Case Presentation. This report presents a 31-year-old otherwise healthy woman with a right hip pain. Clinical and histopathological investigations revealed the diagnosis of diffuse-type tenosynovial giant cell tumor of the hip (the diffuse intra-articular form of PVNS). Open synovectomy and tumor resection and surgical dislocation of the hip were performed. She was free of symptoms and recurrence within a 9-year-follow-up period.  Tenosynovial giant cell tumors are a benign but rare condition with potentially aggressive tumor-like traits which should be considered in young patients with monoarticular joint involvement.  Open synovectomy and tumor resection through surgical dislocation of the hip without adjutant radiotherapy could be a reliable choice for the localization of the hip. Tenosynovial giant cell tumor (TGCT) is a locally aggressive mesenchymal entity previously known as pigmented villonodular synovitis (PVNS) which is a rare condition affecting small and large joints' synovium, tendon sheaths, and bursa of unknown etiology with a yearly incidence of 1.8 per million, generally seen in the third to fifth decade of life  with a sThe primary treatment option is open total tumor resection and synovectomy to preserve joint function, which rarely needs revision surgery with an average time to revision of 6.5 years in previous cases , 16, 17.A 31-year-old otherwise healthy civil engineer female referred to our orthopedic clinic with a complaint of an atraumatic right hip pain, inability to bear weight, and a limited range of motion for one month following a long walking journey outdoors in nature. The pain was dominant at night, exacerbating with pressure or movement. The patient past medical history was negative for any condition except taking Rakuten tablets for acne in several periods. She had received conservative treatments during the recent one month, and even a rheumatologic and infectious condition had been ruled out but no improvement of symptoms occurred.On the physical examination, the hip range of motion in all directions was limited beside a local tenderness in the hip but without swelling or erythema.She had received conservative treatments but no improvement of symptoms occurred. Laboratory blood tests were normal. Initial pelvic radiographs showed joint space involvement and cortical erosion in the right acetabulum and femoral head .MRI of the hip showed characteristic low-signal intensity synovial involvement because of high hemosiderin content . On Tc-9The diagnosis of diffuse-type giant cell tumor was made and the open surgical synovectomy was planned. We conducted surgical dislocation of the joint using the technique described by Ganz et al.  under spThe patient was discharged the next day after surgery with good general condition. She was ordered to come for follow-up visit after 3 and 6 months and then annually with control MRI which all showed no recurrence of the lesion ; a latesTGCT is an infrequent, benign but proliferative disease of the synovium of especially large joints, occurring about 15% in the hip joint. Though an inflammatory or neoplastic origin has been proposed, the exact etiology is still under debate. It is categorized as a localized form or nodular tenosynovitis, diffuse-type giant cell tumor, and PVNS. The diffuse-type tenosynovial giant cell tumor could be either purely extra-articular, located in the soft tissues, or representing the extra-articular component of a PVNS; the histopathologic picture of these three lesions is rather alike consisting of elongated synovial villi and synoviocyte line besides sheets of mononuclear cells intermingled with osteoclast-like multinucleated giant and siderophage and xanthoma cells , 23; hemWhile the nonappearance of juxta-articular osteophytes and osteopenia is typical in TGCT , dense sFor diffuse-type TGCT of the hip, early entire tumor resection and synovium are needed to limit articular destruction and the potential recurrence. The primary treatment options include tumor resection and synovectomy alone and synovectomy plus arthroplasty, which consists of total hip arthroplasty, hemiarthroplasty, cup arthroplasty, and hip resurfacing. Even if total synovectomy seems to be effective to prevent recurrence, according to Vastel et al., it does not postpone the development of secondary osteoarthritis of the hip following synovial disturbance, necessitating a total hip replacement procedure , 16.Excision is sufficient for local PVNS through arthroscopy or arthrotomy; however, if the lesion is diffuse, total synovectomy is compulsory , 27, 28.In our case, open synovectomy with a Ganz surgical dislocation of the hip was performed because it was essential to excise total synovium and for clearing of the acetabular fossa. As no recurrence occurred in the 9-year follow-up, we had no need for surgical revision. Adjuvant therapy was not conducted as well, as it was not necessary and to avoid unwanted adverse effects on adjacent reproductive organs.A provocative onset of hip pain could be a clinical presentation for diffuse-type TGCT, so physicians should consider such condition.Open synovectomy and resection of tumor through surgical hip dislocation without adjuvant radiotherapy could serve as reliable techniques to clear the joint space from pathologic synovium, with no chance of recurrence, as reported in the current case. However, further trail studies seem to be conducted to investigate the outcome of this kind of treatment approach in the condition.


A 48-year-old right-hand-dominant man presented to the clinic with a history of a right index finger mass present for 2 months. He denied any trauma, drainage, or pain. On examination, the dorsoradial aspect of the proximal phalanx of the right index finger demonstrated a single, spherical, soft, nontender, freely mobile swelling that was approximately 1.5 cm long. There was no redness, punctum, or previous scars, and finger extension did not change mobility or position. The patient underwent an ultrasound scan that showed a solid vascular nodule adjacent to a vein, and he subsequently underwent excision. The mass appeared venous and had 3 contiguous vessels. Histology demonstrated a dilated blood vessel with a semiattached thrombus showing fibroblast proliferation, hemosiderin deposition, and focal pseudopapillary projection and neovascularization, consistent with Masson's tumor. The patient had an uneventful postoperative course and signed a release of information and photograph consent. This case report was deemed institutional review board exempt.What is Masson's tumor?How does Masson's tumor present?What are the clinical and histological features?What are the treatment and prognosis?The first description of “vegetant intravascular hemagioendothelioma,” now known as intravascular papillary endothelial hyperplasia (IPEH), was given by MassonMasson's tumor presents as a nonspecific, well-demarcated, slow-growing soft-tissue mass that most commonly manifests in the third and fourth decades of life. Clinically, Masson's tumor presents as a small 0.5-5 cm), well-defined, red or purple nodule or papule that may be located deep or superficially. These lesions tend to arise from the skin or subcutaneous tissues, but there are reports of tumors arising from the lingual mucosa, heart, and small bowel.-5 cm, weVariations in IPEH histology lend itself to classification into 3 types. The primary form usually arises in the soft tissues of the hand, digits, or head and neck, oftentimes in a dilated vein. Secondary, or “mixed tumors,” arise in the presence of preexisting vascular abnormalities. The third type, extravascular IPEHs, arise in hematomas and are least common. On microscopic examination, these consist of characteristic dilated vascular channels, a fibrous connective tissue core, a monolayer of plump endothelial cells, and papillae. Tumors are generally positive for CD31, CD34, SMA, and factor VIII-related antigen. CD105 is positive on vascular endothelial cells and primary vascular neoplasms, which help differentiate IPEHs from angiosarcomas.Treatment of Masson's tumor of the skin and soft tissue consists of surgical excision. The lesion is typically well-defined from the surrounding tissue and does not require additional surgical margins.Our patient presented with a nontender right index finger mass. The clinical appearance and histology supported the diagnosis of Masson's tumor. Our patient had a primary lesion, and he underwent curative surgical resection. He has not had a recurrence.


