The clinical presentation and histologic features are usually unspecific, resulting in frequent misclassification. As an unusual tumor, ESTFs have an unpredictable clinical behavior. (2) Methods: We present two clinical cases referred to our Maxillofacial Surgery Unit for the onset of a symptomless mass involving, in one case, the parotid gland, and in the other case, the sublingual gland. (3) Results: Solitary fibrous tumors could be considered as neoplasms with intermediate biological behavior that are not entirely predictable on the basis of morphological features, as these are mostly still unknown. However, a few histologic, immunohistochemical, and imaging features, such as a hypodense signal at the T1 sequence in an MRI, or positivity for CD34, bcl2, and CD99, and the NAB2-STATS6 fusion gene, could be useful for an early differential diagnosis of ESTFs. (4) Conclusions: All patients were alive at follow-up with no evidence of disease. Surgical management should always be considered as the first choice for oncological radicality, and clinical behavior should always be defined with the help of the study of radiological and anatomopathological features. In 1931, Kempler and Rabin described a series of localized solitary pleural tumors arising from subpleural areolar tissue, and this was thought to be the first description of what we now refer to as the solitary fibrous tumor (SFT) [They proposed that diffuse mesothelioma was mesothelial in origin, while mesothelioma (now known as solitary fibrous tumor) was of submesothelial origin .The histogenesis of SFTs is controversial; little evidence in the literature supports a mesothelial origin, but modern immunohistochemistry and ultrastructural features demonstrate that SFTs are not mesothelial but involve undifferentiated mesenchymal cell histogenesis.The SFT has mesenchymal cell origins and arises from primitive fibroblast-like cells in connective tissue. As a matter of fact, spindle cells tumors bear a close resemblance to this localized pleural tumor, and they have also been described in a variety of anatomic sites that are not lined by the mesothelium .SFTs have been reported in numerous extrathoracic anatomical regions, such as the orbits, oral cavity and salivary glands, thyroid, larynx, trachea, intracranial and spinal cord meninges, liver, pancreas, adrenal gland, kidney, female genital tract, prostate, pelvis, breast, soft tissue of extremities, and periosteum of bone.Currently, it is estimated that about 30–40% of reported SFTs are extrapleural. Among the extrathoracic sites, the orbit and the soft tissue of the extremities (muscles and subcutaneous tissues) are the most commonly reported anatomical regions [As a rare and unusual oncologic entity, ESFTs’ immunohistochemical, radiological, and laboratory features are unspecific, and they may have a clinical presentation as an indolent symptomless mass, and for this reason, ESFTs could be miscategorized and not immediately diagnosed. There are some common features that are used to reach a correct definitive diagnosis, such as the immunoreactivity of antibodies directed against CD34, as well as, in few cases, CD99 and Bcl-2; under a radiologic point of view, the best imaging technique to typify the ESFT is the MRI, as usually this tumor shows a hypodense signal at the T1 sequence, and T2-weighted sequences are useful for the visualization of the fibrous component of the lesions, which has a characteristically hypodense signal in T2.T1-weighted and CT images are also important for the evaluation of the hemosiderin component and calcifications, which are difficult to evaluate in T2 sequences. The enhancement of SFTs on postcontrast CT scans and MR imaging is commonly heterogeneous, a typical appearance also called “geographic paper”. Usually, laboratory features are completely normal, but occasionally hypoglycemia is reported, which is probably related to IGF’s secretion by ESFTs’ neoplastic cells.Surgical resection represents the first treatment choice; when this is not practical, radiation therapy could be considered after evaluating the tumor’s behavior and anatomopathological diagnosis or the margins of excision of the ESFT.Recurrencies are uncommon, but a close follow-up is always recommended.In our experience, a 52-year-old man and a 42-year-old woman were referred to the Maxillofacial Surgery Unit for the onset of a symptomless mass involving, in the first case, the parotid gland, and in the second case, the sublingual gland [A 52 year-old man presented in October 2021 to the U.O.C. of the Maxillofacial Surgery Unit at the “Università degli studi della Campania- L. Vanvitelli with a non-ulcerated, non-exophytic, hyperemic, large, firm mass without everted or rolled margins that was apparently painless, with a maximum diameter of 7 × 2.3 cm that extended from the left preauricular region to the middle-parotid region a.The patient reported the development and swelling had taken place for about a year during the pandemic. Following his primary care physician’s recommendation, an echo-guided FNAC of the lesion was performed for the patient, which described a cytologic result of a blood background and a monomorphic population of basal cells, which was poorly differentiated and predominantly organized in three-dimensional solid groups. No metachromatic matrix or cribriform aspects were observed, and the primary diagnosis was compatible with a basaloid cell neoplasm or a spindle cell myoepithelioma and classified following the Milan System for Reporting Salivary Glands Cytopathology (MSRSGC) as a category IV-B or SUMP .A soft tissue echography was carried out for the patient in September 2021; it was performed with a multifrequency linear transducer. On the left, at the level of the palpable and visible tumefaction, appearing as well-circumscribed and apparently capsulated in the parotid region, there was a coarse structural alteration with a homogeneously hypoechogenic pattern with multiple shoots and septa of medium echogenicity affecting the entire gland. This alteration showed multiple vascular signals on color Doppler inspection that were classified as a mixed ultrasound pattern. Lymph nodes with a reactive appearance and a maximum diameter of 1 cm were described at upper and lower parotid peripolar sites. There was no evidence of dilatation of Stenone’s duct.In September 2021, a CT scan was carried out for the facial massif, performed in basal conditions with a multidetector spiral technique. This examination was performed without contrast, upon request of the patient, and there was evidence of asymmetry of the parotid loggias due to a marked volumetric increase in the left one, which was moreover altered by artifacts from dental implants on the maxillary upper; reactive lymph nodes were represented as peri- and subcentimetric in the left submental, submandibular, and laterocervical stations. b.In October 2021, the echo-guided FNAC of the left parotid region was repeated.The morphological result shows direct smears against a blood background, a modest cellularity consisting of a cohesive cell population in small groups. The cell population was small and almost devoid of cytoplasm, with basaloid differentiation. The diagnostic category for cytology in salivary glands, according to the Milan System, was confirmed to be IV-B SUMP.The patient was operated on and dismissed from our U.O.C. in November 2021.The surgical technique involved a classical Blair incision with subsuperficial musculo-aponeurotic system dissection in the left parotid region. The mucocutaneous flap was raised to the anterior and inferior borders of the gland. The posterior branch of the greater auricular nerve was carefully identified and preserved. Soft tissue dieresis extended to the posterior belly of the digastric, sternocleidomastoid was found, and the facial nerve trunk and its branches were found to be isolated and preserved . Left superficial parotidectomy was performed, with facial nerve branches found to be intertwining and adhered to the neoformation. The deep portion of the tumor was removed, which was strongly vascularized and attached to the masseter muscle. A superficial musculoaponeurotic system (SMAS) platysma flap was sutured to the parotid capsule, and parenchyma was used to replace soft tissue volume loss and reduce the incidence of Frey syndrome . HemostaTwo hours after surgery, the patient had an early postoperative complication caused by abnormal bleeding. He was reoperated on for a wound revision and evacuation of the hematoma to control the bleeding sites. During the operative procedure, the previous incision was used as the surgical access. The bleeding surgical sites were washed with saline solution so the vessel in the active bleeding phase could be found. Clots were aspirated, and a procedure to control the hemostasis was performed; a new suction drain was positioned, and the parotid lodge was closed primarily with resorbable sutures.At the end of the surgery, the patient was transferred to an intensive care unit for 24-h close systemic monitoring, in accordance with our protocol for surgical patients after an early complication a.The postoperative therapy was based on intravenous antibiotic therapy , pain relief therapy , corticosteroid therapy , and proton-pump inhibitors .The patient was discharged home 3 days after surgery with the antibiotic, pain relief, and corticosteroid therapies. We also indicated Kabat therapy ,8 Figurb, and thThe postoperative histopathologic study had a macroscopic description of a nodular specimen of 4 × 2.5 × 2 cm with an adherent portion of salivary gland with dimensions of 4 × 3 × 2 cm, showing a yellowish lobulated injection that appeared apparently capsular, and, once serrated, had a compact whitish appearance. The microscopic description showed a well-circumscribed mesenchymal proliferation that was predominantly hypercellular with sparse hyalinized areas. Focal areas with a pseudocystic pattern were present. The neoplasm predominantly consisted of a epithelioid cell population, with round oval nuclei and cytoplasm with indistinct borders; in some areas, the cellular component took on a more spindle-like appearance with pleomorphism. In the context of proliferation, there was a conspicuous proportion of small-to-medium-caliber vessels, with a wide lumen; in the medium-caliber vascular component, the walls were hyalinized. Histiocytes phagocytosing hemosiderin pigment were present in the hyalinized areas. Erythrocyte extravasation was observed. Necrosis was absent. Poor mitotic activity (up to 2 mitoses/10 HPF) was also noted. Immunohistochemical studies showed positivity for STAT6, bcl, and CD99 and negativity for smooth muscle actin, calponin, CD34, CD31, ERG, p63, GFAP, S100, CKAE1AE3, and CK7. The serous-type salivary gland parenchyma present in the specimen appeared unscathed b. A smalThe histological diagnosis reports morphologic and immunophenotypic aspects consistent with an extrapleural solitary fibrous tumor.At the 6-month follow-up, after Kabat therapy, the patient recovered a facial nerve deficit of about 85% and did not show signs of recurrency a,b.A 42-year-old woman was referred to the Maxillofacial Surgery Unit in February 2018 for a non-ulcerated, non-exophytic, normochromic submucosal mass without everted or rolled margins involving the left oral pelvis, mainly the left sublingual area. Intraoral examination revealed a firm nodule about 3 cm in diameter a. A multThe lesion was shown to be homogeneous and isointense on T1-weighted images and heterogeneous and mildly hyperintense on T2 images; there was no evidence of bone infiltration, and cervical nodes with swelled dimensions were found b.An FNAC of the lesion was performed in March 2018 for the patient, showing scanty cellularity represented by spindle cells with plump nuclei with an inconclusive diagnosis of mesenchymal proliferation.Therefore, a surgical approach to the mass was suggested to a multidisciplinary committee and then planned.The surgical technique involved an intraoral excision to the left floor of the mouth, from the incisive region to the molar region, along the major axis of the neoformation. Exposition of the lesion was performed, and after an intraoperative examination, the mass appeared to be adhered to the surrounding tissues, especially to the sublingual gland that was completely removed. The lingual nerve was not infiltrated by the tumor, and for this reason was spared. At the end of the surgery, the patient stayed in our unit for 48-h close systemic monitoring, in accordance with our protocol for surgical patients at risk of an early complication a.The postoperative therapy was based on intravenous antibiotic therapy , pain relief therapy , corticosteroid therapy , and proton-pump inhibitors .The patient was discharged home 3 days after surgery with the antibiotic, pain relief, and corticosteroid therapies.Gross examination showed a nodule with a diameter of 2.8 cm and a whitish, solid, and vaguely multilobulated cut and capsulated appearance; however, microscopically, the specimen was constituted by a major component of neoplastic proliferation arranged in a multinodular pattern with alternation in the hypocellular and hypercellular areas, and showed oval-to-spindle-shaped cells with undefined cellular borders and bland nuclear features, embedded in a fibrillar and myxoid stroma, and a dense cellular population with very scant stroma. In these fields, the cells showed an oval shape, scant cytoplasm, undefined cellular borders, a more irregular chromatinic pattern, and relevant mitotic activity (about five mitoses on 10 high-power fields). The neoplasm also showed well-marked and capsulated borders. Immunohistochemistry was performed and included the following antibodies: CK (clone AE1/AE3), CD34 (clone QBEnd/10), CD99 (clone O13), bcl2 (clone 124), S100 (clone SP127), desmin (clone DE-R-11), ALDH1 (clone 44/ALDH1), and STAT6 (clone EP325) b.The mass was positive for CD34, CD99, and ALDH1 and focally positive for bcl2. Interestingly, CD99 stained the hypercellular areas more intensely. Stains with STAT6, S100, and desmin were negative. A diagnosis of a malignant extrapleural solitary fibrous tumor was confirmed.The postoperative combined multiparametric MR-PET confirmed the total removal of the tumor and showed no other location of the neoplasm.A follow-up of 2 years was performed, and no recurrence was evidenced.A literature search was also performed in order to compare our findings with other reports. The research was carried out on the PubMed database, for which we identified articles up to July 2022. Article language was limited to English using database-supplied filters. No limitations on study designs were included. The keywords used and combined with Boolean operators were as follows: “solitary extrapleural fibrous tumor”, “parotid gland”, “submandibular gland”, “sublingual gland”, “salivary gland”, “malignancies”, “solitary extrapleural fibrous tumor of the head and the neck”, and “benign tumors”. The literature search identified 10 articles. Six articles were excluded due to having no relevance to this case . Four articles were selected for full-text evaluation. The selection process identified three articles that were compatible with our criteria, which will be discussed in the The etiology of extrapleural SFTs is currently not defined, and no association has been demonstrated with smoking habits or asbestos exposure. Numerous karyotypic aberrations, including both numerical and structural abnormalities, have been reported in extrapleural ESFTs.Several studies reported a gain of chromosomes 5, 8, 13, and 21; others reported a complete loss or partial deletion of chromosomes 1, 6, 9, 13, 15, 17, 18, and X [Another important aspect is the demonstration of the NAB2-STATS6 fusion gene, which encodes a chimeric protein; correspondingly, the C-terminal repressor domain of NAB2 is replaced by a highly variable portion of the STAT6 protein.There is not a specific age of appearance, but they are more frequent in the fifth decade, with no gender difference.The majority of ESFTs have an indolent course, and they have a low risk of recurrence after their surgical treatment. The 10-year overall survival in this case is about 90%.A total of 10–25% of ESFTs may have local recurrence or will present as a disseminate disease. These patients and those who are not eligible for the surgical treatment or cannot resort to surgery have a poor prognosis [In 2013, WHO classified SFTs as fibroblastic–myofibroblast neoplasms with intermediate, rarely metastasizing, biological behavior. The clinical symptoms depend on the anatomical site of development, but they usually occur as slowly growing masses, being often an asymptomatic incidental finding or related to the pressure generated on the adjacent anatomical structures .It is possible, if rare, to have hypoglycemia, probably due to the secretion of IGF by the neoplastic cells .The most useful diagnostic technique used to evaluate solitary fibrous tumors, as mesenchymal-cell-derived tumors involving mainly soft tissues, is magnetic resonance imaging (MRI); most of the tumors (about 80%) show a hypodense signal at the T1 sequence.T2-weighted images are extremely helpful in identifying the fibrous component of the lesions, which has a characteristically hypodense signal in T2.T1-weighted and CT images are useful to evaluate two of SFTs’ distinguishing elements: a hemosiderin component and calcifications, which are really difficult to evaluate in T2 sequences, due to their low signal.In solitary fibrous tumors with a low fibrous content, T2-weighted scans have an intermediate-to-hyperintense signal. Lesions with a diffuse myxoid loose stroma component also appear hyperintense in T2-weighted scans. The contrast enhancement is heterogeneous (with a characteristic “geographic paper” appearance) in most cases .Due to the infrequency of ESFTs, little is known about their pathological characteristics and clinical features. For these reasons, ESFT salivary glands tumors are a diagnostic challenge for every oral and maxillofacial and head and neck surgeon .The management of ESFTs, as a rare oncological entity, should always be discussed among a multidisciplinary committee for an early diagnosis, as the radiological, the anatomopathological, and oncological features could be easily unrecognized or misclassified.The first clinical case that we presented had histologic, immunohistochemical, and imaging features comparable to the ones reported in the literature; we report a characteristic feature of that case, the intense vascularization of the neoformation, which led to postoperative complications and reintervention. However, in the second clinical case, histologic, immunohistochemical, and imaging features were seen, which are completely new in the literature, and comparable to other sublingual ESFT cases.The reported solitary fibrous tumors had a benign course. About 10–15% have aggressive or malignant behavior; cases of fibrous tumors mutated into high-grade sarcomas are reported in the literature [Solitary fibrous tumors could usually be considered as neoplasms with intermediate biological behavior that is not entirely predictable on the basis of morphological features; however, according to the modern anatomopathological literature, a few morphological features are associated with a higher rate of local recurrence and more frequent distant metastases, such as infiltrative margins, pleomorphism, hypercellularity, a mitotic index greater than 4/10, high-power fields, and necrosis . If an eIn one of our cases, we had an ESFT with hypercellularity, nuclear atypias, and a mitotic index of five mitoses per 10 high-power fields, so it could be defined as a “malignant ESFT”. Meanwhile, in the other case of ESFT, we found poor mitotic activity with a mitotic index of two mitoses per 10 high-power fields, pleomorphism, hypercellularity without nuclear atypias, and well-circumscribed margins, without necrosis; thus, we could define it as a “benign ESFT”. ESFTs arising from the parotid gland parenchyma are rare, with only 40 cases (including the current one) reported in the literature since 1995. Most reported parotid gland SFTs (PG-SFTs) show benign behavior; meanwhile, malignant ESFTs are even rarer in salivary glands, and the current literature shows only three case reports, to the best of our knowledge .The first is an article written in 1997 by J. Sato et al. with the title “Solitary fibrous tumor of the parotid gland extending to the parapharyngeal space”, and described a case of an ESFT of the deep lobe of the parotid gland that invaded the parapharyngeal space, with a so-called histological “patternless pattern”, with a successful resection by an external approach. The patient was recurrence-free for 1 year after the surgical operation .The second article, which is compatible with our domain, was written in the 2001 by Alawi et al. with the title “Solitary Fibrous Tumor of the Oral Soft Tissues A Clinicopathologic and Immunohistochemical Study of 16 Cases”, and describes 16 cases of ESTF oral soft tissue, including two salivary-gland-related cases with immunohistochemical, clinical, and radiological features similar to our first described case of “benign ESTF” .The third article is compatible with our outcome domain and was written in 2002 by Muñoz Guerra et al., with the title “Solitary fibrous tumor of the parotid gland: A case report”, and describes a case of a 37-year-old woman with a symptomless mass in the right parotid gland without any evidence of facial nerve paralysis with same radiological, clinical, and histopathological features of our first case. In this article, the ESTF is described as a completely rare entity, with 12 cases in the literature described for the major salivary glands, and only 6 cases for the parotid glands. Additionally, in this case, there was a “patternless” histological pattern, and a very interesting element of the article was the differential diagnosis of soft tissues and the immunohistochemistry profile of CD34-positive tumors of soft tissue, such as solitary fibrous tumors, hemangiopericytoma, spindle cell lipoma, Kaposi’s sarcoma, peripheral neural sheath tumors, or malignant fibrous histiocytoma. This article also highlights that the complete behavior of these neoplasms in the parotid gland is still unknown, and notes that as we have few anatomopathological indicators that can be used to establish the most adequate surgical plan, there are still no defined reliable guidelines for tumor prognosis .SFTs of the salivary glands are extremely rare oncologic entities that most often occur in middle-aged patients, with no predominance in a certain gender, who may have been living with a symptomless mass lesion for a long time. Diagnosis of SFTs is based on classical histologic and immunophenotypic features with CD34, bcl-2, and CD99 immunoreactivity, which allows for distinction and separation from other tumors in the differential diagnosis, but we hope that in the future, clinical and radiological features could also lead to a better early diagnosis. The presence of hypercellularity, relative pleomorphism, and a mitotic index of 2/10 HPF suggest a possible increased aggressiveness of the neoplasm.Given the rarity and unpredictability of the biological behavior of these tumors, it is necessary to schedule long-term follow-ups, as there is a possibility that patients will develop recurrences or distant metastases even many years after surgery . Follow-


