Inflammatory Pseudotumor Research Articles

An Anaplastic Lymphoma Kinase-positive Inflammatory Myofibroblastic Tumor with Rapidly Acquired Resistance to First-line Anaplastic Lymphoma Kinase Inhibitor: A Case and Literature Review

Abstract Inflammatory myofibroblastic tumors (IMTs) are soft-tissue neoplasms with rare metastatic potential. Approximately half of IMTs are positive for an anaplastic lymphoma kinase (ALK) gene rearrangement which causes aberrant expression. Early phase clinical trials have demonstrated the efficacy of ALK inhibitors in the treatment of IMTs. However, there is no definite conclusion on which ALK inhibitor performs best, and data regarding subsequent therapy after first-line ALK inhibitor failure are scarce. Here, we report a case of ALK+ metastatic IMT that demonstrated a dramatic response to first-line alectinib but resulted in rapidly acquired resistance. Repeated biopsy and next-generation sequencing (NGS) showed ALK:c.3604G>A; p.(Gly1202Arg), which is a common mechanism of drug resistance in ALK fusion-positive non-small cell lung cancer. We also report subsequent treatment choices and responses in this patient and perform a literature review regarding similar cases as this rare tumor.

Uterine inflammatory myofibroblastic tumor: a retrospective analysis.

Uterine inflammatory myofibroblastic tumor (UIMT) is a rare tumor of the female reproductive tract with uncertain malignant potential. Previous case series reports have limited our understanding of its diagnosis and treatment. Therefore, we conducted a retrospective analysis of patient files at West China Second University Hospital, Sichuan University to contribute valuable clinical insights to future treatment strategies for this disease. We comprehensively reviewed patient files of individuals diagnosed with UIMT from January 1st, 2013 to May 1st, 2023. We included twenty-seven cases of uterine inflammatory myofibroblastic tumor in our study. Of these, 51.85% (14 cases) were diagnosed with abnormal uterine bleeding, 2 cases had dysmenorrhea, and 12 were unexpectedly diagnosed with suspected uterine fibroids. Ten cases performed total hysterectomy, and 17 cases underwent lesion resection. The positive rate of anaplastic lymphoma kinase (ALK) immunohistochemistry reached 96.3%. After a median of 8 months follow-up time, all patients were disease-free and had survived. Uterine inflammatory myofibroblastic tumor is easily misdiagnosed, making its diagnosis challenging. Histological features, immunohistochemical results, and molecular confirmation using fluorescence in situ hybridization (FISH) or Next-generation sequencing should be used to confirm the diagnosis. Positive ALK immunohistochemistry, ALK rearrangement, ALK fusion are helpful in diagnosis and ALK inhibitor therapy. Total hysterectomy is often performed for women who do not require fertility, while lesion resection and close follow-up may be considered for those who require fertility preservation.

Unique Imaging Features in Hepatic Actinomycosis Accompanied by an IgG4-Related Inflammatory Pseudotumor: A Case Report

Unique Imaging Features in Hepatic Actinomycosis Accompanied by an IgG4-Related Inflammatory Pseudotumor: A Case Report

Abdominal inflammatory myofibroblastic tumour: Clinicopathological and molecular analysis of 20 cases, highlighting potential therapeutic targets.

Inflammatory myofibroblastic tumour (IMT) is a rare mesenchymal neoplasm of intermediate malignant potential, occurring at any age and at multiple sites. Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of IMT, typically involving the abdomen. Most IMTs harbour kinase gene fusions, especially involving ALK and ROS1, but 20-30% of IMTs show no detectable translocations. The aim of this study is to further delineate clinicopathological and molecular characteristics of abdominal IMT and discover potential new therapeutic targets. In 20 IMTs, including four EIMS, RNA fusion analysis was performed, followed by multiplex DNA analysis if no ALK or ROS1 fusion was detected. Fourteen IMTs (70.0%) had an ALK translocation and the fusion partner was identified in 11, including a RRBP1::ALK fusion, not previously described in classical (non-EIMS) IMT. RANBP2::ALK fusion was demonstrated in all EIMS. One IMT had a ROS1 fusion. In all ALK/ROS1 translocation-negative IMTs mutations or fusions - as yet unreported in primary IMT - were found in genes related to the receptor tyrosine kinase (RTK)/PI3K/AKT pathway. Three of four patients with EIMS died of disease [mean survival 8 months (4-15 months)], whereas only one of 14 classical IMT patients succumbed to disease [mean follow-up time 52 months (2-204 months); P < 0.01]. This study shows the wide clinical spectrum of abdominal IMTs and affirms the poor prognosis of EIMS, raising discussion about its status as IMT subtype. Furthermore, the newly detected alterations of the RTK/PI3K/AKT pathway expand the molecular landscape of IMTs and provide potential therapeutic targets.

