AboutDue to the production of epinephrine, norepinephrine and dopamine, the classic triad of sweating, headache and tachycardia can occur. Sudden spells of paroxysmal hypertension are a self-limiting symptom of pheochromocytoma .Pheochromocytoma, especially recurrent pheochromocytoma, is very rare at an older age. The average age of diagnosis is 47 years old for sporadic tumors not associated with hereditary causes. The average size of tumor 4.9 cm . In thisAccording to literature, clinical predictors for metastatic transformation of pheochromocytomas are tumor size and location. A tumor size of greater than 5 cm and extra-adrenal tumor location possess greater risk of metastatic transformation. In contrast, our case shows the location of the tumor to be adrenal– in which case, metastatic transformation risks include looking at dopamine secretion, other secretory molecules and tumor cell necrosis ,19,20,21The task force by Endocrine Society clinical guidelines subcommittee(CGS) guidelines recommend initial biochemical testing of plasma free or urinary fractionated metanephrines. The positive results are followed up by CT as initial test and MRI for patients with metastatic disease and when radiation exposure needs to be limited. They also recommend genetic testing for all patients. Perioperative blockade with alpha adrenergic receptor blocker and preparation including high sodium diet and fluid intake was recommended. Minimally invasive adrenalectomy was recommended for pheochromocytoma. Lifelong follow up is also recommended for recurrence and metastasis .For our patient, plasma free or urinary fractionated metanephrines were screened. Since he was positive CT follow up was also done. However, 123I-metaiodobenzylguanidine (MIBG) scintigraphy and genetic screening was not done in this patient. He underwent a preoperative alpha-adrenergic receptor blockade to reduce postoperative cardiovascular complications. We also did a 18F-FDG PET/CT scanning on the patient to test for metastasis.The 123I-MIBG or (131I-MIBG scintigraphy were used in nuclear medicine to detect pheochromocytoma . (18F-FDVHL), succinate dehydrogenase A (SDHA), succinate dehydrogenase B (SDHB), succinate dehydrogenase C (SDHC), succinate dehydrogenase D (SDHD) and succinate dehydrogenase AF2 (SDHAF2) and hypoxia-inducible transcription factors (2A HIF2A)) while cluster 2 tumors (rearranged during transfection (RET), neurofibromatosis 1 (NF1), transmembrane protein 127 (TMEM127) and MYC-associated factor X (MAX)) mutations that activates kinase signaling and protein translation. PPGLs show high degree of heterogeneity. Genetic screening algorithms using conventional Sanger sequencing (SS) as well as targeted gene panels (TGPs) were used as a diagnostic tool for detecting PPGL. Conventional Sanger sequencing (SS) are useful in syndromic presentation. Next-generation sequencing (NGS) technology is another valuable tool for genetic screening, but the test is expensive and due to the ethical concerns with incidental findings, they are mostly used in research settings. Targeted gene panels (TGPs) screens for genes that are systematically excluded in SS studies. DNA samples for these studies may the obtained from blood, saliva, DNA samples formalin-fixed paraffin-embedded (FFPE) tissue and frozen tumors [Germline mutations contributes to the tumor growth and progression. Around 40% of the pheochromocytomas and paragliomas (PPGL) carry germline mutation . It has n tumors ,27.The treatment approach to patients with pheochromocytoma must be individualized and this is especially true for the elderly. Alpha- and beta-adrenergic blockers and calcium channel blockers can be used to control the hormone-related symptoms. Current perioperative management of pheochromocytoma includes preoperative antihypertensive therapy, including alpha blockade for the negation of alpha 1 mediated vasoconstriction and beta1 mediated tachycardia and inotropy . The alpAll patients who undergo surgery must have a thorough cardiac evaluation. Patients who have longstanding tumors can develop severe dilated cardiomyopathy with varying degrees of heart failure, increasing perioperative risk. Our patient with atrial fibrillation, had and electrocardiogram (EKG) and echocardiogram (ECHO) before surgery, but still developed pulseless electrical activity (PEA) arrest two days post-surgery, further emphasizing the need for thorough cardiac evaluation and follow up.Pheochromocytomas are often radio and chemo resistant. However, the presence of somatostatin receptors in chromatin cells allow for the use of somatostatin analogs for localization during diagnosis and also the treatment of tumor. In patients who express somatostatin receptors and also have a positive 123I-MIBG scan, palliative debulking therapy with 131I-MIBG therapy can be used . The 131The risk-benefit analysis of surgical versus non-surgical management must be thoroughly assessed on a case-by-case basis. Non-surgical modalities can be used in conjunction or as the primary mode of treatment in some cases. Directed trans arterial chemoembolization, percutaneous tumor ablation and external beam radiation are some of the available non-surgical modalities. In surgery, R0 or R1 resection is a better approach compared to R2 or surgical debulking. A multidisciplinary approach is the best way for the management of this complex tumor. In our patient, due to the extensive spread of the tumor, surgical debulking was done.The overall 5-year-survival rate of patients with metastatic pheochromocytoma ranges between 34% and 60% .In conclusion, pheochromocytoma is an extremely rare tumor and the occurrence and reoccurrence in the elderly is even rarer. A low threshold for the tumor suspicion must be maintained if there is hypertension or cyclic hypotension and hypertension. Surgery is the standard treatment of the tumor with medication for symptom control.