Small-intestinal neuroendocrine tumors (siNETs) account for 25% of gastroenteropancreatic NETs. A total of 89% of multiple tumors are located in the ileum, often within 100 cm of the ileocecal valve. According to current guidelines, all localized siNETs should be considered for radical resection with lymphadenectomy. The preoperative workout should focus on symptoms of carcinoid syndrome . Morphological evaluation should include a CT scan with a thin-slice arterial CT, a PET/CT with 68 Ga, and a hepatic MRI. Levels of 24 h urinary 5-hydroxyindoleacetic acid are needed. Regarding surgery, the limiting component is the number of free jejunal branches allowing a resection without risk of short small bowel syndrome. In case of emergency surgery, there is expert agreement that it is not reasonable to initiate resection of the mesenteric mass without comprehensive workup and mapping. The challenge lies in the ability to propose a resection without imposing short small bowel syndrome on the patients.Introduction: Small-intestinal neuroendocrine tumors (siNETs) account for 25% of gastroenteropancreatic NETs. Multiple siNETs appear to develop in a limited segment of the small bowel (SB), 89% of them being located in the ileum, most often within 100 cm of the ileocecal valve (ICV). According to the European Neuroendocrine Tumor Society (ENETS) and the American Joint Committee on Cancer (AJCC), all localized siNETs should be considered for radical surgical resection with adequate lymphadenectomy irrespective of the absence of lymphadenopathy or mesenteric involvement. Surgical management of siNETs: The preoperative workout should include a precise evaluation of past medical and surgical history, focusing on the symptoms of carcinoid syndrome . Morphological evaluation should include a CT scan including a thin-slice arterial CT, a PET/CT with 68 Ga, and a hepatic MRI in cases of suspected metastasis. Levels of 24 h urinary 5-hydroxyindoleacetic acid are needed. Regarding surgery, the limiting component is the number of free jejunal branches allowing a resection without risk of short small bowel syndrome. The laparoscopic approach has been poorly studied, and open laparotomy remains the gold standard to explore the abdominal cavity and entirely palpate the small bowel through bidigital palpation and compression. An extensive lymphadenectomy is required. A prophylactic cholecystectomy should be performed. In case of emergency surgery, current recommendations are not definitive. However, there is expert agreement that it is not reasonable to initiate resection of the mesenteric mass without comprehensive workup and mapping. Conclusion: The surgery of siNETs is in constant evolution. The challenge lies in the ability to propose a resection without imposing short small bowel syndrome on the patients. The oncological benefits supported in the literature led to recent changes in the recommendations of academic societies. The next steps remain the dissemination of reproducible quality criteria to perform these procedures. Small-intestinal neuroendocrine tumors (siNETs) account for 25% of gastroenteropancreatic NETs , but theAccording to the ENETS consensus guidelines , the prePatients with carcinoid syndrome received a continuous infusion of octreotide (2000 µg/day) at least 12 h before surgery, during, and at least 24 h after surgery in order to prevent perioperative carcinoid syndrome . OctreotFor several years, the impact of centralization in digestive surgery on postoperative outcomes and the centralization of activity in expert centers has been debated regarding all areas of this surgical specialty, as well as in other specialties. The vast majority of these studies showed a reduction in postoperative complications and length of stay, regardless of the severity of the pathology, the type of surgery, or the patients’ comorbidities ,15,16,17The laparoscopic approach has been poorly studied. In most published studies, laparotomy is the reference approach. It allows for a much more precise evaluation of the peritoneal and mesenteric involvement if necessary; it allows for a better control of the origin of the mesenteric vessels in case of extensive nodal resection ,18. SimiAs a first step, the abdominal cavity is explored, and the full length of the small bowel is analyzed visually and through bidigital palpation and compression. Any suspected tumors are tagged with a polypropylene suture. The distance from the most proximally suspected tumor to the ileocecal valve is noted in order to predict the future length of the residual small bowel.Then, visual searches for miliary liver metastases are systematically performed, as well as perioperative ultrasounds and liver biopsies. Liver ultrasonography is always necessary to define the diagnosis of liver involvement. Peritoneal metastases are searched, and finally, the ovaries are examined to detect invasion.p = 0.79) or mortality  [Somatostatin analogs represent a main part of the postoperative (in case of R1 and R2 resection) oncological treatment. Those molecules are known to expose patients to the risk of gallbladder stone formation. Prophylactic cholecystectomy can be safely performed without major increase in postoperative morbidity in cholecystectomy vs. no cholecystectomy groups  . To prev = 0.29) .To date, five teams have reported positive oncological results arguing for extensive lymph node dissection .p = 0.01). In addition, patients with more extensive lymph node resection (≥8 nodes) had different survivals based on the number of nodes involved  [Landry et al. described a threshold of seven harvested nodes to have a positive impact on specific survival. The main limitation of this study was that 30% had no lymph nodes analyzed on the surgical specimen . Pasquer= 0.047) . Finally= 0.047) . The definition of an extensive lymph node dissection is still debated. However, complications related to the mesenteric mass appear when the compression is proximal, either by further progression or by lymph node recurrence. Three lymph node groups have been described: group 1 in contact with the small bowel, group 2 in the middle of the mesentery, and group 3 at the origin of the mesenteric vessels under the pancreatic uncus. In the cohort of Pasquer et al. , the pheThe resection starts with dissection of the mesenteric vessels to determine whether the mesenteric nodal block is resectable. Then, the retroperitoneum is opened, followed by a Kocher maneuver to expose the proximal part of the superior mesenteric artery. This strategy allows progressive ligation of vascular branches without any threat to uninvaded jejunal arteries. Lymphadenectomy is performed above the right colic vessels if the nodal mass is located on the right part of the mesenteric axis. If the nodal mass only implies the left side of mesenteric artery, the right superior colic artery can be preserved if the mass is located below its origin. Right hemicolectomy is not mandatory in all patients. Next, the dissection is pursued on the left border of the superior mesenteric vessels. A minimum of three jejunal branches have to be free to avoid the risk of short bowel length ,13. ThenTo date, there are no recommendations concerning the content of the operative report. Nevertheless, we have standardized it and propose the following content.Concerning the clinical history, it must be complete, associating all the preoperative morphological and biological workup, including the stability of the carcinoid syndrome and the preoperative preparation with octreotide.-At the hepatic level: the presence of a miliary, the location of lesions, the realization of biopsy, and ultrasonography report.-Mapping of the carcinosis.-Description of ovarian involvement.-The number of tumors, their location in relation to the mesenteric axis.-Classification of the mesenteric mass as resectable type 1 to 4.-Measurements between the Treitz angle and the first lesion, the resected length and the length downstream to the ileocecal valve.-The need for a right colectomy.Concerning the procedure, the exploration phase must be meticulously described, including:Concerning anesthetic management, it is important to specify the hemodynamic stability and the presence of flushing during the operation .A systematic bilateral oophorectomy in case of clinical complaint (such as heaviness or pelvic pain) or carcinoid syndrome and in the presence of ovarian metastases  should bOcclusive syndrome on retractile mesenteritis or mesenteric ischemia constitutes the main emergencies secondary to a complication of siNETs by local invasion. These patients are sometimes diagnosed at the stage of the complication. Current recommendations are not definitive. However, there is expert agreement that it is not reasonable to initiate resection of the mesenteric mass without comprehensive workup and mapping. It would then seem appropriate to treat the complication if there are signs of severity. It is quite possible to medically treat venous ischemia without necrosis before transferring the patient to an expert center. However, in case of occlusion with signs of severity  or acute arterial ischemia, an intervention to overcome this episode should be proposed. Due to the difficulties of exposition induced by the dilation of the bowel, the risk of missing a tumoral lesion is high, as well as the risk of hemorrhage. Mesenteritis makes dissection difficult, and dilatation of the bowel makes the vascular division even more difficult. Surgery in context of emergency will consist of a resection of the ischemic segments or a simple offloading stoma upstream of the mesenteric mass before transferring the patient to an expert center . It appeThe surgery of siNETs is in constant evolution. Recent studies plead for resection of primary lesions even in metastatic condition in order to preserve patients from the risk of occlusion or mesenteric ischemia. The challenge lies in the ability to propose a resection without imposing short small bowel syndrome on the patients, which is synonymous with quasi-definitive parenteral nutrition as a complement. The oncological benefits supported in the literature led to recent changes in the recommendations of academic societies. The next steps are the dissemination of reproducible quality criteria as described in this manuscript, as well as the definition of expert centers able to perform these procedures .