Inflammatory Myofibroblastic Tumour Presenting as Posterior Mediastinal Mass in a Child – A Rare Case Report

Posterior mediastinal tumours in children are difficult to diagnose due to non-specific clinical symptoms. Most of them are neurogenic in origin. However, large pulmonary neoplasms in children may also present as posterior mediastinal masses and may be misinterpreted as neurogenic tumour. We report a case of 6-year female child with recurrent vomiting who had inflammatory myofibroblastic tumour of the right lung which presented as posterior mediastinal mass on computed tomography.

Unraveling the spectrum of inflammatory myofibroblastic tumors in the lung: A comprehensive case series highlighting endobronchial, pleural, and lung parenchymal tumors

ObjectivesDiverse cases of inflammatory myofibroblastic tumors (IMTs) in the lung (pleural, endobronchial, and parenchymal) are presented while discussing the (preoperative) diagnostic challenges and treatment modalities. Other objectives include emphasizing the significance of gene rearrangements and highlighting the multidisciplinary approach in addressing IMTs. MethodsFour cases of IMT in the lung are presented, including a young adolescent girl with an ETV6-neurotrophic tyrosine receptor kinase 3 (NTRK3) gene rearrangement, a 5-year-old boy with challenging preoperative diagnosis, and 2 middle-aged women with respectively pleural and endobronchial tumors with one peribronchial relapse. ResultsThe cases demonstrate the diverse clinical presentations and diagnostic complexities associated with IMT in the lung. Surgical resection remains the primary treatment modality, with complete resection leading to a cure in most patients. Unfortunately, aggressive relapse can occur, as in our last case of an endobronchial tumor. Frozen section may confirm the presence of malignant cells perioperatively and impact further treatment. The presence of gene rearrangements, such as ETV6-NTRK3, suggests potential therapeutic implications. ConclusionsEarly detection and complete surgical removal of IMT are crucial for effective treatment. Identifying gene rearrangements such as ETV6-NTRK3 holds promise for targeted therapies. Diagnostic challenges, including the controversy of biopsies and preoperative evaluations, underscore the importance of a multidisciplinary approach. Anatomopathological recognition of IMT stays demanding. Close surveillance is necessary due to potential relapse, whereas frozen section perioperatively can help further treatment. This case series emphasizes the diagnostic challenges and therapeutic considerations for IMT in the lung.

CASE REPORT: IDIOPATHIC SCLEROSING ORBITAL INFLAMMATION

Idiopathic sclerosing orbital inflammation (ISOI) also known as sclerosing orbital pseudotumor is a rare, idiopathic, chronic, slowly progressive orbital inflammation, characterized by scarring of the tissue that represents a mass effect. The latter leads to bulging of the eye, restriction of ocular movements and visual impairment up to potential loss of vision. It is a subtype of non-specific inflammatory orbital pseudotumor, which both radiologically and clinically mimics a malignant process or other orbital inflammatory disease, and therefore is challenging to diagnose and manage. This case highlights the challenges in diagnosis and management of ISOI.

Renal Inflammatory Myofibroblastic Tumor in an Infant: Case Report with Review of Literature

AbstractInflammatory myofibroblastic tumor (IMT) is an intermediate-grade neoplasm of myofibroblastic lineage occurring due to a cytogenetic clonal abnormality of chromosome 2p23. Pediatric renal IMTs are rare and infant renal IMTs are almost anecdotal. We herein report a 1-year-old female child who presented with a firm mass in the right lumbar region. Biopsy, and later, surgical resection revealed a tumor composed of spindle cells with intermixed plasma cells. On immunohistochemistry (IHC), the lesional cells were positive for smooth muscle actin and anaplastic lymphoma kinase (ALK). A diagnosis of IMT was made based on morphology and IHC. Diagnosis of renal IMTs become challenging especially in a tiny biopsy wherein clear cell sarcoma of kidney, Wilms tumor with predominant mesenchymal component, congenital mesoblastic nephroma, and metanephric stromal tumor are the differential diagnoses in this age group. Renal IMTs generally have better prognosis as compared to extrarenal IMTs. Approximately, 50 to 60% of these tumors harbor ALK gene rearrangement as demonstrated by positivity for ALK IHC. ALK inhibitors like crizotinib or ceritinib can be given for advanced metastatic tumors.