The buccal space is an unusual location of malignancies. We report here the case of a woman with a melanoma metastasis in buccal fat pad, to evaluate the imaging features which might lead to the correct, although uncommon, diagnosis.A 71-year-old woman presented with a painless visible swelling of the left cheek. MRI revealed the presence of a solid lesion located in the buccal fat pad with features suggestive of malignancy. It showed T1 hyperintensity and T2 hypointensity, and restriction of diffusion. Histological examination showed neoplastic cells compatible with melanoma.The lesion features (T1 hyperintensity and T2 hypointensity) initially lead our team to believe that there was a hemorrhagic component, possibly a residue of the biopsy. However, when associated with other malignancy features, such as low apparent diffusion coefficient (ADC) values and contrast enhancement, they should evoke the suspect of melanoma, provided that no biopsy was performed and no trauma occurred in the 3–7 days before. The buccal space is one of the deep anatomical compartments of the head and neck regions, enveloped by the superficial layer of the deep cervical fascia Fig. . It is dMost of the buccal space is occupied by adipose tissue—the buccal fat pad—which laterally follows the parotid duct ending near the anterolateral portion of the parotid gland; medially, it projects towards the mandible and maxillary sinus; superiorly, it is divided by the temporal muscle into a superficial and deep portion and by the parotid duct into an anterior and posterior portion .The buccal space frequently communicates with the posterior masticatory space and with the infratemporal space; moreover, there is no real lower boundary with the submandibular space. This anatomical space can thus function as a conduit to spread infectious or tumoral tissue between the mouth and the parotid gland.Buccal space lesions are usually associated with cheek mass or facial swelling .Beside inflammatory manifestations and developmental lesions such as accessory parotid tissue (present in up to 20% of the population), dermoid cysts, and vascular malformations, a variety of tumors can occur in the buccal space .The most common tumor processes localized in this area arise from minor salivary glands such as pleomorphic adenoma (PA), adenoid cystic carcinoma, acinic cell carcinoma, and mucoepidermoid carcinoma. Other tumors can originate from connective, muscular, neural, and lymphatic tissue, such as rhabdomyosarcomas and neurofibromas .Metastatic lesions in this region are a very rare occurrence, accounting for less than 1% of oral cavity malignancies . They arWe report here the case of a female patient, with negative oncological history, who presented to our unit with a swelling of the left cheek, which revealed to be a melanoma metastasis only after surgical exeresis.We analyze our experience with a revision of the literature, aiming for a retrospective evaluation of the diagnostic imaging characteristics which might be useful in guiding the differential diagnosis, to set an example to avoid repeating the same error in the future and to be of help to those who will find themselves in such a situation.A 71-year-old woman presented with a painless visible swelling of the left cheek. Skin and oral mucosa were intact and of normal color. At palpation, the mass was mobile and apparently not adherent to the cutaneous and mucosal tissue.For what concerns comorbidities, the patient was only known for Hashimoto thyroiditis in hormone replacement treatment (no relevant comorbidity was known).Under ultrasound guidance, a 3-cm hypoechoic lesion was visualized in the left malar region and fine-needle-aspiration biopsy (FNAB) was performed. Cytological examination showed numerous Red Blood Cells, some lymphoid small B- and T-cells, plasma cells with no polyclonal atypia and a population of small-sized cells with plasmacytoid cells with the following immunophenotype: S-100 protein + , GFAP ± , P63 ± , cytokeratin7-, suggesting a salivary origin. However, a more precise diagnosis was not possible because of the small amount of material available.The following month, Magnetic Resonance Imaging (MRI) with intravenous paramagnetic contrast (Gadobutrol) was performed Fig. . T1-weigThe T1 hyperintensity was referred to possible blood residues in consideration of the recent bioptic procedure.Surgical exeresis was performed 2 weeks later. Intraoral access through the gingival fornix was obtained. The lesion, which extended to the pterygo-maxillary fissure, did not adhere to the surrounding tissues and was easily removed. It was partially solid, with a colliquated component.Histological examination showed the presence of fibro-connective and lymphatic tissue containing malignant neoplastic cells compatible with a nodule of melanoma inside the buccal fat pad Fig. .Fig. 3MiGene mutation analysis for c-KIT and BRAF was performed: c-KIT was Wild Type (WT), while V600 mutation of BRAF exon 15 was found.[F-18]FDG PET-CT Fig.  revealedFull-body computed tomography (CT) with intravenous contrast was then performed, with the following results: no encephalic lesions; 2-cm left cervical lymph node (LN); 5-mm left paracardiac micronodule; 7-cm LN conglomerate in the left axillary region; bilateral adrenal lesions ; multiple enlarged mesenteric LNs, the largest of about 2 cm; 2.8-cm lesion in the left psoas muscle.Dermatological examination revealed a 2-cm ulcerated cutaneous lesion on the distal phalanx of the left fifth finger, compatible with a primitive localization. The patient reported having it for about 1 year but never investigating it. Incisional biopsy of the lesion was performed, and histological examination confirmed the presence of melanoma cells with V600E mutation of BRAF gene.Immediately afterwards, treatment was begun with Dabrafenib 300 mg/die and Trametinib 2 mg/die.Two follow-up full-body CTs at 2 and 6 months were performed and showed significant size reduction of the axillary and abdominal LNs, of the adrenal and mesenteric localizations, and disappearance of the thoracic nodules.A variety of processes can occur in the buccal space, such as infections, developmental lesions, and tumoral processes.Neoplastic lesions in this area origin most commonly from minor salivary glands, such as pleomorphic adenoma, adenoid cystic carcinoma, acinic cell carcinoma, and mucoepidermoid carcinoma.The most frequent benign glandular tumor is PA, which consists of both mesodermal and glandular tissue . It has The most common malignant glandular tumor is adenoid cystic carcinoma. Some authors have suggested that lesions with low or intermediate T2-hyperintensity or invading surrounding tissues tend to be more aggressive, whereas lesions characterized by high signal intensity in T2w sequences have lower cellularity and better prognosis .Other tumors can originate from connective, muscular, neural, and lymphatic tissue, such as rhabdomyosarcomas and neurofibromas ; rhabdomUsually, in diffusion-weighted MRI, the ADC of malignant neoplasms of the head and neck regions is lower than that of benign tumors, and it is an important tool in guiding the diagnostic process .Metastatic malignant melanoma is an uncommon head and neck neoplasm. The most frequent localizations of melanoma metastases are lymph nodes (73.6%), lungs (71.3%), liver (58.3%), brain (49.1%), bone (48.6%), heart (47.2%), adrenal glands (46.8%), and gastrointestinal tract (43.5%) .Melanoma localized in the oral cavity and surrounding anatomical regions, such as the buccal fat pad, accounts for 0.2–8% of the total cases of melanomas of the body . HoweverTherefore, based on epidemiologic criteria, it is rather hard to diagnose metastatic melanoma in the buccal space in patients with an unknown primary tumor, as in our case.Yet, MRI may be helpful in the identification of melanoma metastases .Two main MRI patterns of melanoma can be identified: a melanocytic and amelanocytic pattern .Lesions showing a melanocytic pattern, which is the more common one, are characterized by signal hyperintensity in T1w and hypointensity in T2w images. This is due to the presence of melanin and blood products inside the lesion, although some authors argue that the T1 hyperintensity is more relevantly associated with the presence of blood products –23.Less commonly, malignant melanoma may show an amelanocytic (and therefore a specific) pattern, characterized by hypo- or isointensity in T1w images and hyper- or isointensity in T2w images , which iMacroscopically, metastatic malignant melanoma may present in different ways, ranging from small rapidly growing lesions which may initially go undetected or misrecognized as artifacts, to showing a military pattern .In our case, MRI showed a single lesion that exhibited diffusion restriction with ADC of 0.9 and high contrast medium uptake, characteristics suggestive of malignancy as it was proposed in the report.Moreover, the mass was characterized by T1 hyperintensity and T2 hypointensity, a behavior which may be attributed to the melanoma melanocytic pattern; however, it is not specific, since such features may be ascribed to other paraphysiological and pathological processes, including hemorrhages and proteinic fluid collections.In this case, radiologists supposed that there was a hemorrhagic component inside the lesion, possibly a residue of the FNAB which had been performed 1 month and a half before. Yet, this was a mistake. In fact, only early subacute hemorrhage dating 3–7 days is featured by T1 hyperintensity and T2 hypointensity, whereas chronic hemorrhages dating more than 1 month are rather characterized by both T1 and T2 hypointensity .Overlooking this aspect and ignoring a part of the anamnesis, radiologists assumed that they were dealing with a primitive buccal space neoplasm, namely with a malignant (minor) salivary gland tumor.On the contrary, this case teaches that T1 hyperintensity and T2 hypointensity, when associated with other malignancy features in buccal space lesion such as low ADC values and contrast enhancement, should always evoke the suspicion of melanoma, provided that no biopsy was performed and no trauma occurred in the 3–7 days before MRI examination.