Myxoid liposarcoma (MLS) is an extremely rare tumor of the salivary gland and it arises from undifferentiated pluripotent mesenchymal cells. We report a rare case of a primary MLS in the parotid gland.The patient was a 49-year-old female who presented with a hard fixed mass in the left parotid region.On computed tomography and MR images, this tumor has a low attenuation center with a thick enhancing wall and ill-defined margins. The absence of high-signal-intensity foci on T1-weighted images makes a MLS indistinguishable from most other soft-tissue masses. Pathologically, the tumor was diagnosed as MLS.The patient received total parotidectomy with facial nerve preservation and selective neck dissection.Immediate facial nerve function was House Brackmann Grade III and recovered within 3 months after the surgery. Follow-up period is 57 months and there was no recurrence until now.In this report, we report a rare case of primary MLS mimicking a cystic lesion of the parotid gland. Primary liposarcoma of the salivary gland is an extremely rare malignancy, and it mostly occurs in the parotid gland. The other major salivary glands  are very rarely involved. When the tumor involves a major salivary gland, it present as a painless mass in the gland. When the minor salivary glands are involved, the liposarcomas may occur in the tongue, buccal cavity, palate, and other mouth regions. The cause of salivary gland liposarcoma is suspected to be a genetic abnormality and no definitive risk factors for liposarcoma development have been observed.Liposarcoma is generally asymptomatic, and it can occur anywhere in the body and it usually arises in the deep soft tissue of the lower extremity, particularly the thigh. A biopsy of the tumor is necessary for making an exact diagnosis to start treatment. They are of 4 different types; well-differentiated liposarcoma, dedifferentiated liposarcoma, myxoid liposarcoma (MLS), and pleomorphic liposarcoma. MLS accounts for approximately one third of all liposarcomas and has a predilection for a younger age group, with a peak incidence during the fifth decade of life. Genetic analysis for detecting any chromosomal anomalies can be used for making the initial diagnosis. These results provide supportive evidence for molecular testing when we encounter this differential diagnosis.The signs and symptoms of liposarcoma of the salivary gland include a slow-growing mass in the parotid area and pain while eating. Appropriate diagnosis and identification of the subtype of liposarcoma are essential for proper management of the subtypes. Interestingly, computed tomography (CT) or magnetic resonance imaging (MRI) may not show the typical features of a lipomatous tumor because the fat content of liposarcoma is often less than 10% to 25%. Therefore, it may be difficult to make the correct diagnosis of some MLSs with radiology due to the lack of fat signal intensity. The abnormal findings in magnetic resonance (MR) images also depend on the amount of myxoid material, the degree of cellularity and vascularity, and the presence of necrosis. Thus, some MLSs appear to be cystic on nonenhanced MRI, although they enhance like other solid masses on contrast material–enhanced MRI.–11The following 4 basic criteria for the diagnosis of primary MLS of the salivary gland are proposed: no sarcoma elsewhere, exclusion of metastasis to the gland from malignancies at other sites, exclusion of a carcinosarcoma, and pathological consistence with origin within the gland. Metastases to lung, bone, and soft tissue have been reported in the literature. A combined treatment with surgery, chemotherapy, and radiation therapy could be applied for treating MLS of the salivary gland. Overall prognosis is reported to be relatively good after prompt diagnosis and adequate treatment. We report a case of primary MLS of the parotid gland mimicking a cystic parotid lesion with a review of the literature. Informed consent was received from the patients about the publication.The clinical prognosis is strongly correlated with the histologic features, a more aggressive course being reported for tumors with a rich round cell component.2The patient was a 49-year-old female who presented with a hard fixed mass in the left parotid region. There was no specific past history. Since 3 months before visiting our hospital, the patient complained of a slowly enlarging left parotid mass without facial nerve dysfunction and paralysis. CT images Fig.  revealedWe performed total parotidectomy after modified radical neck dissection. The mass was deeply located in the deep lobe of the parotid gland and it showed adherence to the surrounding tissues, mandibular ramus, and mastoid bone. While dissecting the parotid gland, it was very difficult to identify the trunk of the facial nerve. The deep portion of the facial nerve was found to be completely engulfed by the tumor. The trunk of the facial nerve was well exposed after removal of the mastoid bone. The mass was successfully dissected from the facial nerve and surrounding tissues Fig. . On immu3,5 Chromosomal abnormality such as FUS-DD1T3 causes chromosomal translocation abnormality namely t, and chromosomal abnormality such as EWSR1-DD1T3 causes chromosomal translocation abnormality namely t.The development of liposarcoma is not yet well clarified, but it is thought to arise spontaneously due to genetic mutations. Recent studies do not support the claim that a liposarcoma arises from a lipoma and that there are no specific risk factors. Genetic molecular studies may play a significant role in identifying the tumor type and taking appropriate treatment decisions.,6,9The main signs and symptoms are the presence of a firm and visible swelling in the involved parotid area and a tumor that has been slowly increasing in size. Initially, the overlying skin on the parotid gland may not appear inflamed or show color changes, but changes in the overlying skin including ulceration may be noted as the tumor progresses and becomes larger in size. Also, it may cause neurological signs and symptoms, such as facial weakness and pain, due to facial nerve paralysis.,11 In general, lipomas and well-differentiated liposarcomas typically show high signal intensity secondary to the relatively high fat content. MLS generally shows high-signal-intensity foci within a predominantly homogeneous mass on T1-weighted images and intermediate or more prominent signal intensity on T2-weighted images. These foci represent fat within the tumor and usually appear lacy or linear and amorphous rather than solid. However, some MLSs may not exhibit the signal intensity typical of a fatty tumor. They may appear as cystic masses on non-enhanced images. This MLS consisted of a myxoid matrix as the predominant component and small amounts of mature fat within the tumor. Therefore, it showed low signal intensity on nonenhanced T1-weighted images and high signal intensity on T2-weighted images.For making the correct diagnosis of MLS, radiologic images (CT and MRI) are needed.,6 For differentiating between carcinoma and sarcoma, special immunohistochemical stains using Vimentin, S-100, CK, CK7, EMA, and HMB45 are needed. In sarcomas, cells are positive for Vimentin and S-100, and they are negative for CK, CK7, EMA, and HMB45. The possibility of extraskeletal mesenchymal chondrosarcoma and malignant peripheral nerve sheath tumor should also be excluded. Carcinosarcoma can be excluded because of no distinct carcinomatous component in the tumor. A malignant peripheral nerve sheath tumor can also be excluded because of the shape of the tumor cells originating from the nerve sheath.Because of different morphological lesions associated with liposarcomas, FNAC has certain limitations and it may not be helpful as a diagnostic tool. An open surgical biopsy is more preferred and Hematoxylin and Eosin staining is needed. Special studies such as immunohistochemical stains, molecular testing, and electron microscopic studies are necessary to assist in the diagnosis. Histologically, the tumor reveals variable cellular density, dense proliferation of slightly atypical round cells and rare mitotic figures. Any lesion consisting of edema, an extracellular matrix with a high level of mucopolysaccharide, hyaline cartilage content, and necrosis may appear as a cystic mass. Following surgery, radiation therapy is usually provided for tumors in the head and neck region. When the tumor is situated at an inaccessible location such as base of the skull or the parapharyngeal space, chemotherapy, and radiation therapy may be considered as noninvasive procedures. Careful follow-up with regular work-up is very important to detect recurrence and any metastatic lesions. Data on the prognosis of MLS of the salivary gland is not available due to the rarity of the cancer. In general, the prognosis of MLS of the salivary gland is generally good after prompt diagnosis and treatment. However, it shows poor prognosis in the advanced stage, high cell division rate, and large tumor size.The treatment of choice for MLS of the salivary gland is wide surgical excision with removal of the entire tumor.In conclusion, we report an unusual case of primary MLS of the parotid gland. Radiologically, some MLSs may not exhibit the signal intensity typical of a fatty tumor and may instead appear as cystic masses. The absence of high-signal-intensity foci on T1-weighted images makes an MLS indistinguishable from other soft-tissue masses. Therefore, MLS should be differentiated from benign cystic lesions and other malignant lesions using immunohistochemical staining and MR images.Conceptualization: Yong Tae Hong.Formal analysis: Yunna Yang.Investigation: Yunna Yang.Methodology: Yong Tae Hong.Resources: Hyunjun Lee.Validation: Hyunjun Lee.Visualization: Hyunjun Lee.Writing – original draft: Yong Tae Hong.Writing – review & editing: Yong Tae Hong.


To present the surgical treatment of a full thickness macular hole (MH) caused by a vitreomacular traction (VMT) on top of an adjacent subfoveal pigment epithelial detachment (PED) in age-related macular degeneration (AMD).A 77-year-old female with a subfoveal PED receiving consecutive intravitreal injections noticed a sudden decreased visual acuity (VA) due to the development an occult MH in her right eye after 19 repeated intravitreal anti vascular endothelial growth factor (VEGF)-injections. Her initial VA declined from 20/50 to 20/400. The firm VMT induced a rupture of the multi-layered retina and may progress to an RPE-tear or possible to a subretinal haemorrhage. We discussed with the patient the risks of the natural progression and explained possible treatment options: We continued her anti-VEGF combined with air bubble injections to induce a posterior vitreous detachment, to stabilise the retinal architecture, reduce the subretinal fluid and avoid possible intraoperative bleeding. As injections did release the VMT, vitrectomy released the posterior vitreous from the optic nerve and trimmed it towards the central retina. Peeling with brilliant blue removed the internal limiting membrane without any signs of bleeding, rupture of the PED or enlargement of the MH, prior to the installation of 10% SF6 gas. The postoperative optical coherence tomography (OCT) on day 5 confirmed a closed MH, while the size, shape and pattern of the PED remained unchanged. Her VA increased from 20/400 to 20/50 . To avoid a potential progression of the PED, we maintained her retreatment intervals at 5 weeks for the next 6 months. A literature review presents similar intraoperative approaches and postoperative outcomes in 8 out of the 9 published cases.VMT can induce an occult MH on top of a PED, causing a significant loss of vision. When gas injections are not successful, surgery may release the traction, restore the retinal architecture, and significantly improve and maintain the VA over a documented long-term observation. The epiretinal procedure should be assisted under regular anti-VEGF injections to maintain the subretinal architecture. The coincidental development of such an occult MH on top of a PED will lead to unexplained vision loss. Previously, we described this phenomenon as a natural causeHere, we demonstrate the surgical release of a persisting VMT in an MH over a subfoveal PED to discuss possible considerations in the preoperative preparation and intraoperative approach of our case, and to compare our postoperative outcome with the few published cases of this rare entity and approach.2A 77-year-old female with neovascular AMD and chronic PED OD had been treated with 19 intravitreal anti-vascular endothelial growth factor (VEGF)-injections over the course of 3 years. On optical coherence tomography (OCT) there was a significant PED with some SR fluid prior to each injection. Over time, we noticed an occult VMT at the foveal centre, which became evident when the retinal was thin soon after each anti-VEGF injection. Her visual acuity (VA) remained stable at 20/50 under this treatment regime, until she presented a sudden decrease of VA in her right eye from 20/400. On dilated fundus biomicroscopy, as well as standard OCT-scanning, no AMD-progression was apparent. A meticulous thin lined en face OCT-examination revealed an unchanged pattern of the PED, while a MH with a VMT on top of the PED became visible a. This tA) to stabilise the retinal architecture, B) reduce the SR fluid and C) seal the CNV of the PED as best as possible to avoid further bleeding. Delayed chromovitrectomy was now performed under general anaesthesia with initial staining of the vitreous with triamcinolone. With a high cutting rate of 5000 p.m. and a gentle aspiration of 40 mmHg, we removed the core of the vitreous under slow movements approaching the retinal surface. After the release of the vitreous from the optic nerve head, we gently trimmed the posterior vitreous towards the central retina and further to the periphery without any signs of a