Parasitic leiomyomas (PL) are rare cause of small bowel obstruction (SBO) in young women. Usually, they arise in women who underwent laparoscopic or surgical morcellation of uterine fibroids. PL may present with vague abdominal pain, constipation, obstipation, or rarely SBO. SBO can be primary or secondary, depending on prior surgical history. PL might present as primary SBO due to their mass effect or secondary SBO if the patient’s PL resulted from a surgical procedure. We came across a very remarkable presentation of primary SBO due to an artery supplying the PL. Few cases of primary PL have been reported. Leiomyomas are intraabdominal uterine neoplasms . They arA 59 years old female with a pertinent past medical history of jejunal diverticulitis presented to ED with one day of progressive periumbilical abdominal pain. The pain was 9/10 in intensity sharp intermittent and radiating to the back. The patient denied any aggravating or relieving factors. The patient reported nausea with vomiting and obstipation. The patient vitals are presented in Table On the physical exam she appeared lethargic. The abdomen was distended and diffusely tender. The patient’s blood work is presented in Tables There was a high suspicion of possible small bowel obstruction (SBO), and CT scan of the abdomen and pelvis with contrast showed focal dilation of mid-jejunal small bowel with inflammatory changes in the mesenteric fat and a jejunal diverticulum Figure . GastroeThe surgeon did a stricturoplasty procedure at the obstruction site with a small bowel resection at the jejunal diverticulum site around 2 cm of the jejunum removed with end-to-end anastomosis. The patient had an uneventful postoperative course. She recovered bowel function and fared well.Leiomyoma or fibroid are benign tumors of the smooth muscle of the uterus. PL is a rare variant of uterine leiomyoma that grows outside the uterus. FIGO classification of uterine leiomyoma described eight types, providing a representative map of fibroid distributions, according to FIGO, types 2-5 represent submucosal and subserosal leiomyoma while type 8 represents PL. Leiomyoma has a higher incidence in African-American females than Caucasian females. As most patients are asymptomatic, the true incidence/prevalence is difficult to determine . The prePL is considered a subserosal fibroid, while others believe it is an iatrogenic complication of laparoscopic myomectomy or morcellation . PL is aSBO is a more grave and unusual presentation requiring surgical intervention. The differential diagnosis for PLs includes masses of ovarian origin (primary ovarian neoplasms and metastases) and broad ligament cyst. At the ultrasound, a typical leiomyoma usually has a whorled appearance with variable echogenicity depending on the extent of degeneration and calcification. MRI helps differentiate leiomyomas from solid malignant pelvic tumors. The typical leiomyomas demonstrate low to intermediate signal intensity on T1-weighted images and low signal intensity on T2-weighted images. An ultrasonography-guided biopsy is vital in determining its exact composition before surgery. The PL in our patient did not cause SBO due to the mass effect. It derived its blood supply from the mesenteric vasculature causing strangulation of the jejunum (as described in case presentation). Our patient presented with signs and symptoms of acute abdomen. Our patient did not demonstrate any symptoms suggestive of gynecological etiology for her acute abdomen. SBO is categorized as primary if the patient has no surgical history or secondary bowel obstruction when a patient reports a history of abdominopelvic surgery. In a systematic review and meta-analysis of six studies including 442 patients, the prevalence of neoplastic etiologies for SBO without abdominopelvic surgery history differs from 7.7 to 13.4% at the same time and adhesions were the culprit in 54% of cases . More thLeiomyomas are common benign tumors of the smooth muscle of the uterus. In rare circumstances, subserosal leiomyoma may detach from the uterus and attach itself to nearby organs. It is called PL. The most typical source of blood supply for the PL is the omentum. PL is a rare type of uterine leiomyoma that affects young women. They have an atypical presentation with symptoms ranging from asymptomatic to mild abdominal pain to a high-grade SBO. Still, the most common symptoms are pressure symptoms, early satiety with bloating during eating, abdominal discomfort, and nausea. Diagnosis is often challenging for clinicians. There is a high index of suspicion for PL in middle-aged women with vague abdominal symptoms. The management is usually through surgery either open or laparoscopic.