Metastases from gastrointestinal and gynecologic malignancy may occur through lymphatic channels, through venules of the cancer or into the peritoneal space. The metastatic process involves lymphatic, hematogenous or transcoelomic dissemination.Two patients with splenic metastases are presented. Their clinical course is presented in an attempt to identify the route of cancer dissemination to the spleen. Lymphatic, hematogenous, or transcoelomic patterns of cancer dissemination are possible. Transcoelomic cancer dissemination into the peritoneal space results in peritoneal metastases.In a woman with ovarian cancer peritoneal metastases, a large lesion within the parenchyma of the spleen was described. This disease was present at multiple other sites as peritoneal metastases but no hematogenous or lymphatic metastases. A second patient with mucinous appendiceal neoplasm with peritoneal metastases was studied. Hematogenous metastases from this malignancy is extremely rare and lymphatic metastases were not present at the time of right colon resection. After studying these two patients, our hypothesis is that splenic metastases result from transcoelomic dissemination to the splenic hilum or splenic notches with progression of disease into the parenchyma of the spleen.When oncologists are considering aggressive local-regional treatments for peritoneal metastases important patient management decisions are influenced by the presence versus absence of hematogenous metastases. We suggest that the presence of splenic metastases does not indicate systemic (hematogenous) or lymphatic metastatic process but an extension of peritoneal metastases at the hilum of the spleen or within splenic notches deep into the parenchyma of the spleen. This complex series of events occurs more frequently in some organs than in others. Organs such as the liver have portal vascular systems with a reduced velocity of blood flow . Also, tA second possible route for cancer cells to move from gastrointestinal or ovarian primary cancer to the spleen is the lymphatic system. However, no lymphatic channels from the gut or ovaries to the spleen exist. It is difficult, even impossible, to suggest that the cancer cells that progress to splenic metastases reach the spleen through the lymphatics.A third route for cancer cells to follow to develop distant disease is through a body cavity. This type of spread occurs on pleural, pericardial or peritoneal surfaces. Peritoneal metastases frequently occur from gastrointestinal and gynecologic malignancy. The same sequence of cancer cell adherence, vascularization, and progression occurs with peritoneal metastases as with hematogenous metastases.In this case report two patients with extensive peritoneal metastases also developed splenic metastases. The clinical, radiologic and histopathologic characteristics of these two patients are studied in an attempt to determine if the route of cancer dissemination to splenic parenchyma is hematogenous or transcoelomic.2Cytoreductive surgery which requires peritonectomy procedures and visceral resections was performed on our 2 patients as previously described . As the www.researchregistry.com website with UIN 5552.Data on these patients were prospectively recorded and then retrospectively reviewed at an academic institution. This research work has been reported in line with the SCARE criteria . This st33.1▪In March 2019, the patient complained of abdominal pain. Pelvic sonogram showed a 6.0 × 4.6 × 3.6 cm left ovarian cyst and 4.9 × 3.6 × 3.9 cm complex cystic mass enlarging the right ovary. After completing some personal business out of the USA, a radiologic study was performed.▪In August 2019, CT of abdomen and pelvis showed soft tissue nodules up to 5 cm in diameter compatible with peritoneal metastases beneath the right hemidiaphragm. A 3.8 cm mass was completely intrasplenic . A lobul▪Biopsy of the abdominal wall mass revealed adenocarcinoma likely of gynecologic origin.▪Because of the large extent of disease and the presumed hematogenous metastases to the spleen, neoadjuvant chemotherapy with carboplatin and paclitaxel was recommended. Four cycles were planned. Pretreatment CA125 tumor marker was 1283 units/mL.▪In January 2020, preoperative evaluation revealed significant improvement of abdominal discomfort. CA-125 had decreased to 39.1 units/mL. Repeat CT scan showed reduction of tumor volume at all sites but the disease had persisted. The splenic metastasis had responded to the systemic chemotherapy but was still visible by CT .Fig. 2Th▪On January 13, 2020 at the abdominal exploration, the PCI was estimated to be 23. A debulking procedure was performed. Resections included tumor from the anterior abdominal wall, uterus with bilateral salpingo-oophorectomy, tumor masses anterior to the rectosigmoid colon, appendectomy and greater omentectomy. Tumor beneath the hemidiaphragms and the spleen were not removed. The resection was scored as a CC-3 cytoreduction.▪On January 17, 2020, the patient was discharged from the hospital without adverse events. Plan is additional carboplatin and paclitaxel chemotherapy.A 59-year-old gravida 3, para 3 woman was referred to the gynecologic oncology service because of a pelvic mass.3.2▪On June 1997, a 48-year-old male complained of right lower quadrant pain and fever. On an emergency basis he was taken to the operating theater where he was found to have a ruptured appendix. Not only was extensive inflammation present but there was mucin widely disseminated within both abdomen and pelvis. A right colon resection with anastomosis was performed. Pathology report showed a mucinous adenocarcinoma of the appendix. Four lymph nodes were negative for neoplasm. After recovery from surgery he underwent chemotherapy with 5-fluorouracil and leucovorin. When disease progression occurred, irinotecan was used as systemic chemotherapy.▪On May 9, 2000, a repeat CT scan showed tumor masses beneath the right hemidiaphragm. This was confirmed by biopsy of a perihepatic mass. Tumor masses were seen within the porta hepatis, within the omental bursa and greater omentum. A mass 7 cm in diameter is present within the old ileocolic anastomosis and causing partial small bowel obstruction. The left upper quadrant of the abdomen and spleen showed no evidence of mucinous adenocarcinoma. CA 19-9 tumor marker had increased to 527.8.▪On November 28, 2000, an 8 -h cytoreductive surgery was performed. At exploration the PCI was 17. Extensive peritonectomy of the right upper quadrant and pelvis were performed along with greater and lesser omentectomy. HIPEC with mitomycin C and EPIC with 5-fluorouracil were performed in the operating theater and for the first 5 postoperative days. The patient was discharged without adverse events.▪In June 2004, CT performed on routine follow-up showed a mass enlarging within the spleen. CA 19-9 had reached 2010 on November 23, 2004. CEA was 9.▪On November 23, 2004, repeat CT showed a 7 cm in diameter cystic mass within the central part of the spleen . No othe▪On November 24, 2004, exploratory laparotomy was performed. The mucinous adenocarcinoma was contained within the splenic capsule. The spleen with a portion of the left hemidiaphragm was removed intact. No HIPEC or EPIC was used.▪On March 8, 2013, the patient died but cause of death was unknown.44.1Although splenic metastases are uncommon, there are numerous reports in the oncology literature of their occurrence. If splenic metastases have a hematogenous route of dissemination they should be reported for cancers that have their primary site outside of the abdomen and pelvis. They should occur, at least in some patients, as multiple sites of disease within the splenic parenchyma. They should be associated with other sites of hematogenous metastases such as lung, liver and bone marrow. Finally, they should occur in the absence of peritoneal metastases. Neither of our two patients had these associated clinical features for a hematogenous origin of splenic metastases.If splenic metastases have a transcoelomic route, the primary site should be limited to the abdomen and pelvis . They should occur as a single intraparenchymal splenic mass or only a few masses because access of cancer cells through the splenic capsule does not frequently occur. Also, since the deepest crypts in which cancer cells may become entrapped are at the hilum, a preponderance of the splenic metastases would be near the hilum of the spleen. The splenic masses from invasive implants should not be associated with systemic metastases to liver, bone marrow or lung. They should be associated with other sites of peritoneal metastases. An exception to this criteria would be patients, as our patient 2, who had cytoreductive surgery and HIPEC and EPIC to