bleeding, rupture of the PED or enlargement of the MH. With the uncomplicated progress of the surgery, we even decided to stain the internal limiting membrane (ILM) with brilliant blue and performed a gentile ILM-peeling from the periphery towards the macular hole. The release of the ILM was gentle and with no significant tract; thus, we closed the surgery with a conventional fluid gas exchange and installation of 10% SF6 gas. The patient was asked to keep a prone position over night and was examined during the next few days. The intraocular gas bubble vanished over 5 days and her intraocular pressure remained normal at 18 mmHg. The OCT on her 5th postoperative day revealed a closed MH, while the size, shape and pattern of the PED had not changed (After extensive discussion with the patient, she agreed to continue with her anti-VEGF injections until we observed a shallow PED and compact neuroretina around the MH. The goal of these injections was to  changed b. During3,Elsing et al. reviewed the surgical outcome of such cases with 20-gauge instruments by combined MH-repair and CNV-removal,Hirata et al. reported the removal of a CNV-membrane with 25-gauge instruments through the coincident presence of a macular hole after anti-VEGF therapy for AMD and observed a rapid resolution of the intraretinal macular oedema with a fast anatomical and functional recovery to 20/40.The aging vitreous may play an important role as a coincident factor in the pathogenesis of numerous macular diseases.Grigoropoulos et al. hypothesised that the mechanical insertion of the injection needle leads to a globe deformation and retinal traction, while the removal of the needle may cause a consecutive vitreous micro-incarceration exacerbating the underlying VMT.The development of MH after intravitreal injections has been described in the past in eyes with the primary diagnosis of neovascular AMD, diabetic macular oedema, branch retinal vein occlusion or vasoproliferative tumours.I.The chronic underlying SR PED must be sufficiently treated by ant-VEGF injections. An active lesion may potentially cause SR bleeds, during either the surgical manipulation of the adjacent epiretinal VMT or the postoperative period. An undertreated PED prior to surgery may also cause significant SR fluid accumulation, making the neuroretina less compact and resistant to the presumed tractional shear force during the epiretinal ILM-peeling.II.Any systemic aspirin or warfarin application should be interrupted, if possible, prior to and shortly after the surgery.III.-The vitreous removal on top of such a delicate lesion requires a gentle and slow intentional removal of the vitreous with a high cutting rate and low aspiration, diminishing any possible traction on the perpendicular retinal lesion as much as possible.-Chromovitrectomy with triamcinolone may help to stain and visualise the posterior hyaloid for its safe and reliable removal.-The release of the posterior hyaloid should not start with the VMT itself, as unexpected firm adhesions may induce traction and vigorous force of the PED, possibly inducing a rupture of the thin RPE-monolayer or adjacent choroidal vessels causing a vision threatening subretinal haemorrhage. We recommend initiating the induction of PVD at first over the optic nerve head to release the vitreopapillary adhesion followed by trimming the posterior hyaloid only partially over the macular area.-Another consideration prior to surgery may be pneumatic vitreolysis by the intravitreal application of C3F8 gas to relieve vitreous traction and possibly close the macular hole.-Finally, we discussed whether a plain PVD may be enough to seal the MH on top of the PED, as many small conventional MHs close after a solely PPV without an ILM-peel.The stepwise intraoperative release of the epiretinal traction needs to be well-thought out and structured ahead of surgery:Our case also presented the simultaneous presence of two separate pathologies below and above the retina, requiring additional well-considered proceedings:However, we decided to intraoperatively stain and visualise the ILM with the application of brilliant blue by chromovitrectomy. We observed a well-delimited ILM and started an attempt to remove it at the outer macular area close to the vascular arcade, where the retina was not perpendicular to the fragile PED. We noticed a well-controlled, gentile release of the ILM and decided to extend the peeling in a slow manner from the periphery of the outer macula towards the rim of the MH. This manoeuvre should be avoided if the ILM presents firm adhesions, as small MH frequently close after the release of the VMT. The eye was closed after a conventional fluid-air-exchange and a fast conventional closure of the small MH presented within days. Once the epiretinal pathology has been treated, continuous retreatments of the subretinal PED with consecutive monthly anti-VEGF injections are recommend, as the pharmacokinetic durability of the fluid anti-VEGF drug may be reduced after removal of the vitreous according to previous measurements.While several papers described the rare development of MH after intravitreal injections in greater detail postulating the underling pathomechanism,Our findings correlate with most of the published cases 4The treatment of eyes with PED and VMT or ERM under anti-VEGF injections may, in rare cases, induce a small but full thickness MH-formation. These rare sequelae of occult MH are frequently associated with an unexplained decrease in central vision, requiring intense raster OCT-evaluations. Our case is complementary to others describing the surgical closure of full thickness MHs on PED-lesions after PPV with ILM-peeling. Treatment remains challenging but appears to be safe if caution is considered.All authors state that they meet the current ICMJE criteria for Authorship.All authors read and approved the final manuscript.There was no funding or grant support.Consent to publish the case report was obtained. This report does not contain any personal information that could lead to the identification of the patient.Paula S Meyer: Original draft preparation, Image collection, Formal analysis, Writing-, Marc T Kammann: Reviewing, Editing and Visualization, Carsten H. Meyer: Image analysis, Conceptualization, Methodology, Supervision, Reviewing.All authors have no financial disclosures.


Here, we report a case of synovial chondromatosis of the temporomandibular joint (TMJ) with numerous loose bodies. A 56-year-old woman was examined in the oral surgery department for trismus and pain in the left TMJ when opening the mouth. Imaging indicated TMJ synovial chondromatosis, and the patient was referred to our department for further examination. Her facial features were symmetrical, and no occlusal abnormalities were found. The maximum mouth opening was 30 mm, and movement of the left mandibular condyle was restricted and accompanied by pain and joint sounds. Panoramic radiography showed deformation of the left mandibular condyle and radiopaque lesions surrounding it. Computed tomography showed numerous small granules around the left mandibular condyle, some of which were calcified. Magnetic resonance imaging showed anterior disc displacement without reduction in the left TMJ and hypointense lesions on T2-weighted images. Bone scintigraphy showed an accumulation in the area of the left TMJ. Based on the diagnosis of the left TMJ synovial chondromatosis, the lesions were removed, and plastic surgery on the mandibular condyle was performed under general anesthesia. We removed 386 white loose bodies. Histopathologically, the loose bodies were consistent with synovial chondromatosis lesions. The postoperative course was uneventful, with no recurrence or TMJ dysfunction approximately 5 years after the surgery, indicating that open surgery is the best course of intervention in such cases. Synovial chondromatosis is a benign disease in which synovial cells create cartilaginous metaplasia in synovial tissue, resulting in loose cartilage bodies in the joint cavity. The most common sites include the knee, elbow, and hip. Synovial chondromatosis of the temporomandibular joint (TMJ) was first described by Von Haller in 1764 [A 56-year-old woman presented to our department with a chief complaint of pain in her left TMJ in December 2014. Around November 2012, the patient had noticed pain in her left TMJ when widely opening her mouth, but the symptoms disappeared, and she did not address the matter. Around July 2014, the symptoms recurred, and she began experiencing trismus. She visited the dental and oral surgery department at a hospital for examination. Both computed tomography (CT) and MRI indicated synovial chondromatosis of the TMJ, and she was referred to our department for treatment.Clinical examination found that she had a fairly healthy physique and no signs of nutritional deficiency. There was no visible facial deformation, and the maximum mouth opening was 30 mm. Movement of the left mandibular condyle was poor, and there was pain as well as joint sounds when widely opening the mouth but no occlusal abnormality .Panoramic radiography showed that the left mandibular condyle was flattened, with a radiopaque image near the superior joint cavity . The TMJT1-weighted MRI of the left TMJ showed deformity of the left mandibular condyle and anterior disc displacement without reduction, and T2-weighted MRI showed fine nodular areas of hypointensity in the superior joint cavity Figures . Bone scApproximately 2 months after the initial examination, the masses were removed, and plastic surgery was performed on the mandibular condyle under general anesthesia. Incision lines were set according to the Al-Kayat–Bramley method [Although the articular disc was preserved, the mandibular condyle was deformed. Therefore, plastic surgery of the mandibular condyle was performed using an ultrasonic cutting instrument . A closed drain was placed in the wound, and the incision was sutured to complete the operation .At the time of writing, it has been more than 5 years since the surgery. There have been no signs of local recurrence or trismus. Image findings have also not indicated any recurrence Figures  and 7.The loose bodies removed were 2 to 5 mm in diameter, white, bone-like, hard, and numbered 386 in total .Histopathologically, there was proliferation of cartilage tissue with chondrocytes forming round or irregular small masses surrounded by fibrous tissue, along with fibrosis and ossification. There were no signs of malignancy, which is consistent with synovial chondromatosis .In this report, we presented the case of a patient with TMJ synovial chondromatosis with numerous loose bodies who was successfully treated with surgery and has remained symptom free 5 years after the operation. Synovial chondromatosis of the temporomandibular joint presents with clinical symptoms similar to TMJ disorder, including trismus, restricted movement of the TMJ, pain, and noise , 4. ThusMilgram , 15 clasWe located 202 case reports of TMJ synovial chondromatosis, including the present one, published from 2009 to 2019 , 17–58 . The casThe therapy for TMJ synovial chondromatosis is principally surgical intervention, as the number of loose bodies increases or the symptoms worsen . If the β and tumor necrosis factor α were expressed in both the synovial cell layer and the vascular wall [Synovial chondromatosis is characterized by multiple chondrodysplasia or osteochondrodysplasia and free body formation in a joint due to synovial metaplasia, but its underlying pathological mechanism remains unclear. Possible triggers are trauma and continuous irritation to the synovial membrane . Trauma lar wall , 65. Thelar wall , 67. Howlar wall . ClarifyThe prognosis of this disease is relatively good, and recurrence is uncommon. However, because of reports of malignant transformation to chondrosarcoma in other joints , 69, 70,In conclusion, we reported a rare case of TMJ synovial chondromatosis with 386 loose bodies, which were removed using open surgery. There has been no recurrence in the 5-year follow-up period, indicating that open surgery is the best course of intervention in such cases.


Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma with an incidence of 6 cases per million per year.Blistering is not usually associated with MF, but when present, it is typically associated with aggressive course and poor prognosis.We present a male patient with generalized vesiculobullous MF. The blisters were arranged in an annular pattern mimicking adult linear IgA bullous dermatosis. The lesions rapidly progressed to tumors necessitating aggressive treatment. The diagnosis of bullous MF was made based on the clinical and histologic findings.A 43-year-old man presented with a 1-year history of progressive, generalized pruritic skin eruption. There was no history of viral infections, contact allergy, systemic illness, or oral drug intake.Physical examination revealed generalized infiltrated plaques on the trunk and extremities covering most of his body surface area. The plaques were studded with multiple vesicles and bullae (flaccid and tense), arranged in a characteristic annular pattern mimicking adult linear IgA bullous dermatosis. Some of the blisters were ruptured and associated with exudative superficial erosions . There wA). With higher magnification, lymphoid cells were observed to be large with convoluted nuclei (B). Immunohistochemical analysis revealed the infiltrate to consist of predominantly T cells, the phenotype of which was CD3+, CD4+, and CD8− (C and D). Direct immunofluorescence for IgG, IgA, immunoglobulin M, and C3 was negative.Skin biopsies were taken from the plaques and bullae. Histologic examination revealed both intraepidermal and subepidermal blisters, along with infiltration of the upper dermis and dermo-epidermal junction by atypical lymphocytes with migration into the epidermis (epidermotropism) , A. Withd nuclei , B. Immuand CD8− , C and DA). The patient had a mobile, non-tender axillary lymph node (1 × 2 cm).Based on these findings, the diagnosis of vesiculobullous MF was made. The patient was lost to follow-up but returned 4 months later with new skin lesions on the back. Examination revealed a painless, erythematous, eroded 4 × 6-cm tumor on the back , A. The B). By immunohistochemical analysis, the infiltrate was CD3+, CD4+, and CD8− (C and D). Core biopsies of the axillary lymph node revealed reactive lymphadenopathy. A bone marrow biopsy revealed no involvement. Computed tomography of the neck, chest, abdomen, and pelvis was normal. Clinical and histologic findings were consistent with stage IIB MF (tumor stage). Despite aggressive therapy including cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone, the condition rapidly progressed with continuous appearance of new lesions and tumors, resulting in death of the patient less than 1 year after the onset of the bullous lesions.Histologic examination of the nodule showed diffuse atypical lymphoid infiltrate involving the full thickness of the dermis and extending to the subcutaneous tissue , B. By iand CD8− , C and DThe vesiculobullous variant is a very rare clinical subtype of MF. According to Bowman et al,,The pathologic mechanism underlying blister formation in MF has not been elucidated. One explanation is the confluence of Pautrier microabscesses in MF lesions, which may lead to intraepidermal bulla formation.,,The main differential diagnosis is autoimmune bullous disease, that has been reported to occur concurrently with classic MF.The diagnosis of this rare entity is challenging, and the suspicion can only be raised by the presence of typical lesions of MF along with vesiculobullous lesions. Our patient had generalized MF plaques of prolonged duration with severe itching that developed into tumors in a short period of time.,This presentation highlights the aggressive nature of this variant. The treatment of bullous MF follows the usual treatment of MF according to the clinical stage. Phototherapy, methotrexate, interferon, bexarotene, radiotherapy, and histone deacetylase inhibitors are established treatments used in other variants of MF and have also been shown to improve bullous MF lesions.de novo or on top of typical MF lesions. Bullous MF may present with flaccid or tense bullae, may have negative or positive Nikolsky sign, and may be generalized or localized, without specific predilection site. Regarding the histopathology of bullous MF, the line of cleavage may be intraepidermal, at different levels, or subepidermal; however, the majority of the patients displayed the CD4+ MF phenotype.Epidemiologic, clinical, and histologic aspects of previously reported cases of bullous MF are presented in The case is usually treated as plaque or tumor stage MF, taking into consideration that the appearance of bullous lesions in a patient with MF appears to carry a poor prognosis; almost half the reported patients died within 1 year of the appearance of bullae, despite aggressive therapy.To conclude, we present a case of vesiculobullous MF with a distinguished presentation and a rapidly progressive course. Dermatologists should keep in mind the diverse presentations of MF to avoid misdiagnosis and delayed management.None disclosed.


Irritable bowel syndrome (IBS) is a benign condition of the gastrointestinal tract causing abdominal pain, bloating, diarrhea, and/or constipation. Symptoms of IBS usually improve on passing flatus and defecation. There is no known identifiable underlying pathology; however, several risk factors are known to contribute to the development of IBS, which include a stressful lifestyle and certain foods such as bread, coffee, alcohol, pasta, and chocolates. Intestinal bacteria may also contribute to symptoms of IBS. IBS is diagnosed clinically and treated with various medications to control the symptoms. On the other hand, celiac and mesenteric artery thrombosis (CAMAT) is a condition that may cause significantly higher mortality and morbidity if not recognized early. CAMAT leads to the blockage of major blood vessels to the intestine and several abdominal viscera leading to abdominal pain, nausea, sweating, and, in some cases, symptoms of shock. CAMAT is most likely caused by thrombosis; however, occasionally, embolisms from distant sources in patients with atrial fibrillation can also contribute to the development of CAMAT. CAMAT is usually diagnosed with a computed tomography angiogram (CTA) and treated either surgically or medically with anticoagulants. Vascular thrombus in the thoracic and abdominal region causing ischemia of the stomach and abdominal pain in patients with a history of IBS can easily be missed and cause grave complications with high morbidity and mortality. We present two cases who were initially diagnosed and treated for IBS and later diagnosed with serious intra-abdominal pathology of CAMAT thrombosis.The first case is of a 55-year-old female who was previously diagnosed with IBS and was treated with mebeverine 200mg twice daily and esomeprazole 20mg once daily for 10 weeks. Her pain continued to get worse and she presented to the emergency department by ambulance. She underwent CTA, which showed occlusion of the celiac trunk and superior mesenteric artery causing liver and splenic infarcts. The patient received heparin and underwent a thrombectomy and embolectomy of the superior mesenteric and celiac arteries. No significant abnormality was found in the blood results. Thrombophilia screening was negative. The patient was discharged on warfarin. The second case is of a 53-year-old man who was also initially diagnosed with IBS and was treated with mebeverine 200mg twice daily for eight weeks before presenting to the emergency department with worsening abdominal pain. He underwent a CTA with contrast, which showed occlusion of the common hepatic artery and stenosis of the splenic artery leading to multiple splenic infarcts. No significant abnormality was found in blood test. Thrombophilia screening was negative. He was treated with new anticoagulant medication, dabigatran 150 mg orally twice daily.Both patients were managed with successful outcomes and were discharged home on anticoagulants. There was no recurrence of symptoms at three-month follow-up.These cases highlight that a secondary cause of symptoms such as vascular thrombosis must be sought for patients who fail to improve with conservative management of IBS. Irritable bowel syndrome (IBS) is a functional disorder of the gastrointestinal tract causing long-term recurrent abdominal pain. Patients may also suffer from a bloating sensation with excessive flatus, diarrhea, or constipation. The diagnosis of IBS is based on the Rome IV criteria . PatientThoraco-abdominal aortic thrombus leading to mesenteric ischemia is a rare presentation in the emergency department. Diagnosis requires a high index of suspicion especially in patients with risk factors, which include cardiac embolus related to arrhythmia or valvular heart disease, peripheral artery disease, vasoconstrictive medications, hemodialysis, hereditary or acquired thrombotic conditions, infections, and hypovolemia . Delays Case 1A 55-year-old female with a history of IBS and reflux disease presented to the emergency department with severe abdominal pain radiating to the left hypochondrium and back associated with multiple episodes of vomiting. The patient had stable vital signs. Physical examination showed left upper quadrant tenderness with no guarding. Initial laboratory investigations showed a slight increase in liver enzymes. An ultrasound was performed, which was unremarkable. Due to persistent pain, a CTA was performed. The CTA showed an intramural thrombus of the descending aorta Figure  at the lThe patient was started on a heparin infusion, and vascular surgeons were consulted. The patient underwent thrombectomy and embolectomy of the superior mesenteric and celiac arteries on the same day. The patient’s post-operative course was unremarkable. Thrombophilia screening was negative. The patient was started on warfarin and was discharged in good general condition.Case 2A 53-year-old man was initially diagnosed with IBS and treated with mebeverine 200mg twice daily for several weeks before presenting to the emergency department with worsening abdominal pain. The abdomen was soft and lax with mild tenderness. The bowel sounds were normal. The patient underwent CT with contrast, which revealed dilation of the celiac trunk Figure , 5. The  IBS is a common cause of functional disorder of the gastrointestinal tract, presenting as abdominal pain and alteration of bowel habit. Its prevalence ranges from 9% to 23% of the worldwide population . The mecAcute celiac and mesenteric occlusion due to intramural aortic thrombus causing ischemia is a life-threatening cause of abdominal pain in patients presenting to the emergency department . DiagnosTreatment options include surgery and endovascular thrombolysis or both depending on the hospital resources. Studies show that endovascular treatment has the same efficacy as a surgical approach, with no significant mortality differences between the two methods -10.Both cases highlighted that if abdominal pain symptoms get worse in patients with previously diagnosed IBS, an alternative and more serious pathology should be considered. Early diagnosis and treatment prevent long-term complications in these patients. Both patients presented to the hospital through emergency medical services. Early diagnosis and treatment resulted in prevention of complications.Vascular thrombosis causing visceral ischemia is a challenging diagnosis because it is a rare entity and requires a high index of suspicion to test, particularly in patients with a secondary diagnosis such as functional gut disorder. We should consider it as part of differentials in patients presenting to the emergency department with disproportionate abdominal pain.It is crucial in the emergency department to rule out serious and life-threatening causes of abdominal pain especially in patients with risk factors. Vascular thrombosis causing visceral ischemia is challenging to diagnose as it is rare and needs a high index of suspicion, particularly in patients with functional gut disorder. We should consider it as part of differentials in patients presenting to emergency department with disproportionate abdominal pain.