Background and Objectives: Non-cystic manifestation of autosomal dominant polycystic kidney disease (ADPKD) is an important risk factor for cerebral aneurysms. In this report, we describe a rare spontaneous internal carotid artery (ICA) dissection in a patient with ADPKD. Observations: A 38-year-old woman with a history of ADPKD and acute myocardial infarction due to coronary artery dissection experienced severe spontaneous pain on the left side of her neck. Magnetic resonance imaging (MRI) revealed a severe left ICA stenosis localized at its origin. Carotid plaque MRI showed that the stenotic lesion was due to a subacute intramural hematoma. Close follow-up by an imaging study was performed under the diagnosis of spontaneous extracranial ICA dissection, and spontaneous regression of the intramural hematoma was observed uneventfully. Conclusions: When patients with a history of ADPKD present with severe neck pain, it is crucial to consider the possibility of a spontaneous ICA dissection. A carotid plaque MRI is beneficial in the differential diagnosis. Conservative management may benefit patients without ischemic symptoms. Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary diseases characterized by bilateral renal cysts and liver cysts . The nonA 38-year-old woman presented to our department with severe spontaneous pain on the left side of her neck. Her blood pressure was normal. Physical examination revealed no remarkable abnormality. Neurological examination revealed a score of 15 points (E4V5M6) on the Glasgow Coma Scale (GCS) without focal neurological deficit. She was previously diagnosed with ADPKD. She also had a history of acute myocardial infarction due to spontaneous coronary artery dissection shortly after delivering her first son 18 months prior and was relieved by conservative management. She had no history of hypertension, diabetes mellitus, or dyslipidemia. She also had no history of smoking and drinking. Her mother had been diagnosed with ADPKD. She had no family history of arterial dissection. Her carotid echo examination showed severe stenosis at the origin of the left ICA; the stenotic lesion was hypoechoic, and the peak systolic velocity increased to 332.1 cm/s at the distal ICA. Her echocardiographic findings were normal. Her abdominal computed tomography (CT) showed multiple cysts in the liver and bilateral kidneys A,B. Her Although the patient had severe left ICA stenosis, she was without ischemic symptoms; therefore, we followed her closely with imaging studies without antiplatelet agents.Her clinical course was uneventful. MRA performed three months after onset showed that the stenotic lesion of the left ICA had disappeared A, and plWritten informed consent for treatment and publication was obtained from the patient.ADPKD is the most common hereditary renal disease . It is cSpontaneous extracranial ICA dissection associated with ADPKD is extremely rare, with only nine cases reported in the English literature to date, including our patient  5,6,7,8,87,8,9. The detailed mechanism by which arterial dissection occurs in ADPKD patients remains unclear. ADPKD is a systemic disease resulting from mutations in either polycystic kidney disease-1 (PKD-1) or PKD-2 genes . PKD-1 eThe usefulness of vascular imaging has been reported in diagnosing spontaneous extracranial ICA dissection ,19. MazzIn our case, carotid echo examination showed a hypoechoic severe stenotic lesion at the left proximal ICA. The findings were similar to those of common atherosclerotic lesions and were non-specific. On the other hand, a high-resolution carotid plaque MRI showed a homogenous, high-intensity lesion causing severe stenosis both on T1- and T2-weighted images. These findings are consistent with a recent hemorrhage detected on high-resolution MRI . Intra-pp = 0.58) during the 12-month follow-up. There was also no difference in the presence of residual narrowing or occlusion between those receiving AP (n = 56 of 92) versus those receiving AC (n = 53 of 89). Therefore, it is crucial to thoughtfully determine whether to use AP or AC for ischemic onset cervical artery dissections on a case-by-case basis.There is a lack of evidence regarding the optimal treatment of spontaneous extracranial ICA dissection. The recently published Cervical Artery Dissection in Stroke Study (CADISS) randomized clinical trial included 250 patients  within one week from onset to antiplatelet or anticoagulation therapy . This stFurthermore, there is no evidence of optimal treatment for spontaneous extracranial ICA dissection patients without ischemic symptoms. Published studies showed a similarly low risk of stroke and no evidence of an increased stroke rate in patients with ICA dissection . Thus, eSpontaneous ICA dissection with ADPKD may have a benign prognosis similar to ordinary spontaneous extracranial ICA dissection. The reviewed cases and our cases were examined together, demonstrating that the prognosis was favorable in six cases, except for one patient with cerebral infarction, out of the seven total cases in which the prognosis was described  22]. St. St22]. We reported a rare case of spontaneous extracranial ICA dissection associated with ADPKD. If a patient with ADPKD complains of sudden neck pain, it is crucial to take a closer look considering the possibility of spontaneous extracranial ICA dissection. Cervical MRI is useful in extra cranial diagnostic imaging, especially in carotid plaque MRI, where an intramural hematoma can be identified as a lesion with more characteristic signal intensity depending on its stage. It is also helpful because high-definition images may show the entry point. The pathological condition is the same as that of ordinary spontaneous extracranial ICA dissection, and there is a high possibility of spontaneous resolution of the lesion. Therefore, conservative management based on close imaging follow-up is a practical option.


Space occupying lesions compromising frontal lobes usually may produce in the first place psychiatric symptoms such as progressive change of personality and/or symptoms suggestive of depressive episodes. Thus they can be misdiagnosed and mistreated.A case report is presented as well as an updated review of frontal lobe tumor diagnosis and treatment literature.We present the case of a 45 years-old male patient with no relevant medical history who arrives at the mental health center due to behavioral disorders, depressive mood, workplace absenteeism and personal hygiene neglect in the last 3 months.Since the clinical picture was compatible with depressive disorder the patient was treated with psychotherapy and antidepressant drugs with no remission. Due to the treatment absence of response he attends emergency services where he is performed a craneal tomograpy (CT) where a right frontal lobe tumor (FLT) is observed.In early stages FLT are sometimes presented as psychological mood or anxiety disorders without accompanying neurologic deficits. Thus, mental health professionals should be aware that psychological symptoms might be a presentation of organic disease of the brain and in some cases  organic screening and hence brain imaging should be considered.No significant relationships.


Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a rare T-cell lymphoma associated with textured breast implants. The most common presentation is a periprosthetic seroma that occurs at least 1 year after an aesthetic or reconstructive implantation, and in these cases, the surgical treatment seems to be successful. More rarely, BIA-ALCL presents with locally advanced mass-formed disease and a related regional lymph node involvement. In all these cases with worse prognosis, a multidisciplinary approach is required, including adjuvant chemotherapy, radiation therapy, and surgery. We present a clinical case of a 49-year-old woman who developed on the left side of the breast a mass-formed stage 3 BIA-ALCL 15 years after a bilateral breast augmentation with textured silicone implant. Our multidisciplinary team (MDT) scheduled the patient for a “reverse-strategy” sequential approach consisting of induction chemotherapy, hematopoietic stem cell mobilization, and harvest followed by autologous stem cell transplant (ASCT). After 100 days from the stem cell transplant, the patient showed a complete pathologic response and was a candidate for radical surgery. She underwent removal of both implants with total en bloc capsulectomy. On the left site, the periprosthetic mass was also en bloc removed. We did not perform any axillary dissection. Our surgical and hemato-oncological teams followed the patient every 3 months, and no local or systemic recurrences were observed 24 months after surgery. This case report has demonstrated the effectiveness of neoadjuvant chemotherapy as part of a “reverse strategy” in selected cases of advanced-stage BIA-ALCL in which it was not possible to perform an immediate radical surgery. Furthermore, in our case, the de-escalation strategy adopted permitted a less demolitic surgery with good functional and aesthetic results. Breast implant-associated anaplastic large cell lymphoma (BIA-ALCL) is a recently recognized non-Hodgkin lymphoma of T-cell origin. Despite its low incidence, the increasing use of breast implants for aesthetic reconstruction or post-mastectomy purposes poses BIA-ALCL as an emerging medical challenge . All cliAlthough most patients with BI-ALCL have a relatively indolent clinical course presenting as a delayed effusion or persistent seroma around the implants, one-third of the cases develop a peri-capsular mass and a small number of lymph-node involvement or distant disease , 3.The National Comprehensive Cancer Network (NCCN) guidelines and the UK guidelines on the Diagnosis and Treatment of BIA-ALCL suggest that during the early stage, surgical removal of the implant with the surrounding capsule intact usually has a curative effect. For mass-forming cases, surgery was also indicated with a total en bloc capsulectomy with removal of any associated mass and abnormal regional lymph nodes followed by adjuvant chemotherapy and/or radiotherapy , 5. In aWe report a case of a woman with locally advanced BIA-ALCL treated with an unconventional combined approach in which a sequential chemotherapeutic program was applied in a neoadjuvant setting (reverse strategy).peau d’orange on the left breast ; the local swelling and the increase in skin thickness affect all the breast tissue. In addition, suspicious axillary lymph nodes have been reported in the same side. Given the complexity of the case, the patient was sent to our Breast Surgery Department of the “Regina Elena National Cancer Institute” in Rome, considered as a reference point in central-southern Italy for breast cancer , 7. We s cm; the 6/kg collected: 7.024). At the end of the induction phase, a disease assessment performed by a CT/PET scan showed a complete remission. Afterwards, a consolidation treatment with ASCT after BEAM conditioning regimen  was finally performed , according to our institutional policy and international guidelines about treatment of advanced-stage ALK-negative peripheral T-cell lymphoid malignancies –12. In pOne hundred days after the stem cell transplant, she underwent removal of both implants with total en bloc capsulectomy. On the left, the site of the periprosthetic mass was also removed  associated with breast implants with a textured outer. Since its first description in 1997 by Keech and Creech , an incrThe most common presentation of BIA-ALCL is a large spontaneous periprosthetic fluid collection occurring at least 1 year and on the average from 7 to 10 years following the implantation with a textured surface breast implant. In addition to large fluid collections and delayed seromas, 8% to 24% of patients present an associated palpable mass and 4% to 12% present with lymphadenopathy .Surgical excision is the established standard of care to treat the localized disease, but the BIA-ALCL treatment algorithm suggested by international guidelines is limited in case of unusual, progressive, or complex patient scenarios , 19. ThiOur clinical case falls into one of those advanced BIA-ALCL stages (stage III) , 22 withOur multidisciplinary oncology board, which involved hematologists, surgeons, pathologists, and radiotherapists, did not consider the patient as a candidate for immediate surgery due to the advanced stage of the disease. Such an intervention would have been extremely destructive and not safe. Keeping in mind the importance of obtaining safe negative surgical margins and conservative surgery with oncoplastic techniques, the patient was scheduled to administer chemotherapy and ASCT before surgery, reversing the commonly recommended strategy (reverse strategy).In accordance with our institutional policy and international guidelines about treatment of advanced-stage ALK-negative peripheral T-cell lymphoid malignancies –12, patiKeeping in mind the pivotal role of “free” surgical margins to reduce local recurrences , the surContrary to guideline recommendations, we did not perform any axillary dissection, despite the initial suspicion of multi-lymphonodal involvement. A complete response was obtained for the concomitant axillary lymph node, as expected after the medical management of common ALCL so that we avoided needless axillary dissection. Our patient was not interested in maintaining an enlarged breast and asked to avoid an immediate reconstruction with smooth implants. At follow-up, a device-free reconstruction was planned, leaving the breast in a natural-looking shape. Two other cases, in which neoadjuvant therapy has been administered to treat advanced BIA-ALCL, have been reported in the literature , 19.In both cases, a complete pathological response was achieved after neoadjuvant chemotherapy, and the adjuvant radiotherapy was deemed unnecessary, following a multidisciplinary consensus.Our case has shown that the use of neoadjuvant chemotherapy is effective in cases of advanced BIA-ALCL, when immediate surgical treatment is not indicated for the extension of the disease. In these scenarios, the reverse strategy, which involves the use of neoadjuvant chemotherapy, may obtain near-clinical complete response allowing for conservative surgery.The incidence of BIA-ALCL has continued to rise, but consistent data regarding recommendations for patients with a more aggressive presentation of BIA-ALCL are still lacking in the literature. In this optic, the role of unconventional therapeutic strategy should be investigated. Our report underlines the important role of neoadjuvant chemotherapy in BIA-ALCL to control disease burden before tumor resection in selected cases in which radical surgery is not possible or precluded due to the general condition of the patient.Informed consent was obtained from the participant for the publication of this case report including all data and images.The raw data supporting the conclusions of this article will be made available by the authors, without undue reservation.The studies involving human participants were reviewed and approved by Central Ethics Committee IRCCS Lazio – IRCCS I.F.O. with number RS1719/22. The patients/participants provided their written informed consent to participate in this study. Written informed consent was obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article.SC and FM wrote the main manuscript, its acquisition data, and the interpretation. LP, FPa, and FPe contributed to data acquisition. MCI made a critical review of the article. CB and MCo were in charge of the final approval of the version to be published. The final manuscript has been read and approved by all the authors.


Idiopathic extremity arteriovenous fistulas are rare, abnormal connections between arteries and veins commonly occurring between iliac vessels. We present the case of a 42-year-old female who was referred to our center for a mitral valve replacement with a one-year history of breathlessness and a six-month history of progressive abdominal distension. Physical examination revealed a pansystolic murmur in the mitral area, basal crepitations, and a non-tender pulsatile swelling in the right iliac fossa. Imaging showed severe mitral regurgitation in heart failure along with a large arteriovenous fistula between the common femoral vessels which suggested that the arteriovenous fistula was the etiology of heart failure. Postoperatively, the patient showed significant improvement in symptoms with imaging. On follow-up, the patient reported no progression of symptoms. Our case demonstrated an idiopathic extremity arteriovenous fistula in an unusual location, unexpectedly causing heart failure despite a significant valvular disease. Therefore, arteriovenous fistulas warrant inclusion in the differential of heart failure despite a valvular lesion and absence of classical signs of high output heart failure, as in our case. Arteriovenous fistulas (AVFs) are high flow connections between arteries and veins with a variable etiology, most of them being surgically created extremity AVFs in those with chronic kidney disease (CKD) ,2. FireaSpontaneous unilateral idiopathic extremity AVFs are rare and mostly occur in the iliac vessels ,3. They We report a 42-year-old female with idiopathic extremity AVFs presenting as a high output cardiac failure.A 42-year-old female with no known comorbidities was referred to our outpatient department for a mitral valve replacement with a one-year history of breathlessness associated with orthopnea, and for six months, progressive abdominal distension and subsequent pedal edema three months later. She had no history of trauma or catheter intervention. Her medical history was unremarkable apart from an uncomplicated lower segment Caesarian section 12 years ago.Physical examination was significant for a holosystolic murmur heard in the mitral area, apex beat displacement, and basal crepitations. Further, inspection revealed a soft, immobile, non-tender pulsatile mass in the right groin region measuring around 10x5 cm in size associated with a thrill. On occlusion of the swelling, there was a rapid decrease in heart rate and a rise in blood pressure. We made a clinical diagnosis of severe mitral regurgitation in heart failure. Laboratory investigations were unremarkable except for an increased thyroid stimulating hormone (TSH). The chest X-ray showed a cardiothoracic ratio of 0.78 Figure , and thePreoperatively, an ultrasound of the abdomen and pelvis suggested a possible right iliac artery aneurysm with a dilated inferior vena cava and moderate ascites, necessitating further imaging. Further, computed tomography (CT) of the chest and abdomen revealed a wide neck fistulous communication between the right common femoral vein (CFV) and the common femoral artery (CFA) with focal venous ectasia measuring 7x7 cm with no evidence of arterial occlusion or aneurysm Figure . This suWe counseled her for a surgical repair, and intraoperatively, we found an AVF between the common femoral artery and common femoral vein with numerous collaterals from the profunda femoris with a fistula opening of 2.5 cm. Collaterals were ligated, and the CFA and CFV were separated and repaired separately with 5-0 polypropylene. Postoperatively, the patient showed marked improvement in symptoms. Chest X-ray showed a considerable reduction in the cardiothoracic ratio falling from 0.78 to 0.69 Figure . The echArteriovenous fistulas are abnormal connections between arteries and venous. Based on the etiology, they classify as congenital, traumatic, iatrogenic, and spontaneous AVFs . The patClinically, peripheral AVFs present as pulsatile swellings with edema and hypertrophy of the affected limb . There cDiagnosis of an AVF is clinical and supported by imaging such as duplex ultrasonography and CT angiogram . We suggSurgical repair is the mainstay for all symptomatic AVFs consisting of resecting the fistula followed by primary closure or patch repair . ExpecteIdiopathic extremity AVFs are rare entities that seldom cause heart failure. However, they warrant inclusion in the differential when an extremity swelling is present. Diagnosis is made by Doppler ultrasound and can be aided by a contrast-enhanced CT. Surgical resection of the fistula tract with primary closure is the treatment of choice.