eradicate peritoneal metastases at a prior time. In the ovarian cancer patient and mucinous adenocarcinoma of the appendix patient presented, transcoelomic peritoneal metastases would be selected as the route for cancer cells to reach the splenic parenchyma.4.2It is important to establish if metastases are disseminated by a hematogenous route or if they are transcoelomic peritoneal metastases. Judgments regarding the extent of a cytoreductive surgery often depend on the surgeon’s confidence that the tumor masses being removed by cytoreductive surgery are limited to the peritoneal surface rather than of systemic disease. A local-regional treatment such as cytoreductive surgery is unlikely to be of long-term benefit with hematogenous dissemination of disease. If splenic metastases are from a hematogenous source, the resection of the spleen during cytoreductive surgery would not be indicated. However, if splenic metastases are a manifestation of splenic invasion by peritoneal metastases, inclusion of the spleen and the disease within the organs resected would be completely reasonable. This is why it is important to establish the origin of the splenic metastases in performing surgery for peritoneal metastases, especially ovarian malignancy.4.3Hematogenous metastases do occur in patients with ovarian cancer but were not seen by CT in the patient reported here. However, a hematogenous route for splenic metastases with ovarian cancer may be possible. However, splenic metastases have been reported frequently with pseudomyxoma peritonei of either ovarian or appendiceal origin . Pseudom4.4Another reason to classify splenic metastases in serous ovarian cancer as transcoelomic rather than hematogenous concerns the mechanism for metastatic disease to the spleen. Having cancer cells enter the spleen from the systemic blood circulation through the splenic artery could occur. However, if it did occur, one would expect metastases also within the parenchyma of the liver and at other systemic sites. In the patient reported here, the only site identified by CT for possible hematogenous dissemination of serous ovarian cancer was the spleen. All of this patient’s disease at other sites was limited to peritoneal surfaces. More than likely these splenic metastases were from a peritoneal spread of the disease that becomes invasive into the splenic parenchyma.4.5If the splenic metastases are in reality an invasion of the parenchyma of the spleen by peritoneal implants, how do the cancer cells gain access? One hypothesis is that the cancer cells enter the splenic parenchyma, either at the hilum or through the splenic notches. In fetal life, the spleen is a multilobular structure. These lobules coalesce in fetal life to form in the human a single structure. However, frequently the fusion of the splenic lobules is not perfect and the “splenic notches” will persist . Cancer In summary, we postulate two groups of patients who develop splenic metastases. One group has a large burden of hematogenous metastases to liver, lung and bone marrow. High levels of circulating cancer cells may be expected to enter the splenic parenchyma via the splenic artery and progress to tumor masses demonstrated by CT. These patients are not candidates for splenectomy.The second group of patients, like our two cases, have peritoneal metastases and no evidence by CT of hematogenous cancer dissemination. Splenic tumor masses within the spleen are accompanied by other sites of transcoelomic cancer dissemination. These patients should be considered for splenectomy as part of an attempt to achieve complete cytoreductive surgery. In this regard, the concept of splenic metastases as an unusual manifestation of peritoneal metastases has important implications in the optimal surgical management of gastrointestinal and gynecologic cancers with peritoneal dissemination.Paul H. Sugarbaker has no conflicts of interest to declare.Ebony R. Hoskins has no conflicts of interest to declare.Data management and secretarial support provided by Foundation for Applied Research in Gastrointestinal Oncology.Local IRB-approval for this case report was not required:MedStar Health Institutional Review Board has determined that a case report of less than three  patientsThis case report is of 2 patients.Informed consent was requested with one patient and through surviving family members of another for publication of this case report.Paul H. Sugarbaker, MD: study concept or design, data collection, data analysis or interpretation, writing the paper.Ebony R. Hoskins, MD: study concept or design, data collection, data analysis or interpretation.www.researchregistry.com website with UIN 5552.This study was registered as a case report on the Paul H. Sugarbaker, MD.Not commissioned, externally peer-reviewed.


Splenic subcapsular hematoma is a rare complication of pancreatitis. The splenic vessels and the pancreatic tail lie close together in the lienorenal ligament. The pathologies in the pancreatic tail may occasionally affect the spleen resulting in splenic vein thrombosis, arterial pseudoaneurysm, subcapsular splenic hematoma, and splenic rupture. A 40-year-old male with a history of alcohol abuse and alcohol-induced pancreatitis presented with severe epigastric abdominal pain and was diagnosed with pancreatitis. Later during hospitalization he became dyspneic and hemodynamically unstable, with acute anemia requiring blood transfusion. An abdominal CT with angiography (CTA) revealed a splenic subcapsular hematoma with active bleeding which was managed by urgent exploratory laparotomy and splenectomy. Due to its rarity, diagnosis of splenic hematoma in pancreatitis is challenging with rapid identification and intervention being key to management. Pancreatitis and its complications remain a leading cause of healthcare utilization in the United States . SplenicA 40-year-old male patient with a past medical history of alcohol abuse and alcohol-induced pancreatitis presented to the ED with severe epigastric abdominal pain for two days. The pain was described as severe, throbbing in nature, continuous, and radiating from the epigastrium to the left hypochondrium. It was associated with persistent nausea, without any vomiting and loss of appetite. There was no history of hematemesis or melena. No fever or chills were reported. The patient had been discharged a week prior, after being hospitalized for 10 days due to an attack of acute pancreatitis brought on by excessive alcohol consumption. Following discharge, the patient had resumed his consumption of alcohol daily. No abdominal trauma was reported.On evaluation, his vital signs were noted to be stable. He was afebrile, oxygen saturations at 98% on room air, respiratory rate was 18 breaths per minute, pulse was 88 beats per minute, and blood pressure was 138/75 mmHg. The patient appeared very uncomfortable and was stooping forward while sitting. Abdominal examination revealed no distension, but marked tenderness with guarding was elicited at the epigastrium and left hypochondrium, without any rebound tenderness and bowel sounds were hypoactive. Laboratory investigations showed elevated white blood cells , lipase , amylase , and alkaline phosphatase . Also, hemoglobin was 138 g/L with a hematocrit of 40.2%, with normal total bilirubin, aspartate aminotransferase (AST), and alanine aminotransferase (ALT) at 0.4 mg/dL, 18 IU/L, and 22 IU/L respectively. Chest X-ray revealed normal cardio-pulmonary study. Only a week prior, his abdominal CT had revealed two pseudocysts in the pancreatic tail, along with evidence of acute pancreatitis, hence it was not repeated , but no overt signs of bleeding were found. This raised the concern for a concealed hemorrhage, possibly an intra-abdominal hemorrhage. Packed red blood cells were transfused and an urgent surgical consultation was called. A stat repeat abdominal CT with angiography (CTA) now revealed a large splenic subcapsular hematoma measuring 17.3 cm x 12.0 cm x 12.5 cm, communicating with a pseudocyst in the pancreatic tail, with areas of attenuation suggesting recent hemorrhage , often with a drop in hemoglobin , 6. Due Management of this rare condition is controversial. Hemodynamically stable patients may often be managed conservatively, or with procedures such as splenic artery embolization or ultrasound-guided percutaneous drainage -5, 7-9. Due to its rarity, the diagnosis of splenic hematoma in pancreatitis is challenging and requires a high index of suspicion. Rapid identification and intervention are critical to ensure an optimal outcome, with hemodynamic stability often deciding the mode of management. As the incidence of pancreatitis continues to rise, physicians should be aware of the complications for timely and effective management.