TP53 mutation, Li-Fraumeni syndrome (LFS), is a syndrome that leads to a hereditary cancer predisposition. Here we describe the case of a 13-year-old male who presented with osteosarcoma, family history of LFS, who developed a second primary tumor of the lung. No other similar cases have been reported. After this osteosarcoma diagnosis, he had pre-operative imaging, which included a positron emission tomography (PET) combined with CT (PET/CT) chest. This revealed a subpleural nodule in the lung of unclear etiology. After completing initial therapy, a repeat chest CT showed that the nodule persisted. Pathology revealed an acinar adenocarcinoma. This tumor is not common in pediatric LFS patients. TP53 gene mutation, also known as Li-Fraumeni syndrome (LFS), is an autosomal dominant syndrome that leads to a hereditary cancer predisposition . First dThe patient is a male who presented at the age of 12 to the Pediatric Oncology Practice for persistent right upper arm pain for six weeks. Due to progressive edema, an X-ray was performed which revealed a right humeral lesion Figures . This leTen weeks following neoadjuvant chemotherapy with cisplatin, doxorubicin, and high-dose methotrexate, our patient had a radical resection of his right proximal humerus. One month later, he required further surgeries due to postoperative wound complications. After completing 21 weeks of chemotherapy, a repeat CT chest showed that the subpleural nodule did not demonstrate treatment effect and remained at 5 mm. After a multidisciplinary discussion involving orthopedic surgery, pediatric oncology, and radiology, the decision was made to obtain a thoracoscopic biopsy of the right upper lobe to rule out metastatic osteosarcoma. Pathology revealed an acinar adenocarcinoma with a positive margin. He underwent wedge resection of the lesion with no residual malignancy. Prior to the presentation, the patient’s parents were aware that there was a family history of LFS. During this patient’s first admission for chemotherapy, genetics was consulted, given his family history of LFS. See full pedigree below Figure . His patPatients with LFS have a very high lifetime cumulative risk of developing multiple malignancies and a strong family history of early-onset malignancies. Soft tissue sarcomas, osteosarcomas, premenopausal breast cancer, brain tumors, and adrenal cortical carcinomas are all LFS-associated tumors. As can be seen in the pedigree of this family, there is an abundance of tumors that can be seen in families affected by LFS. His paternal grandmother developed multiple malignancies that are typical of LFS. This patient’s pedigree shows a classic autosomal dominant inheritance pattern, and while it cannot be confirmed by genetic analysis at this time, it is likely that earlier generations may have had carriers of LFS based on his pedigree. Also, important to note is that his father is currently undergoing surveillance for cancer detection. With regards to his younger brother, who has also tested positive for TP53 mutation, he is currently undergoing surveillance imaging per the Toronto Protocol. The Toronto Protocol is a multi-modality protocol that was developed by investigators in Toronto, Salt Lake City, Los Angeles, and Columbus. The goal was to detect tumors early and reduce morbidity and mortality in patients who are TP53 mutation carriers. They found an improved overall survival of 88.8% in five years compared to 59.6% in patients who did not undergo surveillance. This protocol includes complete physical exams and then groups modalities under specific categories of tumors. The different modalities include ultrasound, MRI, colonoscopy, and esophagogastroduodenoscopy (EGD). These screening modalities change depending on the age and sex of the patient, as certain tumor incidences vary based on these factors .Whole-body MRI is becoming more widely used given its utility in assessing multiple organ systems, high sensitivity, and lack of ionizing radiation. Children with LFS have increased radiosensitivity, making CT less suitable for long-term surveillance. For this reason, an annual whole-body MRI from the time of diagnosis is recommended. Whole-body MRI has an NPV of 100% in pediatric cancer predisposition syndrome patients .Our patient underwent a CT chest to further examine the lung nodule found in earlier imaging. Despite the risks of ionizing radiation, the CT chest is currently the best modality in detecting lung nodules. Findings seen in Figure Typically, osteosarcomas in LFS patients occur in the ages between 10 and 14 years and are slightly more frequent in males . The autThis patient was confirmed to have TP53 mutation, which is also known as LFS, an autosomal dominant cancer predisposition syndrome. It involves multiple primary tumors and is associated with a broad spectrum of hematologic and solid malignancies. Current surveillance protocols do not include routine screening modalities for lung cancers. This brings to light that the risks and benefits of surveillance imaging are needed to be considered further in LFS patients. Despite the risks of ionizing radiation, the CT chest is currently the best modality in detecting lung nodules. Findings from his chest CT will impact his treatment course and led to the diagnosis of his lung adenocarcinoma. For this reason, further research on imaging techniques is needed for cancers that are not commonly seen in patients with TP53 mutation.


Poromas or poroid tumors are a group of rare, benign cutaneous neoplasms derived from the terminal eccrine or apocrine sweat gland duct. There are four poroma variants with overlapping features: dermal duct tumor (DDT), eccrine poroma, hidroacanthoma simplex, and poroid hidradenoma, of which DDT is the least common. Clinically, the variants have a nonspecific appearance and present as solitary dome-shaped papules, plaques, or nodules. They can be indistinguishable from each other and a multitude of differential diagnoses, necessitating a better understanding of the characteristics that make the diagnosis of poroid neoplasms. However, there remains a paucity of information on these lesions, especially DDTs, given their infrequent occurrence. Herein, we review the literature on DDTs with an emphasis on epidemiology, pathogenesis, clinical features, diagnosis, and management. Acrospiromas are a broad class of benign skin adnexal tumors of acrosyringial differentiation. A group of benign growths derived from cells of the terminal eccrine or apocrine sweat gland duct, known as poromas or poroid tumors, fall within this class . There aDDTs are predominantly located within the dermis and thus are also known as intradermal or dermal eccrine poromas . They arWhile the exact incidence of poromas is unknown, several studies have revealed specific epidemiological data. One single center study reviewed approximately 1,741,379 epithelial skin tumor biopsies over 17 years and noted the occurrence of 101 poromas 0.0058%) [8% [5]. TPoromas typically occur in the elderly population mean age 65.1–66.6) with a slight predilection for males  ,6. Long-5.1–66.6 The exact pathogenesis of poroid neoplasms is unknown, although immunohistochemical studies have provided clues . Based oYAP1-NUTM1 or YAP1-MAML2) were present in 113/146 (77.4%) poromas [More recently, YAP1 gene fusions have been implicated in the tumorigenesis of poroid neoplasms. The exact role of YAP1 in poromas has not been elucidated; however, results from 2 studies revealed certain fusions , infiltrative dermal papules (40%), or pedunculated tumors (10%)  Figure . Color rDifferential diagnosis of DDTs includes all poroma variants  and other sweat gland tumors . Other dOf the 19 DDTs reviewed by Battistella et al., the clinical diagnosis was most often dermatofibroma (33%), followed by dermal melanocytic nevus (28%), melanoma in pigmented cases (22%), and benign dermal tumors  (17%) .While there are no specific reports reviewing dermatoscopic findings in DDTs, certain vascular patterns have been noted to aid in diagnosis of poromas. Polymorphic, glomerular, linear-irregular, leaf- and flower-like, and looped or hairpin variants are commonly seen . While tThe predictive value of white interlacing areas around vessels, yellow structureless areas, milky-red globules, poorly visualized vessels, and branched vessels with rounded endings has been studied . The preUltimately, differentiation occurs via histopathology. The principal finding in all forms of poroma is a circumscribed proliferation of compact cuboidal keratinocytes with small non-palisading monomorphous nuclei with scant eosinophilic cytoplasm (poroid cells) and larger squamous eosinophilic keratinocytes (cuticular cells) . In someThe aggregates of poroid and cuticular cells in a poroma may show ductal or tubular formations; however, the degree of ductal differentiation may vary between each type . Some poPoroma variants are differentiated based on the predominant cell type present and the degree of epidermal/dermal involvement . HoweverThe histopathologic diagnosis of DDT is rare due, in part, to the fact that some regard DDTs as poromas with a limited epidermal connection that has not been identified on the histologic sections examined or as hidradenomas that are smaller and more superficial . As an eSuperficial lesions may be treated with shaving or electrosurgical destruction . Deeper Malignant transformation risk is minimal; however, poromas may rarely evolve into porocarcinomas. The frequency of porocarcinoma is approximately 0.005–0.01% of all skin cancers . It may Poromas are a benign group of tumors derived from cells of the terminal eccrine or apocrine sweat gland duct with four variants: DDT, eccrine poroma, hidroacanthoma simplex, and poroid hidradenoma. DDT is the rarest and is differentiated from the other variants by its cellular composition, as well as its location in the dermis and small foci of solid or cystic components. However, these features may not always be clear cut and can overlap with other variants. Futhermore, all poroma variants can mimic many other dermatologic conditions. Therefore, it is essential to have a complete understanding of poromas to ensure proper diagnosis and treatment.