Solitary fibrous tumours are uncommon in head and neck region, especially in the nasal cavities and paranasal sinuses, with most cases reported in the thoracic region in the pleura. It is often considered a borderline or low-grade malignant soft tissue tumour. Complete surgical resection is currently the treatment of choice, though intracranial and orbital extension of these lesions must be carefully evaluated and navigated to ensure a safe outcome.A 36 years-old lady presented with a long one-year history of left-sided nasal obstruction with facial pain, headaches and mild visual disturbances. She had been treated for sinusitis for a prolonged period. Clinically, there was a left nasal mass obliterating the ostiomeatal complexes and the roof of the nasal cavity. MRI showed heterogeneously enhancing mass occupying the left ethmoid sinuses extending laterally eroding the left lamina papyracea to the orbit, medially towards the right nasal cavity eroding the nasal septum, and superiorly to extend intracranially. After inconclusive biopsies were performed, the mass was excised with a combined endoscopic and open lateral rhinotomy approach with left medial maxillectomy and reconstruction of the skull base defect. The tumour was eventually reported as a solitary fibrous tumour.Solitary fibrous tumour is a rare differential of tumours in the sino-nasal region, diagnosed via histopathology. Although generally slow-growing, these lesions may extend the adjacent structures namely the orbit and skull base. Definitive treatment via surgical resection may be performed safely after careful radiological assessment and multidisciplinary consideration. Solitary fibrous tumours (SFTs) are a rare type of spindle cell neoplasm which is mesenchymal in origin. Its histogenesis is controversial, whether its origin is mesothelial or undifferentiated mesenchymal cell . The majA 36 years-old lady, of Indian descent, presented to our clinic in September 2019 with a complaint of chronic left-sided nasal obstruction for one year. This was associated with ipsilateral headache and intermittent facial pain, with mucoid rhinorrhea and anosmia. She also complained of epiphora of the left eye and mild visual disturbances. She had frequented her local practitioner and was regularly treated for chronic rhinosinusitis. Upon review, there was an inapparent asymmetry of the left nasal bridge with fullness medial to the left medial canthus. On nasoendoscopy, there was a large left nasal mass arising from superiorly, occupying nearly the entire left nasal cavity . A 4mm sThe patient had earlier performed computed tomography (CT) of the paranasal sinus which reported an extensive lobular mass along the left lateral nasal wall predominantly centred in the middle meatal region with breeching and destruction of the medial wall of the left maxillary sinus and possible extension into it, with soft tissues densities occupying the ethmoid and frontal sinuses, extending superiorly with breaching of the skull base and laterally eroding the lamina papyracea into the extraconal orbit. There were also soft tissue densities occupying the left sphenoid sinus .In view of the extension into the anterior skull base, the patient was further evaluated with magnetic resonance imaging (MRI) of the brain. MRI brain revealed the presence of heterogeneously enhancing mass measuring 4.7x4.9x3.5cm (CCxAPxW) occupying the left ethmoid sinuses extended medially to both left and right nasal cavity with obliteration of nasal septum noted. The left lamina papyracea was eroded by the mass with extension to the medial part of the extraconal space of the left orbit compressing the left medial rectus, with no abnormal enhancement. The mass also extended superiorly to the left frontal sinus and erosion of frontal bone was seen with intracranial extension to the left frontal region with mass effect into the left frontal cortex and focal thickening of intact dura in the region. There was no abnormal enhancement or focal lesion of the left frontal lobe or other intraparenchymal lesions. There was mucosal thickening in the left maxillary and sphenoid sinuses . The imaThe decision was made for a biopsy of the left nasal mass to be performed in the clinic for histopathological evaluation. This was performed twice, over a week, however, with both samples yielding only granulation tissue. No deeper biopsies were performed due to significant bleeding during the procedure which was controlled with compression.The decision for surgical intervention endonasally, was made after further discussions and consultations with the local neurosurgical team. The patient was subjected to a left medial maxillectomy and resection of the left sino-nasal mass via a combined endoscopic and open  approach, with subsequent reconstruction. No peri-operative embolization was performed. Intra-operative findings showed a large firm, lobulated mass occupying the entire left nasal cavity and partially extending into the left maxillary sinus and bulging into the medial wall of the left orbit with a severely thinned out lamina papyracea. The tumour was excised en bloc with no significant bleeding encountered. A significant posterosuperior septal perforation was noted. There was a presence of skull base defect located posteromedial to the left frontal sinus opening, measuring 2cm x 1cm. After complete resection of the tumour, the dura was inspected and appeared to be intact. The skull base defect was reconstructed using a segment of fresh septal cartilage and mucosal flap from the lower left lateral wall of the nose pedicled to the posterior stump of the inferior turbinate. Polypoidal mucosae were also removed from the maxillary and sphenoid sinuses. The left lacrimal sac was marsupialized. Post-operatively the nasal cavity was packed in BIPP impregnated nasal packing which was removed after a week. The post-operative period was uneventful.Histopathological evaluation of the resected left sino-nasal mass , measuriPost operatively, she was reviewed on regular intervals with surveillance nasoendoscopies performed up to one year later. There was no clinical evidence of local recurrence, refer to  with MRIThe solitary fibrous tumour (SFT) is a rare soft tissue neoplasm. It has been postulated that it is mesenchymal in origin rather than mesothelial due to its ubiquitous location . The rea In a study done by Gold et al, solitary fibrous tumours of the head and neck region account for 6% with most cases reported in the oral cavity . Meanwhi Most of the sino-nasal solitary fibrous tumours are generally described as slow-growing tumours, with a range of duration of symptoms from 0.25 to 24 months, with an average of 9.9 months . DuratioIn addition, local recurrence with or without metastasis has been reported too. Thus, it is classified as a borderline or low-grade malignant soft tissue tumour in WHO Classification of Head and Neck Tumours 2017. To suggest it is an aggressive tumour, predictive factors of age more than 55 years old, tumour size more than 15cm, and more than 4 mitoses per 10 high-power fields are considered . In gene Definitive management of this disease is complete surgical resection of the tumour. Several surgical intervention approaches had been reported, namely the endoscopic approach  and the Certain considerations need to be taken into account in choosing the appropriate approach. In this patient, a combined endoscopic and open lateral rhinotomy approach is preferred due to the extensive extension of the tumour locally and intracranially. The main aim is to achieve a clear margin of the tumour and to prevent a recurrence. However, complete surgical excision might be difficult due to the close proximity of vital structures such as the orbit, dura and the vascularized nature of the tumour. Pre-operative embolization of the feeding vessel before the operation had been reported , to prevAdjuvant therapies were also described with conventional chemotherapy and were reported to be effective in controlling or stabilizing locally advanced or unresectable and metastatic solitary fibrous of all anatomical sites . 80% of Therefore, this finding needs to be coupled with the findings of negative expression of other immunohistochemical markers, such as S-100 protein and desmin. Moreover, vimentin and bcl-2 positivity can be seen sometimes , but it Solitary fibrous tumours (SFTs) are an uncommon type of sino-nasal neoplasms. The nature of the expansion of SFTs over time and the proximity of the region to the skull base and orbit, predilect an extension towards these structures. The diagnosis of SFTs may only be confirmed after a histopathological evaluation of the surgical specimen. Therefore, a comprehensive radiological assessment is necessary for determining the extent of the tumour for safe surgical management, with the subsequent need for close interval clinical surveillance to exclude recurrence.


Post-esophagectomy hiatal hernia is a rare complication having varied presentation from asymptomatic cases detected incidentally on follow-up imaging to acute surgical emergency for strangulation or gangrene. Patients presenting as a surgical emergency have a prolonged post-operative course with significant morbidity.We present three cases of post-esophagectomy hiatal hernia. Two of the three cases were operated for esophageal squamous cell carcinoma (SCC) and one patient was operated for esophageal leiomyomatosis. Two of the three cases  underwent minimally invasive Mckeown’s esophagectomy and one case underwent robotic transthoracic Ivor-Lewis esophagectomy. All cases underwent contrast enhanced CT (CECT) and were biopsy proven prior to their index surgery. Both cases of SCC had prior neoadjuvant chemoradiation followed by surgery while esophageal leiomyomatosis underwent upfront surgery. All three cases have improved symptomatically and are doing well on follow up . All three of our cases have different clinical presentation in terms of symptoms, severity, and time duration from index surgery. Two of the three cases underwent emergency surgery and one case which was asymptomatic detected incidentally on surveillance imaging and was managed conservatively. Post-esophagectomy hiatal hernia is a rare entity with varying presentation. The management options in such cases vary depending on the severity of symptoms and time after index surgery. In cases presenting as surgical emergency, successful management depends on prompt detection, early surgery, proper post-operative care, and rehabilitation. Hiatal hernia is an uncommon complication of esophagectomy. It can present incidentally, symptomatically, or as an emergency requiring urgent surgical intervention. The post-esophagogastric surgery hiatal hernia prevalence is 3.18%, and 2.01% of patients require surgical treatment [Case 1 A 62-year-old female with a body mass index (BMI) of 11.5 kg/m2 presented with complaints of severe abdominal pain, vomiting, decreased urine output, and difficulty with respiration for 3 days. She was a known case of squamous cell carcinoma (SCC) of the middle one-third of esophagus. She had received neoadjuvant chemo-radiotherapy (NACRT), following which she underwent a hybrid Mckeown’s esophagectomy with thoracoscopic mobilization of esophagus followed by laparotomy and gastric conduit formation and cervical esophago-gastric anastomosis 4 years prior. At the time of presentation, she had tachycardia, hypotension, and features of severe dehydration. There was tenderness but no abdominal signs suggestive of peritonitis or acute abdomen. On auscultation, air entry was absent on the left side. After receiving her in emergency, initially IV fluid was given and oxygen supplementation was started. Her routine blood investigations with arterial blood gas were done. and a chest X-ray was obtained. She was started on broad spectrum antibiotics. Her chest radiograph Figure  was suggShe then underwent exploratory laparotomy followed by reduction and resection of the herniated gangrenous transverse colon with primary repair of the hiatal defect and proximal ascending colostomy. Intraoperatively, there was a gross pleural effusion (2000 mL) present without any feculent peritoneal/pleural contamination. The esophageal hiatus was around 4 cm with a gangrenous transverse colon as the contents, present on the left side of the gastric conduit Figure . The disPostoperatively, she had a prolonged ICU stay and underwent tracheostomy. She was then gradually weaned off the ventilator after establishing her enteral nutrition and shifted out of the ICU on post-operative day (POD) 39. She was then rehabilitated in the ward and was discharged on POD 46. Her tracheostomy was decannulated two months later. She underwent a restoration of bowel continuity three months later, which was uneventful.Case 22 underwent minimally invasive esophagectomy for esophageal leiomyomatosis. A hybrid Mckeown’s esophagectomy with thoracoscopic mobilization of esophagus followed by laparotomy and gastric conduit creation with cervical esophagogastric anastomosis was done. A routine chest radiograph on POD 5 suggested herniation of the bowel loops in right pleural cavity with compression of the right lung and a mediastinal shift to the left. A contrast-enhanced computed tomography (CECT) of thorax and upper abdomen was done  as compared to the retrosternal route because the hiatal widening is done using the mediastinal route to prevent obstruction . A few aIn a study by Narayanan et al., the use of neoadjuvant therapy was also found to be associated with an increased incidence of diaphragmatic hernia, which may relate to more extensive perihiatal dissection required in patients with more advanced diseases who receive chemo-radiation . It has Management usually depends on the type of presentation and severity of symptoms. Most of the time, emergency surgery is required for acute presentation, but a hiatal hernia may be picked up incidentally during routine or follow-up imaging for malignancy. Bona et al. reported 57.2% of cases requiring emergency repair, among whom 6.9% required bowel resection as well . The resClinical presentation varies from asymptomatic in incidentally detected patients to severe respiratory distress. Patients may also have abdominal complaints like dyspepsia, dysphagia, constipation, vomiting, and regurgitation. On chest auscultation, decreased air entry on the side of the hernia along with a fall in oxygen saturation aids in clinching the diagnosis, especially in emergency situations. On abdominal examination, there might be signs of peritonitis in cases of perforated viscera. Diagnosis of diaphragmatic hernia post-esophagectomy is clinically difficult and requires a high level of suspicion. A chest radiograph is the first investigation followed by CECT of the thorax and upper abdomen; this has good sensitivity and gives excellent anatomical details on the contents of the hernia and the viability of the contents, such as the bowel. Early CT should be done to avoid delay in diagnosis as there might be strangulation and perforation of bowel which are associated high risk of mortality, if not treated promptly. As per the Society of American Gastrointestinal and Endoscopic Surgeons, asymptomatic hiatal hernias can be conservatively managed depending on the patient’s age and comorbidities. However, all symptomatic patients should be offered surgical management . The preSurgical options include reduction of the contents and primary repair of the defect. Bowel resection may also be required in around 7% of such cases, and a primary anastomosis or stoma should be performed as per the clinical condition of the patient. There is very limited data available in the existing literature regarding whether the bowel should be primarily anastomosed or a two-stage procedure should be done . VallböhAnother novel technique is using biologic mesh for plug repair. Narayanan et al. described the technique of repairing the diaphragmatic hiatus with a biologic mesh plug without re-approximation of the crura. This was an effective method to prevent recurrences without compromising the gastric conduit . ComplicSignificant morbidity is associated with hiatal hernia repair post-esophagectomy, with a pooled prevalence of morbidity of 35% and in-hospital mortality rate of 5%. Associated complications are pulmonary 14.1%), cardiovascular (9%), and anastomotic leak (1%). These rates are especially higher in emergency patients. The recurrence rate after surgical repair is also high (16%) in these patients, and reoperation might also be required in 12% of patients [4.1%, carPost-esophagectomy hiatal hernia is a rare entity with presentation ranging from asymptomatic in the immediate post-operative period or a delayed acute emergency. Significant morbidity is associated with patients presenting as an emergency. Successful management in such cases depends on prompt detection, early surgery, proper post-operative care, and rehabilitation.