Football sign is a rare radiographic finding on abdominal x-ray that suggests massive pneumoperitoneum. Free air outlines the abdominal cavity and falciform ligament which creates the radiolucent oval contour of a football. Football sign is hardly reported in older children or adults. We present the first clear image of football sign caused by gastric perforation in an adult patient. A 57-year-old male with a history of hepatocellular carcinoma was diagnosed with an undrainable liver abscess and partial gastric outlet obstruction. He developed acute onset of severe abdominal pain afterward and abdominal plain film showed a large oval radiolucency over the central part of the abdomen without interruption by intestine, a classical finding of pneumoperitoneum also known as a “football sign”. Emergent laparotomy revealed a 0.5 cm perforation hole at the anterior surface of the gastric antrum. Despite timely intervention, the patient died from progression of multiorgan failure. This case describes an alarming radiographic finding that rarely occurs in the adult population. Air could be identified on x-ray in this patient due to presence of massive ascites in his abdominal cavity. Recognizing radiographic patterns that suggest pneumoperitoneum on supine plain radiographs could expedite the diagnostic process and surgical intervention. Non-traumatic gastrointestinal (GI) preformation is a life-threatening condition that requires timely diagnosis and intervention . PresencA 57-year-old man with a history of hepatocellular carcinoma first presented to the emergency department with fever for 10 days. Abdominal computed tomography (CT) revealed a distended stomach, a subphrenic liver tumor, and a fluid collection with free air at the perihilar area of the liver, indicative of tumor necrosis with abscess formation. Esophagogastroduodenoscopy demonstrated gastric ulcers and a protruding mass at the antrum of the stomach causing gastric outlet obstruction. A diagnosis of liver tumor necrosis with abscess formation and partial gastric outlet obstruction was made. Since the abscess was located adjacent to the hilar vessels, drainage of the abscess was not attempted. Fever and abdominal pain improved after five days of flomoxef, and he was discharged with oral antibiotics.Ten days after discharge, he started to have recurrent fever with worsening abdominal distension and hypotension. Physical examination upon admission revealed altered mental status and diffuse abdominal tenderness with muscle guarding. Laboratory data was notable for elevated white blood cell counts (WBC) of 52,200 /μL with 97.2% of neutrophils, alanine transaminase of 585 U/L, aspartate transaminase of 764 U/L, direct bilirubin of 2.4 mg/dL, and alkaline phosphatase of 600 IU/L. Abdominal x-ray revealed large oval radiolucency over the central part of the abdomen without interruption by intestine, a classical finding of pneumoperitoneum also known as “football sign” Figure . An enhaEnterococcus faecium, Candida glabrata, and Candida albicans. Despite timely management, the patient passed away on hospital day 23 due to progression of multiorgan failure.Emergent laparotomy showed a 0.5 cm perforation at the anterior surface of the gastric antrum Figure . GastrorSince the 1900s, plain film has become a valuable aid in diagnosing GI perforation. Although CT has been widely used to detect GI perforation due to its superior diagnostic accuracy . Supine Football sign was first described in 1960 by Miller et al. as one of radiographic patterns of perforated viscus in neonates . It occuWe presented a rare case of an adult patient who had gastric perforation and presented with a football sign on supine plain film. Recognition of the football sign can aid diagnosis and management of pneumoperitoneum in adult patients.


Liposarcomas are considered to be the most common soft tissue sarcomas and have five histological subtypes. The myxoid subtype often occurs in the lower limbs and the retroperitoneum; however, the abdominal wall location is extremely rare. The clinical presentation and radiological findings are non-specific. Wide local excision with a minimum margin of 3 cm remains the mainstay of treatment to prevent local recurrences. We herein report a rare location of myxoid liposarcoma in the abdominal wall. Liposarcomas (LPSs) represent the most common soft tissue sarcomas . Five hiA 43-year-old man presented with an abdominal wall mass of nine months duration, increasing rapidly in size without any other associated symptoms. On physical examination, the mass was located in the left abdominal wall. It was soft and mobile, and had a diameter of 9 cm. An abdominal MRI with intravenous contrast revealed a heterogeneous mass in the left rectus abdominis muscle tumor lesion enhanced early and continuously in the late phase, measuring 88 x 65 x 52 mm Figure . A thoraBiopsy with Tru-Cut®  of the mass was consistent with an angioleiomyoma. Immunohistochemistry staining was performed, which revealed the following: CD34 (-), vimentin (-), myogenin (-), and S100 (-). We decided to perform a large excision of the parietal tumor with clear surgical margins followed by the repair of the fascial wound using a nonabsorbable mesh Figure .The mass was roundish, measuring 9 cm. The gross section showed a yellowish myxoid tumor well-circumscribed capsulated with the following resection margins: 0.2 cm for the deep limit, 0.5 cm for the internal limit, 0.3 cm for the external limit, 0.4 cm for the upper limit, and 0.3 cm for the lower limit.The histological examination and immunohistochemistry stainings  confirmed the diagnosis of myxoid LPS  and rarely the retroperitoneum .MDM2 and CDK4 genes [Characteristic genetic alteration for myxoid/round-cell LPS is classically a t or t translocation found in more than 95% of myxoid/round-cell LPS, whereas well-differentiated/dedifferentiated LPS is related to the amplification of the 12q13-15 region that comprises the In our case, the mass was thought to be benign pre-operatively; therefore; an accurate diagnosis was needed. The suspicious abdominal masses should undergo a biopsy prior to surgical resection in all cases. The biopsy usually has a sensitivity of 80 to 95% to define the presence or absence of malignancy . The mosThe management of myxoid LPS needs to be discussed over multidisciplinary meetings including surgeons, oncologists, and radiotherapists.The gold standard treatment of abdominal wall sarcoma consists of the en bloc resection of the lesion including 3 cm as a minimum margin of free tissue, involving the entire width of the abdominal wall. If there is tumor adherence or invasion, this resection must also involve adjacent structures or internal organs ,9. AbdomWell-known negative prognostic factors for abdominal wall LPS are age (>45 years), tumor size (>10 cm), high histological grade, and a high percentage of round cells and positive resection margins. On the other side, large and extensive surgical removal has a significant effect on the survival rate ,13. OverMyxoid LPS of the anterior abdominal wall is an extremely rare tumor. The suspicious abdominal masses should undergo a biopsy prior to surgical resection in all cases. Wide local excision of the tumor remains the mainstay of treatment with a minimum margin of 3 cm in light of their tendency to occur locally.


Nasal septal schwannoma is a rare tumor. It causes complaints such as nasal congestion, nosebleeds, and headaches. There are many diseases such as nasal polyps, antrochoanal polyp, chronic rhinosinusitis, concha bullosa, inverted papilloma, and retention cyst with schwannoma diagnosis. The diagnosis is made histopathologically, and the treatment is surgery. In this case report, we presented a male patient with septal schwannoma who had nasal obstruction for a year and reviewed the last 20 years of literature on nasal schwannoma. Schwannomas are benign tumors of the nerve sheath commonly associated with cranial nerve VIII. It is observed with a frequency of 25–45% among all head and neck tumors –4. Only A 28-year-old male patient was admitted to our clinic with progressive nasal congestion and recurrent epistaxis. In rigid endoscopy, there was a polypoid mass originating from the septum with smooth surface and prone to bleeding. It was causing nasal obstruction on the left side of the nose . In parath and 6th decades, and there is no gender dominance [Schwannoma is a class sheath tumor often seen in the head and neck region. While approximately 25–45% of schwannomas are seen in the head and neck region –4, only ominance . Nasal sominance . Paranasominance . In prevominance –14.The definitive diagnosis of schwannoma tumor is made histopathologically. Schwannoma tumors are macroscopically seen as bordered and encapsulated. When stained with hemotoxylin-eosin stain and analyzed microscopically, it is divided into two patterns, Antoni A and Antoni B. Antoni A consists of spindle cells that form palisade by lining the nuclei side by side. Antoni B consists of loose myxoid stroma and a small amount of spindle cells. It is stained strongly with S-100 immunohistochemically , 15, 16.