An inflammatory myofibroblastic tumor of the ampulla of Vater, an exceptional location: a case report

Introduction and importance: Inflammatory myofibroblastic tumors constitute a group of mesenchymal tumors associated with inflammatory infiltration. They occur mainly in young patients. It is classified by the World Health Organization as a borderline neoplasm. They are observed in many organs, particularly the lungs. Digestive localization is rare, and localization into the ampulla of Vater has been reported once. Case presentation: We report the case of a 39-year-old patient who was admitted for cholestatic jaundice with right hypochondrium pain. Computed tomography and magnetic resonance imaging revealed a tumor at the biliopancreatic junction. A cephalic duodenopancreatectomy was performed, and a histological examination of the surgical specimen revealed an inflammatory myofibroblastic tumor of the ampulla of Vater. The postoperative evolution was without any complications. Clinical discussion: This is the second case of localization of an inflammatory myofibroblastic tumor in Vater’s ampulla. The therapeutic approach is the complete excision of these inflammatory tumors, thus reducing the risk of local recurrence. In the literature, all cases of incomplete excision have resulted in recurrences. Conclusion: Inflammatory myofibroblastic tumors are rare. The diagnosis was based on histopathological findings and confirmed using immunohistochemical techniques.

A Rare Case of High grade Epithelioid Malignant Neoplasm with CAM 5.2 Expression and (EML4–ALK) Fusion in a Pediatric Patient

Abstract Introduction/Objective ALK (anaplastic lymphoma kinase) fusions have been identified in lymphomas and diverse cancer types including carcinomas, melanomas and mesenchymal neoplasms such as inflammatory myofibroblastic tumors (IMT), epithelioid fibrous histiocytoma (EFH). Herein we present a (non IMT, non EFH) ALK rearranged aggressive epithelioid neoplasm. Methods/Case Report We report a 17-year-old male with a 14.0 cm mediastinal mass and extensive metastatic disease involving brain, bones, pleura and lymph nodes. The tumor was made of epithelioid like cell with round nuclei, prominent nucleoli and a moderate amount of cytoplasm, arranged in a nested pattern. Ki-67 showed a high proliferative index, &amp;gt;70%. The tumor cells were positive for CAM 5.2; weakly positive for AE1/3, ALK, CD30; focally positive for p63, p53, S100. Additional stains including CD117, PLAP, OCT-4, Sall-4, glypican-3, synaptophysin, chromogranin, CK5/6, p40, pan-melanoma, myogenin, CD99, WT1, CD34, CD45, CD138, MUM1, CD2, CD4, CD5, CD3, CD7, CD15, CD23, CD31, CD20, CD79a, CD15, PAX8, PAX5, CD43, MPO, desmin, D2-40, Calretinin, ERG, SOX-10, PD-L1 and NUT-1 were all negative. Both BRG and INI1 were retained. The keratin positivity and the lack of “hallmark” cells made anaplastic large cell lymphoma (ALCL) less likely. The absence of desmin and CD30 excluded the rare epithelioid IMT sarcoma. Next-generation sequencing detected an (EML4–ALK) fusion. A diagnosis of high- grade epithelioid malignant neoplasm with (EML4–ALK) fusion was made. Most cases of this category were described as low grade-appearing spindled cells within a myxoid stroma arranged in fascicles (+S100, +CD34) and have unaggressive behavior. Only three cases, were characterized by epithelioid high- grade morphology and metastatic nature. They shared features like little collagenous stroma, conspicuous mitotic activity and tumor necrosis. Results (if a Case Study enter NA) NA Conclusion Identification of kinase fusions was essential for precise classification, better knowledge of this epithelioid high grade malignant neoplasm and for targeted therapy purposes. The significant clinicopathologic heterogeneity of this emerging group requires further investigation.