Liposarcomas are a group of malignancies that mainly affect adults. Myxoid pleomorphic liposarcoma (MPL) is a newly added subtype of liposarcomas . It is e Myxoid pleomorphic liposarcoma (MPL) is an extremely rare type of liposarcomas, which has a highly aggressive malignant pathology . It is cA 58-year-old female presented to the outpatient clinic with a 1-year history of vague abdominal pain associated with nausea and vomiting. She denied any changes in her bowel habits. Her past medical history includes Type 2 diabetes mellitus, dyslipidemia and hypertension. On examination, she had mild generalized abdominal tenderness without guarding or rebound tenderness. No enlargement in the lymph nodes. On laboratory analysis, a full blood work-up was done including complete blood count, electrolytes, liver function test and they were normal. The tumor markers CEA, CA19-9 and CA 125 were also normal.An ultrasound of the abdomen revealed a homogonous hypoechoic lesion anterior to the left hepatic lobe . Color dThe patient underwent exploratory laparotomy. The surgical exploration revealed a mass originating from the falciform ligament measuring around 6 × 6 cm that was soft in nature . There wHistologically, on initial evaluation, the resected mass was measured 7.0 × 6.5 × 5.0 cm and it was composed of fibrofatty tissue and peritonized surface. Microscopic examination demonstrated that the tumor is composed of cellular sheets of highly atypical pleomorphic cells. B The tumet al. [Liposarcoma is a type of malignancy that affects the fat tissue. According to World Health Organization (WHO), liposarcomas were initially classified into well-differentiated, dedifferentiated, pleomorphic and myxoid . Howeveret al. . It is det al. . It is eet al. . HoweverNone declared.None.


The benign nasopharyngeal teratoma is a benign and rare congenital disease, made up of the three embryological leaflets . It is of slow and progressive growth, pressing against adjacent structures without invading themThe goal of the present paper is to present the case of an adult patient with a nasopharyngeal teratoma, who was submitted to endoscopic surgery for exeresis.V.S., 35 years, female, presented unilateral nasal obstruction since childhood. Video-nasofibroscopy showed a pedicled oval-shaped mass in the pharyngeal ostium of the Eustachian Tube, obstruction about 60% of her nasopharynx. Paranasal sinuses CT scan presented a mass in her left nasopharynx .Figure 1The patient was submitted to endoscopic surgery under local anesthesia, with lesion exeresis after pedicle identification and resection . The tumThe patient is being followed up in our outpatient ward for 7 months now, without recurrence of the lesion or respiratory symptoms.Teratomas are benign, cystic, semicystic or solid tumors, which derive from the three embryonic leaflets. Its prevalence in the population is of 1 to 4.000 newborns and only 2 to 5% of the cases are located on the head and neck. Most of these lesions are diagnosed at birthDespite their controversial etiology, the most accepted theory is that these lesions stem from totipotent cells from the germinative layers of the embryo, and they grow in a potentially limited fashion and end up forming disorganized and disordered clustersSymptoms depend on lesion size and location. The most frequent symptoms include nasal obstruction, dyspnea and difficulty to breastfeed, it may also cause difficulty to swallow and vomit. Intermittent symptoms such as cyanosis, nasal obstruction and dysphagia may also be present when the pedicle is longDifferential diagnosis of nasopharynx obstruction in the newborn include also choanal atresia, intranasal glioma and encephalocele, rhabdomyosarcoma, dermoid cyst, lymphangioma, hemangioma and other neurofibromatosisCT scan and MRI help outline the lesion in order to plan for surgery, ruling out intracranial extensionIn the case hereby reported, CT scan and the endoscopic exam were enough to assess lesion origin and extension. Clinical signs and symptoms of unilateral nasal obstruction should raise suspicion about neoplastic processes. Surgery was carried out with the 30° rigid scope, and the entire lesion was removed from the pharyngeal ostium of the Eustachian tube.Although rare, teratomas may affect the nasopharynx. We stress the importance of a careful exam of patients complaining of unilateral nasal obstruction, especially newborns. Surgical treatment can be carried out through the endoscopic approach and complete lesion exeresis according to preoperative planning guided by image exams.


Mucoceles are benign lesions, covered by pseudostratified epithelium, that affect paranasal sinuses. Most of them occur in the frontal sinus (60%). Bilateral involvement is extremely rareA male patient aged 37 years with a history of cranioencephalic trauma 21 years ago, reports a left frontal tumor beginning 4 months ago. Magnetic resonance imaging revealed a lesion suggesting mucocele . The patMucoceles develop following obstruction of the drainage ostia, growing slowly within the sinuses, eventually eroding adjacent bone structuresMucoceles are mostly located in the frontal sinus, although bilateral involvement is extremely rareThe diagnosis is made based on the clinical history, physical examination and radiologic imaging. Symptoms vary in frontal ethmoid mucocele from absence of symptoms to incapacitating pain, headache and visual disturbancesComputed tomography shows an isodense homogeneous image, with loss of the normal sinus contour that is not enhanced with contrast if not infectedMagnetic resonance imaging is indicated if there is any doubt in diagnosis. The typical finding is a T1 hyposignal area T1 and a T2 hypersignal area, however any combination of signal intensities may be found depending on the presence of blood particles or the degree of hydration of the contentCurrently ample marsupialization of the mucocele and simple drainage of the sinus by endoscope has been done with excellent surgical results. The advantages include low morbidity, low risk of complication and a rare recurrence rateEndoscopic treatment seems to be the best treatment option. However, for technical reasons anatomical variants may preclude ample marsupialization done only endoscopically.