Meningiomas combined with meningioangiomatosis (MA-M) present similarly to more invasive lesions because of their appearance on neuroimaging. These lesions are especially rare in pediatric patients and suggestive imaging can help identify them for differential diagnosis. An 11-year-old male child who presented with diplopia and a headache was found to have an edematous invasive appearing temporal lobe mass on magnetic resonance imaging. Despite the lesion’s appearance, it was completely resected and found to be a benign MA-M upon histopathologic examination. The present case demonstrated a rare meningioma with meningioangiomatosis that appeared to be a higher grade or invasive lesion upon initial imaging in a pediatric patient. A review of the literature was performed on patients who presented similarly. Despite the rarity of this condition in children, neuroimaging should be carefully examined prior to surgical resection of similar masses in preparation for highly vascular tissue, and post-operative course can be better anticipated when MA-M is considered during differential diagnosis. Interpretation of neuroradiologic findings is critical in the treatment of neurosurgical patients, and benign intracranial masses on magnetic resonance imaging (MRI) may often mimic more invasive lesions due to concomitant presentation. The co-occurrence of meningiomas with meningioangiomatosis (MA) can appear similar to an invasive meningioma on imaging, which presents challenges along the course of treatment. Meningiomas are rarely found in children despite being the most encountered primary neoplasm in the adult population, and few reports in the literature discuss their co-occurrence with MA . MA is aOccurrences of meningiomas combined with MA (MA-M) are rare and thought to form either through neoplastic transformation of hamartomatous MA or through meningiomas that spread perivascularly along Virchow-Robin spaces ,4. MA-M The authors report a case of an 11-year-old male child who presented with diplopia and a headache. MRI revealed an edematous temporal lobe mass suspected to be an invasive meningioma. But upon surgical resection, the pathologic results found the mass to be a rare World Health Organization (WHO) grade 2 meningioma with accompanying MA. This study aimed to discuss the features of MA-M including its clinical presentation, histopathologic features, and radiologic characteristics through discussion of our present case and a review of the current literature .An 11-year-old male patient with no significant past medical history or known genetic syndromes presented to the emergency department three days after onset of left frontal headache and associated diplopia. The patient did not have nausea, emesis, paresis, or paresthesia. Physical examination was significant for diminished visual acuity, bilateral papilledema, and anisocoria in the left eye. He also had right pronator drift. He showed no other neurologic deficit. A T1-weighted MRI with contrast showed a heterogeneously enhancing left anterior temporal extraaxial tumor with mass effect and significant surrounding vasogenic edema suggestive of an invasive meningioma, based on its location and appearance  immunostaining demonstrated a moderate cellularity meningothelial meningioma with increased mitotic activity. There was also suggestion of early focal necrosis, and high Ki-67 labeling indices were found supporting that this portion of the mass was a WHO grade 2 meningioma with atypical features. In addition, some flanking areas demonstrated distinct disturbances in cerebral parenchyma underlying the meningioma, and a strip of vertically oriented proliferating vessels were seen in vascular columns. This area was also flanked by meningothelial cells that were entrapping and distorting the cortical grey matter between them. This indicated meningioangiomatosis in addition to the mentioned meningioma tissue.There were no postoperative complications and the patient’s presenting symptoms were completely resolved following surgery. The patient’s physical examination improved post-operatively and he was discharged without any sequelae of neurologic deficits. Upon a three-month follow-up, the patient continued to remain asymptomatic and without signs of recurrence on surveillance imaging. There was no plan for adjuvant therapy given no evidence of residual on imaging Figure . A review of the English-language literature revealed nine papers published between 1982 and 2020 that reported cases of pediatric MA-M (Table The search found a total of 30 patients, age range 0.8-17 years old, with 26 male and four female. Seizures, headaches, vomiting, and nausea were among the symptoms reported by patients, with seizures being the most common. Imaging findings upon presentation and histopathologic findings of the concomitant meningiomas are summarized in Table MA may occur alongside meningiomas, but a review by Zhang et al. also found reported cases of MA co-presentation along with arteriovenous malformations, encephaloceles, oligodendrogliomas, meningeal hemangiopericytomas, and focal cortical dysplasia . TransitOften a transition zone between the MA and meningioma may be seen on histopathologic examination, and this was similar in structure to the flanking meningothelial cells we found that marked a plane between the meningioma and MA components . PatholoDifferentiating presenting intracranial masses on neuroimaging can be based on subtle signs, but in some instances, the appearances between different pathologic subtypes of tumors can mimic one another. Current reports of MA-M suggest that they appear as non-enhancing hypointense masses on T2-weighted MRI , but othWe reported a case of a pediatric patient with MA-M and reviewed related literature. Despite the rarity of MA-M in children, when suggestive imaging signs are encountered, then preparation for highly vascular tissue during resection should be made and post-operative course can be better anticipated. Adequate preparation and careful surgical technique may lead to better clinical outcomes in these patients and avoid potential intraoperative complications.


Teaching point: The probable mechanism of pseudoaneurysm formation related to metastatic neoplasm is a tumor embolus penetrating and destroying the vessel wall.Pulmonary artery pseudoaneurysm (PAP) related to metastatic neoplasm is rare. We describe a unique case of multiple PAPs secondary to metastatic uterine leiomyosarcoma and demonstrate the serial chest computed tomography to support the theory that the tumor begins as a tumor embolus, followed by infiltration and breakdown of the vessel wall, leading to aneurysmal dilatation and invading the perivascular tissue. Figure 1). Computed tomography pulmonary angiography (CTPA) was performed to rule out acute pulmonary thromboembolism and showed innumerable heterogeneously enhanced masses scattered in both lungs, surrounding segmental branches of pulmonary arteries. Multiple fusiform pulmonary artery pseudoaneurysms surrounded by soft tissue masses were also noted . A review of serial chest computed tomography (CT) revealed tumor emboli in the peripheral branches of pulmonary arteries , which finally developed into pseudoaneurysms with surrounding soft tissue masses .A 49-year-old female with known uterine leiomyosarcoma and lung metastasis with multiple tumor thrombi in pulmonary arteries was receiving palliative chemotherapy. Prior to her fourth chemotherapy session, the patient developed acute-onset dyspnea. Chest radiograph revealed a larger size of the multiple well-defined pulmonary masses scattered in both lungs; therefore, progressive pulmonary metastasis was suspected . The serial chest CT also showed gradual dilatation of peripheral branches of right pulmonary arteries ; this could be due to tumor growth and continued destruction of vessel walls.A similar mechanism of pathogenesis is thought to have occurred in this case. From the serial chest CT, the PAPs with surrounding soft tissue masses had previously shown intraluminal thrombus and dilated pulmonary artery, which resembled tumor thrombus (Abnormal dilatation of the pulmonary arteries and veins was also observed in the patient’s CTPA with similar findings previously described in a reported case of metastatic uterine sarcoma . The onlIn conclusion, PAPs caused by metastatic neoplasm may firstly begin as tumor emboli, later progressing into pseudoaneurysms as we elucidated in the serial chest CTs and CTPA findings in this case.