Inflammatory pseudotumor of frontal sinus mimicking an aggressive malignancy: a rare case with diagnostic challenge on fine needle aspiration

Abstract Introduction/Objective Inflammatory pseudotumor (IPT) of sinonasal tract is a rare disease that sometimes mimicks malignancy in radiographic findings. Diagnosis of IPT on fine needle aspiration (FNA) is challenging due to limited cellularity and lack of architectural evaluation. Methods/Case Report Patient is a 59-year-old female presented with left front head swelling, facial pain and pressure. Imaging demonstrated a 3.0 cm hypermetabolic frontal sinus mass with osseous erosion through the calvaria table and deviating the adjacent dura, suspicious for malignancy. Also, an incidental 6.8 cm renal mass was identified and was proven to be clear cell renal cell carcinoma. FNA from frontal sinus showed loosely cohesive atypical cells with prominent nucleoli and occasional mitotic activity mixed with lymphoplasmacytic inflammation and eosinophils. Extensive immunostains showed that the lesional cells were only weakly stained for CD10 and desmin. Because of prominent cytologic atypia and radiological concerning for malignancy, FNA was diagnosed as malignancy not further classified. The patient underwent frontal sinus resection and the surgical specimen showed spindle cell proliferation surrounded by reactive woven bone with plump osteoblasts in a myxocollagenous stroma containing mixed lymphoplasmacytic cells and eosinophils. The spindle cells are positive for actin and calponin, negative for ALK. The spindle cells and plump osteoblasts corresponded to the atypical cells observed on FNA. The overall finding is consistent with IPT, although inflammatory myofibroblastic tumor cannot be completely ruled out. During follow up, the patient developed metastatic renal cell carcinoma in lung. Her frontal sinus lesion was stable for 17 months after surgery. Results (if a Case Study enter NA) NA Conclusion IPT of sinonasal tract is a rare lesion that sometimes demonstrate aggressive radiologic features. FNA from IPT poses a diagnostic challenge and can be misinterpreted as malignancy due to prominent cytologic atypia. Being aware of this rare entity helps avoid misdiagnosis and triggers the request for core biopsy during onsite evaluation.

Uterine Inflammatory Myofibroblastic Tumor with ALK Polysomy: Challenging Case Report and Review of Literature

Abstract Introduction/Objective Inflammatory myofibroblastic tumor (IMT) is a distinct neoplastic process with specific molecular alterations. Given its low likelihood of local recurrence and metastasis, it is currently regarded as a neoplasm with intermediate biological potential. IMTs of the uterus variably express CD10 and smooth muscle markers, which present a diagnostic challenge and make it difficult to distinguish from mesenchymal neoplasms of smooth muscle and endometrial stromal origin. Methods/Case Report We present a case of a 46-year-old woman who underwent a hysterectomy due to abnormal menstrual bleeding. Grossly, a 5.0 cm tan, firm, well-circumscribed subendometrial nodule was identified at the fundus, which had a fleshy, soft-cut surface with no hemorrhage or necrosis. Microscopically, the nodule is composed of somewhat tightly packed spindle cells arranged in a fascicular pattern, with little-to-no myxoid stroma and dense lymphoplasmacytic infiltrate forming scattered aggregates. Immunohistochemical stains show lesional spindle cells are positive for SMA, desmin, and vimentin with patchy staining for ER while negative for S100, CD10, ALK-1, STAT6, and CD34. CD68 highlights scattered histiocytes in the background. Ki-67 highlights background lymphoid cells. RNA next-generation sequencing detected no fusions involving ROS1, PDGFR-β, or ETV6, which were previously described in a subset of ALK-negative IMTs. The FISH assay did not reveal rearrangements of the ALK or ROS1. However, extra intact ALK fusion signals were observed, suggesting polysomy for the ALK region. Results (if a Case Study enter NA) NA Conclusion To our knowledge, this is the first report of a uterine IMT with ALK polysomy. The significance of additional ALK copies in our case is not clear but highlights the need for further molecular studies investigating genetic alterations in ALK-negative IMTs. Detecting these rearrangements may be of diagnostic value and help guide future targeted therapies.