Spontaneous gastric intramural hematoma is a rare disease. Herein, we present a case of a previously healthy 28-year-old male patient who presented with diarrhea and diffuse abdominal pain of one-week duration. The patient was diagnosed with spontaneous gastric intramural hematoma post urgent partial gastrectomy for a bleeding gastric tumor. Six other cases of spontaneous gastric intramural hematoma are published in the literature; therefore, when encountering a case of intra-abdominal mass attached to the gastric wall, gastric intramural hematoma should be considered in the differential even when no cause is present. Gastric intramural hematoma (GIH) is a very rare non-cancerous lesion. Most gastrointestinal intramural hematoma described in the literature are found in the esophagus or duodenum ,2. WhileThe rarity of this disease especially with the absence of a trigger can make the diagnosis of spontaneous gastric intramural hematoma (SGIH) very challenging. To our knowledge, only six cases of SGIH are reported in the literature, of which four were managed surgically and two conservatively -9. HereiA 28-year-old male patient, previously healthy, presented to our institution for severe abdominal pain of one-day duration. The history goes back to one week prior to presentation when he started to develop watery diarrhea associated with mild diffuse crampy abdominal pain, nausea, one episode of non-bilious vomiting, one episode of subjective fever, chills, night sweats, decrease in appetite, unintentional weight loss of 9 kgs in five months, but no melena or hematochezia. The patient self-diagnosed himself with gastroenteritis and took rifaximin for three days followed by metronidazole for another three days but symptoms didn’t resolve. One day prior to presentation, the patient developed severe abdominal pain that was diffuse, cramping in nature, constant, 10/10 in intensity, radiating to his back and shoulders, exacerbated by movement, and with no relieving factors. He also developed several episodes of presyncope described as lightheadedness, blackout, and diaphoresis with a documented systolic blood pressure reaching as low as 85mmHg so he presented to the emergency room (ER) in a peripheral hospital for evaluation. To note, the patient takes no medications or illicit drugs, has no allergies, is a social smoker, drinks alcohol occasionally, has a negative family medical history, and has previously undergone a right inguinal lymph node dissection about three years previously that turned out to be reactive in nature with no signs of malignancy. In the peripheral hospital, the patient was hydrated and laboratory tests were unremarkable except for a white blood count of 18.6 x 10^3/ul with neutrophils of 88%. Computed tomography (CT) scan of the abdomen and pelvis with IV contrast Figure  demonstrIn our ER, his vitals were stable. During the physical exam, he was pale, had abdominal guarding, severe diffuse abdominal tenderness, and mild distension. Laboratory tests showed no pertinent changes in values except for a noted hemoglobin drop from 14 g/dl to 11.2g/ dl. The patient was hydrated, started on IV antibiotics, and was admitted to the facility. Gastroscopy Figures -2b showeAn urgent CT scan of the abdomen and pelvis with IV contrast was done Figure  that shoOn laparotomy, two liters of blood were aspirated from the abdomen and a bleeding gangrenous gastric mass was identified in the body and antrum of the greater curvature Figure  in contaThe stomach is an uncommon location for gastrointestinal intraluminal hematoma but when present it is usually in patients who take anticoagulants, have gastric pathologies, experienced endoscopy, or rare cases, spontaneously . The rarEven though GIH is mostly asymptomatic, complaints such as nausea, blood-stained vomit, or epigastric pain can be present; moreover, hematomas can impact the osmotic gradient within the gastrointestinal tract wall which can lead to hemorrhagic effusion into the peritoneal cavity . The chiCT scan is considered the most specific and sensitive in diagnosing intramural hematomas . The hemCT angiography can also be used in the diagnosis of GIH since it can detect active hemorrhage or the presence of a visceral arterial aneurysm in the vicinity of the stomach. In case of an active hemorrhage, angiography can also facilitate the initiation of treatment by embolization of the damaged vessels . In the Gastroscopy can also play a role in the investigation of GIH. It can show a gastric luminal narrowing with or without submucosal bright-red or dark-red mass and rarely with the presence of active mucosal bleeding . In the EUS can help in the diagnosis of GIH. Indeed, it can facilitate the determination of the wall thickness from which the lesion originates and assess its echostructure and the thickness of its infiltration. Moreover, it permits the physician to perform thin-needle or core-needle biopsy to collect the material for cytological and histopathological analyses . EUS witThere is still no agreed modality for the treatment of SGIH; however, GIHs are considered in general self-restrained and the majority of them are reabsorbed within two to three weeks ,3. In GIIn case of active bleeding or a trend toward enlargement, transcatheter arterial embolization (TAE) may be indicated ; howeverIn general, surgical management of GIH is recommended where there is unclear diagnosis, suspected complications, hematoma involving a large part of the gastric wall, ongoing bleeding, failure of minimally invasive therapy, or when one cannot differentiate it from malignant gastric tumors; moreover, the decision to operate emergently and kind of surgery is mainly driven by the clinical scenario ,12. In fIt is noted that in the majority of the cases of SGIH and in all the cases that were treated surgically, the working diagnosis, whether preoperatively or intraoperatively, was gastric submucosal mass. Nevertheless, because of the dilemma in the diagnosis, these patients may have received unnecessary gastrectomies or aggressive surgical procedures (as in mesocolon resection in our case) for a benign pathology that was identified postoperatively in all the cases. In the reported cases, the differential diagnosis was broad including  visceral artery aneurysm, compression from extrinsic mass, gastric varices, pancreatic pseudocyst, and others. For instance, only one case suspected GIH and it was treated conservatively . Since oSGIH is a very rare disease. When suspected in a patient presenting with an intra-abdominal mass attached to the gastric wall, GIH should be taken into consideration and included in the differential. As our review showed, this is true even in young and healthy patients with no cause for the disease. Diagnosing SGIH is of great importance, since it is a benign etiology that can be treated conservatively avoiding unnecessary aggressive surgical procedures.


Caecal volvulus is an uncommon surgical condition affecting mostly females in their second and third decade of life. It is of vital importance that the general surgeon recognises, resuscitates, diagnoses, and effectively treats these cases in a timely manner to maximise the chance of a positive outcome for the patient. Whilst there are several types of caecal volvulus, the treatment involves, in most cases, surgical intervention. There is a wide variety of surgical interventions that can be performed, ranging from caecopexy or fixation to lateral wall to performing a right hemicolectomy with primary ileocolic anastomosis. There are several factors that influence this decision and can also be based on an individual surgeon's expertise and experience. We present a case of a 21-year-old female who presented to our Emergency Department with lower abdominal pain, nausea, and vomiting. She was diagnosed with caecal volvulus with the aid of CT imaging, following which she underwent laparotomy in which caecal volvulus was noted. She underwent appendicectomy and caecopexy and was discharged after an uneventful recovery on post-operative day five and remains well on follow-up. Caecal volvulus accounts for 25-40% of all cases of colonic volvulus . It is dWe report a case of a 21-year-old female presenting to the Emergency Department with a one-day history of lower abdominal pain, associated with nausea and vomiting. On examination, she was diffusely tender across the lower abdomen, predominantly in the right lower quadrant. Laboratory blood tests revealed elevated white cell count and C-reactive protein (CRP). Of note, this young lady had no significant past medical or surgical history, was not on any medications, and had no family history of bowel pathology. Upon presentation, she further underwent radiological imaging in the form of a plain film of abdomen (PFA) followed by a CT of the abdomen and pelvis, which suggested a distended loop of bowel, most likely caecum, to the left of the midline with volvulus (Figure Following adequate initial resuscitation with intravenous fluids, administration of analgesia, and antibiotics, she was taken to the operating theatre for surgical management.A midline laparotomy was performed. Intraoperatively, a caecal volvulus was noted, in the form of a highly mobile caecum twisted by more than 180 degrees around its axis Figure . This waCaecal volvulus is a rare clinical entity that occurs when the segment of bowel gets entangled or encircled upon a mesenteric axis . Caecal While this pathology is multifactorial, incomplete caecal volvulus development, anatomical variations, malrotation or partial intestinal rotation during developmental embryogenesis are considered to be major contributing factors . CongeniThe most well-theoreticized cause of caecal volvulus is related to embryogenesis. This is characterised by a highly mobile caecum and ascending colon being fixed only partially .Surgical intervention is the definitive form of treatment for this condition. The type of operation depends on the duration of onset of symptoms, past medical or surgical history, overall functional status, and appearance of the bowel at laparoscopy or laparotomy. If there is evidence of intestinal gangrene or viability is uncertain, resection is unavoidable.If the bowel segment is viable, then it may suffice to untwist the affected segment and perform a caecopexy to the lateral wall as done in our case. This could also be done via laparoscopy if an experienced surgeon is present. Alternatively, a caecostomy can be performed. A laparoscopic procedure may be associated with decreased post-operative pain, leading to quicker recovery, and decreased length of stay in the hospital . HoweverOur case highlights the importance of recognition, resuscitation, diagnosis, and treatment of caecal volvulus in a timely fashion. This is best assessed through an adequate and detailed history and complete clinical examination. Any patient with a high index of clinical suspicion of caecal volvulus must be worked up with routine blood tests and relevant imaging as deemed necessary based on the status and history of the patient. Accordingly, surgical management should be undertaken as discussed above depending on patient pre-operative and intra-operative findings and surgeon experience.In our opinion, due to the wide range of surgical options for treatment, as discussed previously, an individualised approach should be opted for to ensure that management is tailored according to each individual case. In any case, the patient and their family must be advised of the risk of recurrence and should be counselled about the possible surgical options available. This case also highlights the importance of an experienced radiologist in aiding the general surgeon with diagnosis and an eventual satisfactory outcome for the patient.


Asymptomatic presentation is common in benign mature mediastinal tumours. Symptoms of the above diseases are sometimes life-threatening and can cause massive hemoptysis, recurrent pulmonary infection, hypoxia related to the pulmonary parenchymal hemorrhage, or pressure effect on or more of the major bronchi. A 16-year-old boy presented with frequent episodes of hemoptysis and recurrent fever unresponsive to antimicrobials. On investigation, it was found to be a benign mature mediastinal mass with cystobronchial connection to the right middle lobe. Resection of this mass resulted in the complete recovery of the individual. An anterior mediastinal benign teratodermoid tumour with intraparenchymal extension through cystobronchial connection is very rare. Surgical resection is challenging but offers the complete cure. Only 15% of the anterior mediastinal mass is a germ cell tumour. The typical presenting age is the second and fourth decade. Nonseminomatous tumour is more common than the seminomatous tumour in the anterior mediastinum ,3. We arA 16-year-old non-smoker male presented to the ED with cough and frequent episodes of hemoptysis. On clinical examination, the patient had a respiratory rate of 18/min, oxygen saturation (SpO2) of 98% in room air, and normal bilateral vesicular breath sound with no added sound. After initial resuscitation, hemoptysis was controlled.On chest X-ray (CXR), ill-defined opacity in the right hilar and para hilar region was seen Figure . CT scanThe patient was taken for surgery, and after sternotomy, mass was dissected from the adjacent right atrium and aorta. Mass was resected, cystobronchial communication was identified, and right middle lobectomy was done after dissection and ligation of hilar structures Figure .Post-surgery, the patient was extubated after six hours. In post-operative CXR, there was no pleural or mediastinal collection or lung atelectasis. Intercostal drain (ICD) was removed after three days. The total duration of hospital days was seven days, including three days in the critical care unit.A histological examination of the mediastinal mass shows a cyst lined by the respiratory and intestinal epithelium. The stroma shows the presence of smooth muscle, sebaceous gland, lymphoid aggregates, and fibrous tissue. Section examined from the lung shows expanded alveolar spaces filled with foamy macrophages, indicating lipoid pneumonia due to obstruction Figure . The patient was asymptomatic during a three-month follow-up and without any recurrent lesion on CXR Figure .The most common extragonadal site of germ cell tumour is the anterior mediastinum . The mosAs the most common presentation is asymptomatic, most cases are diagnosed during CXR and CT scans. Chest tightness, dyspnea, neck mass, superior mediastinal syndrome, and Horner syndrome may be presenting symptoms due to compression. The patient may present with pleural effusion and hemothorax, which cause dyspnea, hemoptysis, obstructive pneumonia, and pericardial effusion and tamponade. On CXR and CT scan, partial lobulated and calcified round mass is present in the anterior mediastinum. Tooth and bone can be found in the tumour. Mature teratomas are mostly cystic masses, while immature teratomas are primarily solid masses. CT is the primary imaging modality to diagnose bronchial tree fistulas and delineate possible causes like an abscess, pneumonia, and tumour. In the case of hemoptysis, CTA is useful to identify vessels, and in case of emergency, embolization can be done. AFP, β-HCG, and LDH are used to diagnose and monitor cases .Surgical resection is an effective treatment in benign mature teratoma. However, due to extensive adhesion, large wound surface, large size, incomplete lung expansion, and residual cavity in the chest cavity, dissection is challenging, and postoperative bleeding chances are higher. During dissection, adequate care should be given to protect the phrenic nerve, recurrent laryngeal nerve, vagus nerve, and brachial plexus nerve in case of large adherent mass. If the middle mediastinum, innominate vein, superior vena cava, head and neck vessels are involved, it is advisable to choose median sternotomy for surgical resection when vascular reconstruction is needed. When the tumour extends to neck through the thoracic outlet, a “T” shaped incision should be made through the median sternum combined with the neck collar incision. A posterolateral thoracotomy may be preferred when wedge-shaped resection of the lung, lobectomy, or pneumonectomy is needed. For large tumour, lung, and pericardial invasion due to the perforation of the surrounding tissues or dense adhesion, partial pericardiectomy, pulmonary lobectomy, and wedge-shaped excision of the lung are done . ThoracoAnterior mediastinal benign teratodermoid tumour with intraparenchymal extension through cystobronchial connection is very rare. CXR and CT thorax are used to diagnose it. Surgical resection is complicated, but it offers the complete cure. Blood LDH, β-hCG, AFP are used to monitor the response to therapy. Histopathological diagnosis is confirmatory.