Masson’s tumor or IPEH represents a rare exuberant endothelial proliferation within a thrombus through an uncomprehended phenomenon. Being reported for the 1st time in Saudi Arabia, plastic surgeons should keep it in the list of differential diagnosis.We report a case of 17-year-old-girl who presented with a 6-month-old, painful mass on the volar side of her left 4th MCP. Radiology was inconclusive. Histopathology reported Masson’s tumor following surgical excision with good functional outcome and no recurrence.Comprising 2%–4% of overall skin vascular tumor and with no identifying clinical or radiological feature, IPEH poses as a diagnostic challenge. The literature reports similar tumors in the hand with different locations and presentations. Surgical excision remains the cornerstone of management, yet the role of radiotherapy remains undefined. Incomplete excision may result in recurrence, which requires a consensus on the extend of marginal excision. Rare cases of recurrence were reported. Histopathology is the only reliable method of diagnosis.We present the 1st reported case of IPEH involving the hand in Saudi Arabia. Plastic surgeons should be aware of such rare conditions and be able to differentiate them from relatively identical yet more sinister tumors, especially angiosarcoma. Despite showing a clear predilection for the head, neck, and extremities, Masson’s tumor has been recently reported in few other peculiar locations including the eyelid, parotid gland, sinonasal cavity, and kidneys, with no pathognomonic features . Given t2A 17-year-old lady, not known to have any medical illnesses, presented to the plastic surgery clinic in our institute with a painful, progressively growing mass on the left middle finger. Six months prior to presentation, it has started as a painless, green mass, after which she was referred to us. Physical examination revealed a dark red swelling on the palmar side of the left 4th MCP with preserved range of motion and sensation. Capillary refill or sensation were not compromised. Magnetic resonance imaging (MRI) with gadolinium showed a subcutaneous rounded soft tissue lesion with internal high T2 and T1 signal intensity. Local excision was performed under general anesthesia. Grossly measuring 1 × 1 × 0.5 cm, the lesion was well-circumscribed, brown-black in color, and rubbery in consistency. Pathology report indicated an organizing thrombus with papillary endothelial hyperplasia or Masson’s tumor. With no peri-operative or post-operative complications, no recurrence was noticed 1 year later during a follow-up visit , Fig. 3.3First documented in 1923, Pierre Masson, a French pathologist, described a lesion in the lumen of an ulcerated hemorrhoidal vein and labeled it "hémangio-endothéliome végétant intravasculaire”, or better known today as Masson’s tumor or IPEH ,5. It isThe diagnosis of Masson’s tumor is challenging owing to its clinical and radiological resemblance to other vascular tumors, especially angiosarcoma See . Masson’Ultrasound can detect one or more vessels associated with the lesion, differentiating IPEHs from other soft tissue masses . MRI demTreatment is mainly through surgical resection, which can be curative and result in excellent outcome and prognosis with slim chances of recurrence, especially in type II. As of yet, there is no consensus regarding the margins of resection as generally there is no indication for wide margins of excision; however, incomplete resection may result in recurrence . Occasio4Masson’s tumor or (IPEH), is being encountered by the plastic surgeons more and more, albeit rarity. We narrate a case of a 17-year-old medically free girl with a mass in the hand, which was found to be Masson’s tumor after resection and histopathology analysis. No cardinal features can be pin-pointed at, in both clinical examination or radiology, that lead to IPEH. Consequently, it may not take a place in the differential diagnosis list of the treating surgeon.None.None.Exempted from the IRB approval.Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.1.Mohammed Almarghoub: Manuscript writing and editing.2.Qutaiba Shah Mardan: Manuscript writing and editing.3.Ahmed Alotaibi: Manuscript writing and editing.4.Norhan Ahmed: Manuscript writing and editing.5.Moraya Alqahtani; Supervision; Treatment of the patient and manuscript editing.This paper does not require registry as it is a case report about a condition that has been discussed in prior papers. There is no additional harm to the patient nor an innovative intervention is being applied on the patient.Mohammed Almarghoub.Qutaiba Shah Mardan.Not commissioned, externally peer-reviewed.


Abdominal pain can arise from numerous sources, including those extra‐abdominal. It is important to obtain additional imaging in the setting of clinical suspicion for malignancy. Abdominal pain can arise from numerous sources, including those extra‐abdominal. It is important to obtain additional imaging in the setting of clinical suspicion for malignancy. This case describes an atypical presentation of sarcoma manifesting as abdominal pain, which previously has not been found in literature. We highlight the importance of pursuing additional workup when clinical suspicion for malignancy arises.This case illustrates a diagnosis of synovial sarcoma of the thigh presenting as generalized abdominal pain. Synovial sarcoma is a malignant mesenchymal neoplasm that makes up 5‐10 percent of soft tissue sarcomas, and it tends to affect young adults.Most cases of sarcoma present as localized pain and bulge at the affected site. No previous cases have described sarcoma manifesting as abdominal pain. This case integrates anatomy of abdominal and quadricep muscles with the understanding of abdominal pain. Specifically, we hypothesize that this patient's abdominal pain arises from the external compression of abdominal muscle insertion sites, along with nerve irritation causing referred pain along the abdominal sheath. This case demonstrates the necessity of obtaining additional imaging in the setting of weight loss and early satiety, especially when workup is initially unrevealing.2A 47‐year‐old male triathlon athlete presents to primary care clinic with 2 months of abdominal discomfort that evolved from an epigastric to a generalized abdominal pain. It is associated with nausea, decreased oral intake, early satiety, odynophagia, and unintended weight loss of 20 pounds over 2 months. The pain is worse with meals and did not improve with the use of H2 blockers. He did not have unexplained fever, respiratory symptoms, or chest pain. He also had no changes in bowel movement, melena, or hematochezia. Review of systems was unremarkable other than feeling like he pulled a muscle in this right thigh a few months ago as he was training for a triathlon, for which he takes ibuprofen. His right hip and thigh area gradually grew more sore, contributing to performance decline. On examination, he had abdominal tenderness to palpation in all quadrants without organomegaly, guarding, rebound, or referred pain.3Initial labs with CBC, CMP, and lipase were within normal limits. Patient underwent an upper endoscopy after initial presentation of abdominal pain associated with food intake and weight loss due to concern for upper GI malignancy. The EGD was negative.In the setting of progressive symptoms, imaging with CT abdomen and pelvis was ordered. It showed a large soft tissue mass measuring 7.3 × 8.3 cm in the right anterior quadriceps femoris muscle, with mass effect upon the vastus lateralis, vastus medialis, and rectus femoris muscles Figures  and 2.Pathology from the mass biopsy revealed high‐grade monomorphic synovial sarcoma, spindle cell type. The synovial sarcoma had a SS18 gene locus rearrangement present.4There is an extremely wide range of possible causes of generalized abdominal pain, such as constipation, gastritis, gastroenteritis, irritable bowel syndrome, inflammatory bowel disease, malignancy, mesenteric ischemia, and more. However, this patient's unintentional 20 lb weight loss raised concern for malignancy. Basic labs and lipase were ordered to evaluate for pancreatitis. Upper endoscopy was ordered to evaluate intra‐abdominal causes of pain and weight loss, such as gastric ulcers or gastric malignancy. With unrevealing workup thus far yet ongoing symptoms, we next investigated with a CT abdomen and