Gall stone ileus is one of the rare complications of patients with cholelithiasis and usually affects elderly females. The usual sites for the stone to get impacted are the distal ileum and ileocaecal valve. Computed tomography (CT) remains diagnostic and surgery is the treatment of choice. A 60-year-old diabetic female, who was diagnosed with gall stone-induced pancreatitis one month ago, presented to the surgical emergency department with complaints of right upper abdominal pain with recurrent vomiting and constipation of five days duration. The patient was managed conservatively. A provisional diagnosis of subacute intestinal obstruction was kept and a barium meal follow-through (BMFT) was requested. However, BMFT was inconclusive. After two weeks, she presented again to the emergency department with clinical features of subacute intestinal obstruction. The patient was planned for exploratory laparotomy in view of recurrent episodes of obstruction and the presence of peritonism. Intraoperatively, we encountered a cholecystogastric fistula with a gall stone of size approximately 6.5x4 cm impacted at approximately 60 cm from the ileocaecal junction and dilated proximal small bowel loops.The surgical procedure comprised enterolithotomy and cholecystectomy along with repair of cholecystogastric fistula done. The patient had an uneventful postoperative course. Gall stone ileus is a rare cause of small bowel obstruction. Gall stone ileus presenting with a recent history of pancreatitis further makes the suspicion very unlikely. Gall stone ileus is one of the rare complications of cholelithiasis, accounting for 0.3-0.5% of the patients with cholelithiasis . The namGall stone ileus usually presents in elderly females (70-80 years) with a female to male ratio of 3.5 to 4.5:1 [A computed tomography scan remains the investigation of choice with a specificity of 100% . SurgeryOur patient was a 60-year-old diabetic female who presented to the surgical emergency with complaints of right upper abdominal pain with recurrent vomiting and constipation for the past five days. She was diagnosed and treated for gall stone-induced pancreatitis one month ago in a different institution. She was worked up for the same with an ultrasound abdomen and contrast-enhanced computed tomography (CECT). On review of her previous investigation records, the ultrasound of the abdomen revealed a single large calculus in the gall bladder with normal common bile duct anatomy. The CECT that was done during the episode of pancreatitis revealed acute pancreatitis and did not reveal any gall stone. The patient gave a history of being treated successfully for pulmonary tuberculosis 15 years ago. On examination, she was mildly dehydrated with a pulse rate of 105/min and normal blood pressure. The abdomen was soft, mildly distended, non-tender, with exaggerated bowel sounds. Her hematological and biochemical investigations, including amylase and lipase, were within normal limits. The abdominal radiograph revealed dilated bowel loops with few air-fluid levels Figure .Two weeks after undergoing barium meal follow-through, the patient developed recurrent episodes of vomiting and non-passage of flatus and feces with abdominal distention. On examination, the patient was tachycardic and the abdomen showed signs of peritonism. After adequate resuscitation, erect and supine X-rays of the abdomen were done. These revealed multiple air-fluid levels with dilated small bowel loops and no pneumoperitoneum. In view of recurrent episodes of obstruction and the presence of peritonism, the patient was taken up for exploratory laparotomy.Intraoperatively, we encountered a cholecystogastric fistula (approximately 1x1.5 cm) Figure  with a gGall stone ileus usually presents in elderly females 70-80 years). The oldest patient of gall stone ileus who successfully underwent surgery was 91 years old and the case was reported from Japan 0 years. , and in Patients with gall stone ileus very rarely present with gall stone-induced pancreatitis; only three cases have been published so far. However, our patient with gall stone ileus presented with a prior history of gall stone-induced pancreatitis. According to the available literature, a plausible reason for this is that the stone was causing external compression at the ampulla, as seen in the case of Bouveret syndrome, and this could be the reason for the initial presentation of the patient .The most common pathophysiology of gall stone ileus is the migration of a stone through a bilioenteric fistula. Bilioenteric fistulas account for only 2% of all biliary fistulas, and they accompany gall stone disease in 90% of the cases . There mA plain abdominal radiograph can be helpful for the following findings of the Rigler triad: 1) pneumobilia, 2) small bowel obstruction, and 3) ectopic radio-opaque gall stone. Two out of these three findings suggest gall stone ileus but the literature reports that it is seen in only 9-87% of cases .The sensitivity of ultrasound, when combined with a plain radiograph, is around 74% . CT is tSurgery remains the mainstay of treatment but the choice of the best surgery is still controversial. Enterolithotomy alone is recommended for patients with sick patients and significant comorbidities (American Society of Anesthesiology (ASA) class III & IV). In this surgery, the bowel is opened longitudinally, just proximal to the site of obstruction. The disadvantages of performing only enterolithotomy include recurrent gall stone ileus (33%) and cholangitis (60%) .Enterolithtotomy with cholecystectomy with fistula repair at the same setting (one-stage procedure) is preferred in patients who are hemodynamically stable at the presentation or falling in the ASA I and II groups. Enterolithotomy with delayed cholecystectomy and fistula repair is also advocated to reduce mortality and morbidity. Clavien et al. reported that the age and comorbidity matched patients had no difference in outcome among these two procedures . As in oLaparoscopic enterolithotomy is also an available option but the conversion rate is more than 50%, as reported in the literature . EndoscoGall stone ileus, although a rare cause of small bowel obstruction, is rarely diagnosed preoperatively. Patients with gall stone ileus presenting with a recent history of pancreatitis further make the suspicion very unlikely. A high index of clinical suspicion is required to diagnose them preoperatively. Most of the time, the diagnosis is often established after a laparotomy, irrespective of an inconclusive investigation. Despite no definitive preoperative diagnosis, it is justified to proceed with the laparotomy, as the delay hampers the outcome of the surgery. The choice of surgery is based on the evaluation of the patient’s condition and the surgeon’s intraoperative judgment.


Osteoid osteoma of the distal phalanges in the hand is rare and difficult to diagnose. We report a case of a 37-year-old Caucasian female patient who presented with a mass on the distal phalanx of the index finger. The patient was suffering from intermittent nocturnal pain for more than 18 months along with thickening, localized swelling, and clubbing of the distal phalanx of the right index finger. Radiographs revealed a lytic lesion of the distal phalanx of the right index finger with surrounding sclerosis. An MRI showed an intramedullary lesion with infiltration of the bone marrow, cortex, and surrounding tissue with focal sclerosis and elements of enhancements. A presumptive diagnosis of osteoid osteoma was made and surgical removal of the lesion by curettage and bone grafting was the treatment of choice. The curetted specimen was sent to pathology and the diagnosis of osteoid osteoma was confirmed. The patient was asymptomatic at six months postoperatively. Osteoid osteoma should be included in every differential diagnosis for patients presenting with atypical features of the distal phalanx of the hand. Osteoid osteoma is a relatively frequent benign bone-forming tumor with an incidence of 11% of all benign tumors . Pain isA 37-year-old right-handed Caucasian female patient presented to the outpatient orthopedic clinic with thickening of the distal phalanx of the right index finger that gradually increased over 18 months with intermittent pain that exacerbates at night, responding well to non-steroidal anti-inflammatory drugs (NSAIDs). On physical examination, localized swelling with clubbing of the distal part of the right index finger was noted, with both the pulp and the nail plate being enlarged and the nail fold angle disrupted. A slightly decreased range of motion in the distal interphalangeal joint was documented when compared to the contralateral hand. The patient denied any history of trauma or infection, and her blood profile for rheumatic and inflammatory disorders was normal as per the previous visit assessment in another facility. Radiographs performed revealed a lytic lesion of the distal phalanx of the right index finger with surrounding sclerosis  was done, which showed an intramedullary lesion of the index finger distal phalanx with infiltration of the bone marrow, cortex, and surrounding tissue with focal sclerosis and elements of enhancements Figure .Based on clinical and radiographic evidence, a presumptive diagnosis of osteoid osteoma was made; however, other conditions including infection, glomus tumor, and blastoma were to be ruled out. The patient underwent surgery. Using a lateral approach, the terminal phalanx bone was exposed and the lateral cortex perforated. Curettage of the nidus was made with a dental burr and drill followed by removal of the sclerotic edges without further perforation of the cortex Figure .Using fluoroscopic guidance, verification of complete evacuation was done, and bone grafting using a demineralized bone matrix was performed. The curetted specimen was sent to pathology. Postoperatively, the finger was immobilized with a splint and the patient reported immediate pain relief after a few days. Histopathological examination confirmed the diagnosis of osteoid osteoma Figure .The patient was followed up every three months and showed full clinical recovery at six months. Follow-up radiographs at three months Figure  and nineIn 1935, Jaffe introduced the term osteoid osteoma for a certain small, round tumor containing a central core referred to as a nidus . High leDelay in diagnosis and, in most cases, misdiagnosis has been frequently reported with such lesion, which leads to the wrong choice of treatment initially . In a stRegarding the management of osteoid osteoma, a variety of interventions have been described in the literature. They can range from a minimally invasive percutaneous radiofrequency ablation introduced by Rosenthal et al.  in 1992,Although osteoid osteoma is a relatively frequent benign bone-forming tumor, its pathophysiology remains unclear when it comes to the hand. In the distal phalanges, osteoid osteoma can present with unusual clinical, radiological, and histological characteristics, resulting in a delay in diagnosis. Therefore, in patients presenting with any uncommon features involving the distal phalanx, it is a necessity to include osteoid osteoma in every differential diagnosis.


Granular cell tumors are rare benign soft-tissue lesions that most commonly occur in the head and neck. They usually present in adulthood and are rarely seen in children. Here we present a 13-year-old girl who experienced symptoms of hoarseness of voice for most of her childhood and was unsuccessfully treated for asthma, acid reflux, allergies, and bronchitis before direct visualization revealed what was initially thought to be a vocal cord cyst. Surgical excision and pathology revealed the unexpected diagnosis of a vocal cord granular cell tumor. The patient has had resolution of dysphonia and is undergoing voice therapy.  Granular cell tumors (GCTs) are rare neoplasms that most commonly affect the skin and subcutaneous tissue -3, with GCTs can affect any age group, but are most commonly present during the fourth to sixth decade of life ,2,5 and A 13-year-old girl presented for evaluation of hoarseness. The patient’s mother noted that the patient had always spoken with a hoarse voice and that the hoarseness had worsened in the past five years. During this time she attempted treatments for asthma, acid reflux, allergies, and bronchitis, with no improvement of hoarseness. The patient had not had a formal otolaryngology workup before now. The patient denied sore throat, dysphagia, or globus sensation. She had no difficulty swallowing and denied postnasal drip or congestion. She has no chronic medical conditions. She underwent tonsillectomy and adenoidectomy three years ago due to recurrent infections. She is otherwise healthy with normal development.Her voice was mostly rough per GRBAS scale . She underwent rigid videostroboscopy which revealed full vocal fold mobility bilaterally, large left-sided infraglotttic swelling of the entire length of the vocal fold, with a rounded swelling at the midmembranous area. Glottic closure was complete with no lesions in the vallecula, larynx, or hypolarynx. Mucosal wave propagation showed chasing wave asymmetry with decreased amplitude on the left but normal on the right. There were no signs of reflux, the trachea was widely patent, and she displayed no gross aspiration. A presumed diagnosis of vocal fold cyst was made.2 laser was brought in to remove any protrusion of the vocal fold to give a straight phonating edge  growth, Deafness) ,5. WhileTreatment is complete surgical excision, and regular follow-up is required as recurrence is known to occur even in benign lesions. For benign lesions, complete surgical excision is considered curative, with a recurrence rate between 2% to 8% . IncomplGranular cell tumors are a rare cause of dysphonia in children. Patients are often unsuccessfully treated for bronchitis or asthma for years before accurate diagnosis and treatment. Visualization of the tumor may be inconclusive as it closely resembles more common vocal cord masses and biopsy is often required for definitive diagnosis. Treatment is surgical, and curative in most patients, though speech or voice therapy may be beneficial in some cases, and regular long-term follow-up is recommended.