Malignant peripheral nerve sheath is a rare neoplasm seen in humans, with a very small percentage of occurrence in the cervical region. It is an aggressive form of sarcoma, usually arising from peripheral nerves. In this case study, we present a 61-year-old male who was reported to the hospital with a chief complaint of swelling on the right side of his neck. Magnetic resonance imaging (MRI) revealed a heterogeneous mass in the cervical region, which is reported as a schwannoma. The patient was posted for surgery for tumor excision. The patient was managed with analgesics, physical therapy, and wound care. The patient's symptoms were relieved, and he was discharged. In conclusion, combined surgical resection and adjuvant treatments like physical and medical therapy improved the functional outcome. However, this literature does not address radiation therapy. Malignant peripheral nerve sheath tumor (MPNST) is a connective tissue tumor, and because of its appearance and origin, it falls under the category of sarcomas. The most common presentation includes numbness, dizziness, and edema, with or without pain in the affected limb. It may also reduce joint mobility. Although the pathophysiology of MPNST is unclear, it has a strong genetic association due to the high prevalence in both the first and second-degree relatives of the patient. ,2. AboutA 61-year-old male reported to the outpatient department with a complaint of swelling in the right side of the neck for the past six months, which was painful during neck movements. On examination, there was a 6x5 cm firm swelling on the right side of the neck with no local rise in temperature and no overlying skin changes. The swelling was non-tender and non-mobile, with no other palpable cervical lymph nodes. The swelling was fixed to the underlying tissue and the skin. It was without any discharge or bleeding  revealed a heterogeneously enhancing lesion with areas of necrosis in the deep soft tissue plane of the right supraclavicular region. It measured 6.2 x 5.2 x 4.5 cm in size and was heterogeneously hyperintense on T2WI film and hypointense on T1WI film. Superiorly, the mass lesion was seen posterior to the sternocleidomastoid muscle, causing its anteromedial displacement. Inferiorly, the lower pole of the lesion was seen posterior to the clavicle. The fat planes with surrounding structures were maintained. The tumor mass was vascular with no capsular invasion. This was suggestive of a soft tissue tumor, with differentials being schwannoma, neurofibroma, leiomyosarcoma, and a dermoid cyst Figure .Fine-needle aspiration cytology (FNAC) was done, and the patient was diagnosed as having a malignant peripheral nerve sheath tumor. Surgical excision of the mass was planned. The patient had no comorbid conditions, was an American Society of Anesthesiologists (ASA) class 1 patient, and was fit for surgery. The patient was positioned under all aseptic precautions, with parts painted and draped. An elliptical incision was made over the right-sided neck over the Langer line (previous scar site included), measuring 4 cm. Incisions were made, and the platysma was divided. The neck mass was identified and found to be adherent to surrounding structures. A blunt dissection was done surrounding the mass. The base of the mass was separated with bipolar cautery. Proper hemostasis was achieved. Drain No. 14 was placed, and the incision was closed in layers. A sterile dressing was applied, and the procedure was declared uneventful Figures .Postoperatively, the patient was extubated and shifted to postoperative recovery with no intraoperative complications. The patient was managed with analgesics, daily dressings, and daily physiotherapy. The patient was discharged after seven days of surgery, with the advice to follow up in the OPD next week for complete suture removal. On follow-up, the patient was hemodynamically stable with no fresh complaints. The histopathology report was suggestive of a schwannoma.Table Malignant peripheral nerve sheath tumor (MPNST) is a recurrent mutation of polycomb repressive complex 2 (PRC 2), embryonic ectodermal proteins (EED), and suppressor of zest 12 homolog (SUZ 12). This mutation causes altered chromatin, which promotes MPNST malignancy . The occChemotherapy as an adjuvant has been opted for in cases with tumor sizes greater than 5 cm and high-grade MPNST, irrespective of the tumor site. However, recent studies have shown that patients who opt for chemotherapy without surgery have the worst prognosis and lower survival rates; the same results have been cited in many other studies ,12. In lIn this case study, we expect a positive outcome compared to the available literature as the surgical margins were negative and we opted for daily physical therapy for functional improvement in the shoulder and respiratory function of the patient. Even though we do not have a five-year post-treatment result, the surgical approach and the adjuvant therapy will have promising results.Malignant nerve sheath tumors are uncommon, particularly those affecting cranial nerves. In our case, there was no involvement of the capsule and surrounding structures; hence, it was easy to remove, and a pre-operative FNAC guided the treatment plan. A thorough neurological examination also aids in this. Suspicion of neurofibromatosis should be strong in cases with malignant peripheral nerve sheath tumors. As this remains a very rare neoplasm, the negative surgical margins and adjuvant physical therapy have improved the condition and relieved symptoms in our patient. A close postoperative follow-up is mandatory to eliminate recurrence. The available literature has not portrayed the impact of chemotherapy and radiotherapy on the condition. This treatment strategy has improved the functional status in our case. A protocol needs to be established for managing these tumors.


Classical infratentorial superficial siderosis (iSS) is characterised by repeated insidious bleeding into the subarachnoid space, leading to haemosiderin deposition within the subpial layers of the brainstem, cerebellum and spinal cord, sometimes with supratentorial involvement. Although nearly always associated with a dural defect  there is little evidence to support definitive investigation and management strategies. Here, we present a novel investigation strategy to identify a dural defect and subsequent successful surgical repair with biochemical resolution of subarachnoid bleeding.A 55-year-old gentleman presented with a 15-year progressive history of sensorineural deafness, followed by a slowly worsening gait ataxia. He had previously sustained cranio-spinal trauma. On examination there were features of myelopathy and ataxia. MRI demonstrated classical iSS, affecting cerebellum and cerebral cortices, with a cervicothoracic epidural CSF collection. Lumbar puncture (LP) revealed elevated ferritin 413 ng/mL and red cell count of 4160. Reverse CT myelography, a novel technique involving contrast injection into the collection, delineated a dural defect at the T9/T10 level that was not present on conventional myelography. Following surgical repair, repeat LP twelve months later demonstrated biochemical improvement . There was no further neurological deterioration in symptoms during eighteen months follow-up.We show the value of a rational targeted investigation pathway in identifying a surgically reparable dural defect underlying classical iSS. We also provide proof of concept that surgical repair can facilitate biochemical resolution of subarachnoid bleeding and might prevent progression of neurological disability.The online version contains supplementary material available at 10.1007/s10072-022-06181-x. Classical infratentorial superficial siderosis (iSS) is characterised by haemosiderin deposition in the pial layers of the central nervous system, hypothesised causes include, repetitive or sustained low volume bleeding into the subarachnoid space , 2. WhilThe most common cause of iSS, hypothesised to be due to subarachnoid bleeding from a dural defect , was refHere, we present a case of a classical iSS due to a thoracic dural defect identified by the novel use of reverse dynamic CT myelography; after surgical repair, there was subsequent biochemical improvement of bleeding biomarkers in the CSF with stabilisation of neurological symptoms. This case report builds upon a limited evidence base for thoracic dural repair in classical iSS.A 55-year-old male builder presented with a 15-year history of progressive bilateral sensorineural hearing loss and a 4-year history of gait ataxia. His background included mild head trauma from a cycling accident at age twelve and a severe blunt-force injury to the thoracic spine 8 years prior to presentation. His injuries at the time did not mandate imaging and he remained asymptomatic for several years. Patient consent for this case report was obtained. He had brisk reflexes throughout with down going plantars and marked gait ataxia. Sensory examination was intact.MRI demonstrated extensive siderosis affecting both cerebral and cerebellar hemispheres. The retro-odontoid venous plexus was prominent below C1, suggestive of a CSF leak. No vascular abnormality was identified. Lumbar puncture revealed a CSF ferritin of 413 ng/mL and a red cell count of 4160. MRI spine with contrast demonstrated a ventral cervicothoracic epidural collection Fig. . StandarThis case was reviewed at the siderosis multidisciplinary team meeting with a decision to repair the dural leak given the marked continued clinical progression and biochemical evidence of ongoing bleeding into the subarachnoid space. He underwent a T8-10 laminectomy with intradural repair of ventral T9-10 dural defect. Intra-operatively, the leak was identified secondary to prolapse of arachnoid through a ventral dural defect between the right T9 and T10 exiting nerve roots. Post-operative imaging performed at 6 months demonstrated resolution of the epidural collection  iSS, we recommend reverse spinal CT myelography if conventional imaging fails to identify a dural defect. We suggest that prompt surgical repair might prevent neurological progression. Further research is needed to better establish the risks and benefits of this clinical approach.Supplementary file1 (MP4 86694 KB)Below is the link to the electronic supplementary material.