Diagnosis of inflammatory myofibroblastic tumor in a pediatric patient initially suspected of tuberculosis

BackgroundSymptoms of inflammatory myofibroblastic tumor (IMT) are atypical, and histopathological misdiagnosis of IMT is still inevitable. Here we present a pediatric case that an eight-year-old boy with recurrent fever for fifteen months, received anti-tuberculosis therapy for five months and was ultimately confirmed to be IMT.Case presentationAn eight-year-old boy experienced a recurrent fever for fifteen months, accompanied by cough, vomiting, meteorism, night sweating, and emaciation. Thoracoabdominal computer tomography revealed multiple enlarged lymph nodes in the thorax, abdomen, and axilla, as well as minimal bilateral pleural effusion. Histopathological examinations of the intestines and greater omentum implied fibrous tissue hyperplasia along with eosinophil and lymphocyte infiltration. The patient was initially misdiagnosed with tuberculosis, and symptoms were relieved partially following anti-tuberculosis treatment. However, after four months, the symptoms aggravated again and a subsequent histopathological analysis of a second sample from the greater omentum revealed the presence of IMT. Eventually, after surgical resection of the lesions and chemotherapy, the clinical symptoms in the child gradually alleviated.ConclusionsThe clinical course of IMT is variable, and pediatricians should pay attention to differentiating IMT from tuberculosis.

An alternative spliced UPF2 transcript in pancreatic inflammatory myofibroblastic tumors

BackgroundInflammatory myofibroblastic tumors (IMTs) are characterized by myofibroblast proliferation and an inflammatory cell infiltrate. Our previous study on IMTs reveals that disrupt NMD pathway causes to lower the threshold for triggering the immune cell infiltration, thereby resulting in inappropriate immune activation. However, myofibroblast differentiation and proliferation is not yet known. MethodsRT-PCR, RT-qPCR, DNA sequence, western bolt, 5′race analysis and site-specific mutagenesis were used in this study. ResultsHere, an alternative spliced (ALS) UPF2 mRNA skipping exon 2 and 3 and corresponding to the truncated UPF2 protein were found in 2 pancreatic IMTs. We showed that the uORF present in the 5′UTR of UPF2 mRNA is responsible for the translation inhibition, whiles ALS UPF2 is more facilitated to be translated into the truncated UPF2 protein. Several mRNA targets of the NMD were upregulated in IMT samples, indicating that the truncated UPF2 function is strongly perturbed, resulted in disrupted NMD pathway in IMTs. These upregulated NMD targets included cdkn1a expression and the generation of high levels of p21 (waf1/cip1), which may contribute to triggering IMTs. ConclusionThe disrupt UPFs/NMD pathway may link to molecular alteration associated with differentiation and proliferation for IMTs.

Crizotinib combined with bronchoscopic interventional treatment in ALK-positive inflammatory myofibroblastic tumor of left main stem bronchus: a case report

BackgroundInflammatory myofibroblastic tumor (IMT), also known as an inflammatory pseudotumor, is a unique type of intermediate soft tissue tumor that commonly occurred in the lung. Its unclear etiology and cellular activity brought about the confusion not only in naming of it, but also in diagnosis and treatment.Case presentationWe reported the case of an 18-year-old male student who suffered from shortness of breath, chest tightness and chest pain. Chest computed tomography scan showed a spherical neoplasm blocking left main stem bronchus. After fiberoptic bronchoscopy procedure, the results of histopathological and immunohistochemical analysis indicated an IMT. The targeted next generation sequencing based genomic profiling of the tumor using formalin-fixed and paraffin embedded tissue was performed and a EML4-ALK fusion was detected. The patient began to receive Crizotinib, a ALK tyrosine kinase inhibitor, at a dose of 250 mg twice daily orally. The patient has recovered well after the operation, and no recurrence or metastasis has been found after 12 months’ follow-up.ConclusionBy means of the diagnosis and treatment of this case, the characteristics and therapies of IMT are illustrated. In addition, it also provides a reference for the therapeutic strategy of IMT in the future.