pelvis. Fortunately, the incidental CT finding of thigh mass helped expedite the workup that led to the patient's diagnosis of synovial sarcoma.5He was treated with four cycles of neoadjuvant chemotherapy with ifosfamide and mesna, as well as neoadjuvant radiation therapy. He underwent surgery of the thigh mass 3 weeks after completion of neoadjuvant treatment. He continued on adjuvant chemotherapy with adriamycin and ifosfamide and has completed six cycles.6The patient tolerated the chemotherapy and surgery well and had resolution of his abdominal pain after mass resection. Surgery revealed clean margins and no involvement of regional lymph nodes. CT chest revealed no pulmonary metastasis, making his stage T3N0M0. He has completed adjuvant chemotherapy and continues to participate in a physical therapy rehabilitation program. Unfortunately, in July 2020, he developed a new 1.3 cm pulmonary nodule in the left upper lobe concerning for metastatic disease.7There are numerous intra‐abdominal and extra‐abdominal pathologies that could manifest as abdominal pain. Extra‐abdominal causes of abdominal pain include pyelonephritis, ureterolithiasis, inferior wall cardiac ischemia, lower lobe pneumonia, tuberculosis, thyrotoxicosis, portal vein thrombosis, porphyria, and lymphoma.Synovial sarcoma is a high‐grade soft tissue sarcoma that comprises 5 to 10 percent of all soft‐tissue sarcomas. It is commonly diagnosed during the early decades of life, though some cases have also occurred in adults in their eighth and ninth decades.Radiologically, synovial sarcomas tend to be large, lobulated, and frequently close to joints. For initial staging, magnetic resonance imaging is the imaging of choice due to its intrinsic signal characteristics and its superior ability to assess for tumor invasion.There are two major histological subtypes of synovial sarcoma, biphasic and monophasic spindle cell types. There are also rarer subtypes including monophasic epithelial, poorly differentiated, calcifying and myxoid types. Histological features associated with a poorer outcome include mitotic indices of greater than 10 mitotic figures/10 high power fields, high nuclear grade, presence of rhabdoid cells, poor differentiation, and the presence of extensive tumor necrosis. On the other hand, extensively calcified synovial sarcoma is associated with a more favorable prognosis of 80% 5‐year survival.P = .0111]). Higher histological grades have also been associated with worse prognosis, with grade 3 tumors having a 42.8% OS at 10 years, compared to 49.5% for grade 2 (P = .083).The patient described in our case had high‐grade, monomorphic synovial sarcoma, which signifies a relatively poor prognosis. Monomorphic synovial sarcoma has shown to have significantly worse overall survival (OS) than biphasic synovial sarcoma (42.3 vs. 59.7% 10‐year OS [Treatment for nonmetastatic sarcoma usually includes wide surgical excision, followed by radiation and sometimes adjuvant chemotherapy. Synovial sarcoma has shown to be more sensitive to chemotherapy compared to other adult soft tissue sarcomas, especially to alkylating agents. Therefore, first‐line chemotherapy includes ifosfamide and doxorubicin. It is estimated that 50% of patients with synovial sarcoma attain partial or complete remission with regimens containing ifosfamide and doxorubicin. Due to the rapid spread of disease and late‐stage diagnosis in the majority of cases, prognosis is poor. However, early recognition and diagnosis can significantly improve outcomes. Typically, 5‐ and 10‐year survival rates are about 60% and 50%, respectively. Metastatic disease occurs in about half of the patients, more commonly among adult patients. Common sites of metastasis include the lungs and pleura, bones, and lymph nodes.None declared.KP, NW, JS, and ZZ: designed the study, acquired and analyzed the data, wrote and revised the manuscript, gave final approval of the manuscript, and agreed to be accountable for all aspects of work.


Pial arteriovenous fistula (PAVF) is a rare intracranial vascular disease, and its presentation with a huge tumor-resembling thrombus is rarer.A 38-year-old female patient presented with a sudden left-side motor disorder and loss of consciousness. The patient was otherwise in good health and had no history of hypertension or diabetes. During the physical examination, she appeared lethargic and manifested left limb paralysis with level zero muscle strength and a positive pathological reflex.Because imaging failed to rule out a tumor stroke, an intracranial lesion resection was performed immediately. Because the lesion was considered to be a vascular structure, digital subtraction angiography was undertaken before the surgery, and PAVF was diagnosed.Endovascular embolization was conducted, followed by PAVF and hematoma resection.At the 3-month follow up, her left limb muscle strength was level 4, and she could live on her own .It is noteworthy that PAVF with a large thrombus may appear as a tumor in the initial diagnosis, and therefore it is necessary to perform an intracranial vascular examination in patients with tumor stroke symptoms. In most cases, hemorrhage can manifest as an intracranial hematoma or subarachnoid hemorrhage.,4It can cause a series of neurological symptoms such as headaches, hemorrhage, seizures, focal neurologic deficits, and raised intracranial pressure.In this report, we present a rare case of PAVF with a large thrombus, which caused a mass effect and appeared as a hemorrhagic tumor in the imaging results. A literature review of similar reports was also performed.2A 38-year-old female patient presented with a sudden left-side motor disorder and loss of consciousness. The patient was otherwise in good health and had no history of hypertension or diabetes. During the physical examination, she appeared lethargic and manifested left limb paralysis with level zero muscle strength and a positive pathological reflex.Head computed tomography findings showed lesions with hemorrhage occupying the right temporal lobe space, compression of the ipsilateral ventricle, and the midline structure offset to the contralateral side Fig. A,B. FurtIntracranial lesion resection was undertaken because the patient was diagnosed with a right temporal lobe tumor with hematoma. The operation was performed under general anesthesia using a right temporal operative approach. A reddish cystic mass was observed on the surface of the brain the dura was opened. It felt tough and had a large number of nourishing arteries on its surface. Because the lesion was considered to have a vascular structure, we immediately closed the skull and transferred the patient to the catheter room for digital subtraction angiography. The angiography showed that the lesion was a PAVF fed by the right middle cerebral artery. Blood flow was through the lateral fissure vein and Labbé vein to the sigmoid sinus Fig. .The PAVF was completely embolized with endovascular treatment using coils and onyx Fig. . SubsequPostoperative pathological results showed attachment of several clots to the intima of the lesion's vessel wall and accumulation of hemosiderin-phagocytosing tissue cells. The medial membrane manifested hyaline and mucoid degeneration, and focal calcification. The extravascular membrane was scattered with chronic inflammatory cells. All these features were consistent with the pathological changes of intravascular thrombosis Fig. C,D.The patient gradually recovered postoperatively. After 1 week, the patient was discharged, and her left limb muscle strength was level 2. The patient was transferred to another hospital for further rehabilitation training. At the 3-month follow up, her left limb muscle strength was level 4 and she could live on her own .3Our institution does not require formal ethical approval for case reports. Written informed consent was obtained from the patient and her parents for publication of this case report.4 The most important anatomical feature is the direct connection between the intracranial arterial system and the venous system of the Willis ring or its secondary branch vessels.,5 PAVF can cause headaches, intracranial hemorrhages, seizures, and focal neurological deficits; hemorrhages generally manifest as intracranial hematoma or subarachnoid hemorrhage.,4 In this case, the patient had a ruptured PAVF with a large thrombus. It caused a mass effect