Pseudotumor inflamatorio ocular asociado a IgG4. Reporte de un caso

IgG4-associated ocular inflammatory pseudotumor is a rare, non-neoplastic inflammatory process with an incidence of 0.28-1.08 per 100,000 people. The diagnosis of this pathology is one of exclusion, having to meet histopathological criteria, enlargement of a specific organ, and serious concentrations of IgG4. Treatment aims to avoid secondary complications. Clinical case: A 54-year-old woman with no previous re-ported pathologies, reports that for the past 6 months she has itching at the level of the eyeball and the left eyelid, suddenly and without apparent cause, in addition to decreased visual acuity and retro-ocular pain; this is why she asked for the evaluation. The physical examination revealed normal eye movements, left proptosis, pain on palpation, subconjunctival hyperemia, corneal opacity, absence of red reflex, and the left eye being more resistant to pressure in relation to the contralateral eye. The cranio-thoraco-abdominal tomography reported images at the left ocular level that were suggestive of choroidal melanoma without findings of metastatic activity. Enucleation of the left eye was performed. The specimen was sent to pathology which reported diffuse lymphoplasmacytic infiltrate positive for IgG4. Conclusion: Ocular IgG4 disease is a very rare pathology with non-specific symptoms and complex diagnosis. However, a quick and correct approach is essential to avoid complications.

Assessment of The Utility of The Sarcoma DNA Methylation Classifier In Surgical Pathology.

Diagnostic classification of soft tissue tumors is based on histology, immunohistochemistry, genetic findings, and radiologic and clinical correlations. Recently, a sarcoma DNA methylation classifier was developed, covering 62 soft tissue and bone tumor entities. The classifier is based on large-scale analysis of methylation sites across the genome. It includes DNA copy number analysis and determines O 6 methylguanine DNA methyl-transferase methylation status. In this study, we evaluated 619 well-studied soft tissue and bone tumors with the sarcoma classifier. Problem cases and typical examples of different entities were included. The classifier had high sensitivity and specificity for fusion sarcomas: Ewing, synovial, CIC -rearranged, and BCOR -rearranged. It also performed well for leiomyosarcoma, malignant peripheral nerve sheath tumors (MPNST), and malignant vascular tumors. There was low sensitivity for diagnoses of desmoid fibromatosis, neurofibroma, and schwannoma. Low specificity of matches was observed for angiomatoid fibrous histiocytoma, inflammatory myofibroblastic tumor, Langerhans histiocytosis, schwannoma, undifferentiated sarcoma, and well-differentiated/dedifferentiated liposarcoma. Diagnosis of lipomatous tumors was greatly assisted by the detection of MDM2 amplification and RB1 loss in the copy plot. The classifier helped to establish diagnoses for KIT-negative gastrointestinal stromal tumors, MPNSTs with unusual immunophenotypes, and undifferentiated melanomas. O 6 methylguanine DNA methyl-transferase methylation was infrequent and most common in melanomas (35%), MPNSTs (11%), and undifferentiated sarcomas (11%). The Sarcoma Methylation Classifier will likely evolve with the addition of new entities and refinement of the present methylation classes. The classifier may also help to define new entities and give new insight into the interrelationships of sarcomas.

SPINAL TUMORS-05 CERVICAL DENDRITIC FOLLICULAR SARCOMA: ABOUT A CASE WITH LITERATURE REVIEW

Abstract Dendritic follicular sarcoma (DFS) is a very rare and often poorly known neoplasia. It develops mainly in peripheral lymph nodes at the expense of dendritic follicular cells. However, extra nodal locations were also found in the literature. Diagnosis is often difficult, based mainly on histopathological and immunohistochemical data. There is currently no consensual therapeutic strategy proposed by the National Understanding Cancer Network. The aim of this work is to focus on the various epidemiological, histopathological, immunohistochemical and therapeutic aspects of the follicular dendritic sarcoma. We report the case of a cervical dendritic follicular sarcoma with literature review. This is a 51-year-old patient with no particular pathological history, consulting for a right lateral cervical mass evolving for 5 years without dyspnea, dysphagia, dysphonia, nor other associated signs evolving in a context of apyrexia and general state conservation. The clinical examination had objectified a firm, fixed, painless right upper jugulocarotid mass without inflammatory signs in sight. The cervical MRI had objectified two contigüe lateral cervical tissue masses well encapsulated, well limited necrotic with mass effect on the larynx without vascular enclosing. The patient had benefited from tumor reduction. Histology was in favor of a dendritic follicular sarcoma. Cervical CT was performed 2 months postoperatively which had shown a mixed right lateral cervical mass with thickened wall and hypodense center pushing in and back the primary carotid and forward the internal jugular vein without any sign of invasion locoregional. The patient then benefited from a complete removal of the tumor mass with ligation of the internal jugular vein. The anatomopathological study had objectified a tumor proliferation made of fusiform cells with nuclei augmented of irregular volume, nucleoli presenting moderate atypies without peri nervous engainement or neoplastic vascular emboli. Immunohistochemistry had shown positive antibodies anti-CD23, anti-CD35, anti-vimentin and anti-PS100. The Ki-67 index was positive on 15% of the cells. This profile concluded in a dendritic follicular sarcoma. The post-operative follow-up was simple. The patient was then referred to oncology for complementary radiation therapy. At 6 months no sign of recurrence was noted. Dendritic follicular sarcoma (DFS) is a relatively rare entity derived from dendritic follicular cells, which are B-cell antigen-presenting cells in the germinal centres of lymphoid follicles. It mainly affects the peripheral lymph nodes especially those of the head and neck. However, extra nodal tissues are affected in about 30% of cases especially the pharynx, oral cavity, thyroid gland. The diagnosis of SFD is difficult and often poorly understood, due to its silent symptomatology, extremely varied morphology and aspecific imaging. The main differential diagnoses are inflammatory myofibroblastic tumors, pseudotumoral tumors, interdigitant dendritic cell sarcomas, lymphomas and undifferentiated carcinomas. Diagnosis is mainly based on pathological examination with immunohistochemical complement.

A rare case of inflammatory myofibroblast tumor of the stomach successfully treated by inverted laparoscopic and endoscopic cooperative surgery

BackgroundAn Inflammatory myofibroblastic tumor (IMT) is a rare intermediate malignancy characterized by myofibroblast proliferation and inflammatory cell infiltration. Various organs are the primary sites of origin. However, primary tumors originating in the stomach tend to be extremely rare, making the diagnosis difficult. Herein, we present a case of IMT originating in the stomach that was effectively managed using inverted laparoscopic endoscopic cooperative surgery (LECS).Case presentationA 47-year-old male who was admitted to the hospital because of a submucosal tumor that was discovered during upper gastrointestinal endoscopy. The diameter of the tumor was approximately 20 mm. A KIT-negative gastrointestinal stromal tumor was suspected based on the biopsy findings. Therefore, partial resection of the stomach was performed using inverted laparoscopic and endoscopic cooperative surgery. Histopathological examination revealed collagen fiber proliferation from the submucosal layer to the muscular layer, accompanied by infiltration of spindle-shaped cells, lymphocytes, and numerous inflammatory cells. Immunohistochemistry results were positive for SMA and negative for CD34, desmin, and c-kit. IgG4-positive cells were observed with an IgG4/IgG ratio > 50%, and specific nuclei were positive for ALK. Therefore, IMT was diagnosed. This condition may be difficult to diagnose both before and after surgery because of its rarity and submucosal tumor-like morphology.ConclusionWhen a submucosal tumor originating in the stomach is observed, IMT should be considered. Partial resection of the stomach with LECS and immunohistochemical diagnosis may be useful.

Inflammatory pseudotumors in the liver associated with influenza: A case report

Patients with liver cirrhosis are advised to limit their sodium consumption to control excessive fluid accumulation. Salt is the most common form in which sodium is consumed daily. Consequently, various recommendations urge patients to limit salt intake. However, there is a lack of consistency regarding salt restriction across the guidelines. Moreover, there is conflicting evidence regarding the efficacy of salt restriction in the treatment of ascites. Numerous studies have shown that there is no difference in ascites control between patients with restriction of salt intake and those without restriction. Moreover, patients with cirrhosis may have several negative effects from consuming too little salt, although there are no recommendations on the lower limit of salt intake. Sodium is necessary to maintain the extracellular fluid volume; hence, excessive salt restriction can result in volume contraction, which could negatively impact kidney function in a cirrhotic patient. Salt restriction in cirrhotic patients can also compromise nutrient intake, which can have a negative impact on the overall outcome. There is insufficient evidence to recommend restricted salt intake for all patients with cirrhosis, including those with severe hyponatremia. The existing guidelines on salt restriction do not consider the salt sensitivity of patients; their nutritional state, volume status and sodium storage sites; and the risk of hypochloremia. This opinion article aims to critically analyze the existing literature with regard to salt recommendations for patients with liver cirrhosis and identify potential knowledge gaps that call for further research.