which made it appear as a hemorrhagic tumor in the imaging results, a phenomenon rarely reported.PAVF is a rare vascular abnormality that accounts for about 1.6% of all intracranial vascular malformations, and is most common in children. Most pediatric patients are considered congenital, and multiple PAVFs may be associated with hereditary hemorrhagic telangiectasia. This patient was a middle-aged woman without any history of trauma or surgery and had no clinical before the onset of the disease. Congenital cause of formation of PAVF could not be excluded.Pial AVF can be congenital, iatrogenic, or caused by trauma, and it enables direct communication between arteries and veins.,8The occurrence of thrombus in PAVF is rare, and its cause is still unclear; induction mechanism similar to the thrombosis in giant aneurysms is considered a leading possibility. Slow blood flow and turbulence in the PAVF may lead to endothelial cell dysfunction, resulting in contact between the matrix proteins and blood flow, subsequent activation of the coagulation cascade and eventual thrombus growth. The thrombus can further lead to cell lysis and release of thrombin, thereby contributing to thrombus growth again.,10 This is consistent with the abundant distribution of blood vessels observed on the surface of the lesion in our case. Therefore, the existence of nourishing arteries may also be responsible for the continued growth of the thrombus. Furthermore, pathological studies have already demonstrated that inflammatory responses play a key role in thrombosis of giant aneurysms and arteriovenous malformations.–13 In this case, inflammatory cell infiltration was also observed in the vessel wall. It is speculated that inflammation may also be the cause of giant thrombus formation in PAVF.In addition, the wall of a giant aneurysm contains nourishing vessels; thus, the intracapsular thrombus can continue to grow even after the aneurysm is embolized. It is not difficult to diagnose PAVF alone. Most patients with intracranial hemorrhage will have a computed tomography angiography or digital subtraction angiography examination to determine the cause of bleeding. However, in this case, there was a large thrombus in the PAVF, which had a mass effect and resembled as a hemorrhagic tumor, thereby causing an easy misdiagnosis.PAVF is characterized by high disability and mortality rates, therefore, surgical intervention should be performed actively, especially in cases with bleeding symptoms. To reduce the probability of recurrence due to the existence of nourishing vessels, PAVF should be removed during microsurgery, especially in patients with mass effect. PAVF's embolization before microsurgery is more conducive to its removal; therefore, it was not difficult to remove the lesion and thrombus.In most cases, microsurgery or endovascular embolization is a simple and effective way to disconnect the abnormal connections between the PAVF arterial system and the venous system.It is noteworthy that PAVF with a large thrombus may appear as a tumor in the initial diagnosis, and therefore it is necessary to perform an intracranial vascular examination in patients with appearance of hemorrhagic tumor.Conceptualization: Yuhao Zhao, Xuan Chen, Jinlu Yu.Data curation: Ying Yu, Weikai Yao, Haiyang Xu.Project administration: Yuhao Zhao.Supervision: Xuan Chen.Writing – original draft: Yuhao Zhao, Yinmeng Qu.Writing – review & editing: Yinmeng Qu, Xuan Chen, Jinlu Yu.


Ameloblastoma is a locally aggressive tumor that most commonly arises in the mandible. It has a high rate of recurrence if inadequately excised. We report a case of a patient who developed recurrence of his ameloblastoma in his fibula flap mandibular reconstruction despite clear resection margins 23 years after resection. This is the first reported case of recurrent ameloblastoma in a neo-mandible reconstruction in the setting of negative margins. We discuss its surgical management using digital planning and reconstruction using a contralateral free fibula flap. Ameloblastoma is a locally aggressive entity that requires complete excision. Recurrence can even occur in the reconstruction, which can present a challenge to manage. Consideration should be given to repeat excision and second osseous flap reconstruction. Ameloblastomas are the most common odontogenic tumor of the jaws, with 80% of tumors occurring in the mandible . It is aWhile conservative surgical management of ameloblastomas  is associated with very high recurrence rates, the recurrence rate associated with en bloc resection is rare -5. To miThere are sporadic reports in the international literature of recurrence despite en bloc resection but the status of the resection margins is normally unclear. We report a case of a patient who developed recurrence of his ameloblastoma in his fibula flap mandibular reconstruction despite clear resection margins and discuss its reconstruction.A 59-year-old man was referred to the Head and Neck Unit with swelling of the mucosa of his left mandible and exfoliation of his dental implants. He had a history of previous segmental resection of the left mandible and free fibula flap reconstruction from the right lower leg for an ameloblastoma 23 years previously. Histological examination confirmed complete excision with negative surgical margins. The patient subsequently proceeded to implant insertion for dental rehabilitation Figure .At re-presentation, he was investigated with plain film X-ray and CT imaging of his mandible Figure .The imaging indicated recurrence within his fibula flap neo-mandible. The mandibular mass was biopsied and histology confirmed tumor recurrence. After digital surgical planning, the patient proceeded to radical resection of the recurrent tumor with wide resection margins, but sparing the right mental nerve to preserve sensation of the contralateral lip and chin.Figure En bloc resection of ameloblastomas is the gold standard, particularly where there has been failure of more conservative surgical approaches. Because they can reach a large size before becoming symptomatic, en bloc resection normally involves segmental resection of the mandible. While this is associated with significant morbidity, patients can normally be reassured that recurrence of their tumor is exceedingly rare. There are sporadic reports in the international literature of recurrence despite segmental resection . AmeloblRecurrence can occur in the native remaining mandible, as well as in the mode of reconstruction. Eckardt et al. reported cases of recurrence in nonvascularized bone grafts; however, they acknowledged that adequacy of the surgical margins on histological analysis could not be determined .Basat et al. first described ameloblastoma recurrence in a free fibula flap reconstruction . This ocSharma et al. also described recurrence in a free fibula flap reconstruction . No commVarious methods for reconstruction of the resulting defect have been reported, including titanium plates and nonvascularized bone grafts. However, where microvascular expertise is available, bony free tissue transfer is normally advocated, especially for defects over 6 cm. In this case, vascularized bone was essential as an osteotomy was required to form the anterior neo-mandible and a soft tissue component was required to reconstruct the resected mucosa. The posterior mandible (Brown Class 1) is the most commonly affected site for the development of an ameloblastoma. In these cases, we favor the deep circumflex iliac artery (DCIA) flap . The vol The free vascularized scapula flap can also been used in mandibular reconstruction . For lonTo date, there are only two cases in the literature reporting recurrent ameloblastoma in the free fibula flap after segmental resection and reconstruction. Neither of these cases reported on the adequacy of the initial surgical resection. We present the first described case of recurrent ameloblastoma involving a fibula neo-mandible in the presence of adequate initial surgical margins. We also present the first case of management of reconstruction in the setting of recurrence in a fibula neo-mandible and demonstrate that it is possible to achieve a good outcome using a contralateral fibula flap. The case illustrates the value of digital planning in reconstruction of bony defects of the jaws resulting in successful reconstruction using the contralateral fibula.