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Introduction and Importance: Synovial sarcoma is a high-grade soft tissue sarcoma primarily affecting teenagers and young adults. Inguinal region involvement is rare, making diagnosis and treatment challenging., Case Presentation: A 35-year-old Tunisian man presented with a progressively enlarging right inguinal swelling. Imaging revealed a mass behind the inguinal ligament, confirmed as biphasic synovial sarcoma through biopsy. The patient underwent successful surgery with clear resection margins. Histopathological examination revealed a biphasic sarcoma with spindle cell and glandular components, supporting the diagnosis of synovial sarcoma. Following surgery, the patient received adjuvant radiotherapy. Regular outpatient follow-up is being conducted to monitor progress., Clinical Discussion: Synovial sarcoma is characterized by slow growth and local invasiveness, with potential for metastasis. It typically presents as a solid mass that can compress nearby structures such as blood vessels. Imaging studies offer valuable insights into tumor location, size, invasiveness, and potential metastases. Local tumor staging relies on MRI, while distant metastases are detected using chest CT or bone scans. Diagnosis is confirmed through histopathological examination and immunohistochemical analysis., Conclusions: This case report highlights a rare presentation of inguinal synovial sarcoma and emphasizes the importance of individualized multimodal therapy in its management., Competing Interests: Declaration of competing interest None declared., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Hypophysitis is an extremely rare inflammatory disease that can mimic the clinical and radiological features of a pituitary adenoma. In this case report, we describe a 45-year-old woman with secondary xanthogranulomatous hypophysitis (XGH) who presented with signs of a pituitary macroadenoma. The patient complained of headaches, visual impairment, and amenorrhea-galactorrhea syndrome. Her physical examination was normal. Laboratory investigation revealed corticotropin, thyrotropin, and gonadotropin deficiencies. She also had low visual acuity in her right eye and an altered visual field. Pituitary magnetic resonance imaging revealed an intra and suprasellar mass measuring 13 × 11 × 16 mm, with hemorrhagic necrosis, that was having a discrete mass effect on the patient's optic chiasm and pituitary stalk. The patient was treated with hydrocortisone and levothyroxine, and then transferred to the Neurosurgery department for total transsphenoidal resection of the mass. Histological examination of the tumor permitted a diagnosis of XGH of a remodeled Rathke's pouch cyst to be made. Systemic conditions such as tuberculosis, sarcoidosis, and other granulomatous diseases were excluded. The etiopathogenesis of XGH remains poorly characterized, but it may be a progressive form of lymphocytic hypophysitis or a remodeled Rathke's pouch cyst. Screening for autoimmune pathology and systemic diseases is essential to guide appropriate management., Competing Interests: Declaration of conflicting interestThe authors declare that there is no conflict of interest.

Extrahepatic cancers of the biliary system are typically asymptomatic until after metastasis, which contributes to their poor prognosis. Here we examined intrahepatic cholangiocarcinomas (n = 8), carcinomas of perihilar bile ducts (n = 7), carcinomas of the gallbladder (n = 11) and hepatic metastasis from carcinomas of the gallbladder (n = 4) for the expression of the extracellular matrix glycoproteins tenascin-C and tenascin-W. Anti-tenascin-C and anti-tenascin-W immunoreactivity was found in all biliary tract tumors examined. Unlike tenascin-C, tenascin-W was not detected in normal hepatobiliary tissue. Tenascin-W was also expressed by the cholangiocarcinoma-derived cell line Huh-28. However, co-culture of Huh-28 cells with immortalized bone marrow-derived stromal cells was necessary for the formation and organization of tenascin-W fibrils in vitro . Our results indicate that tenascin-W may be a novel marker of hepatobiliary tumor stroma, and its absence from many normal tissues suggests that it may be a potential target for biotherapies., Competing Interests: Some of the results showed herein have been used in the patent “Tenascin-W and biliary tract cancers” (WO2017072669). The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Hendaoui, Lahmar, Campo, Mebarki, Bichet, Hess, Degen, Kchir, Charrada-Ben Farhat, Hefaiedh, Ruiz, Terracciano, Tucker, Hendaoui and Chiquet-Ehrismann.)

Background: Permanence of front-line management of lung cancer by immunotherapies requires predictive companion diagnostics identifying immune-checkpoints at baseline, challenged by the size and heterogeneity of biopsy specimens., Methods: An innovative, tumor heterogeneity reducing, immune-enriched tissue microarray was constructed from baseline biopsies, and multiplex immunofluorescence was used to profile 25 immune-checkpoints and immune-antigens., Results: Multiple immune-checkpoints were ranked, correlated with antigen presenting and cytotoxic effector lymphocyte activity, and were reduced with advancing disease. Immune-checkpoint combinations on TILs were associated with a marked survival advantage. Conserved combinations validated on more than 11,000 lung, breast, gastric and ovarian cancer patients demonstrate the feasibility of pan-cancer companion diagnostics., Conclusions: In this hypothesis-generating study, deepening our understanding of immune-checkpoint biology, comprehensive protein-protein interaction and pathway mapping revealed that redundant immune-checkpoint interactors associate with positive outcomes, providing new avenues for the deciphering of molecular mechanisms behind effects of immunotherapeutic agents targeting immune-checkpoints analyzed.

Background: The CIC-rearranged sarcoma is a very rare highly aggressive malignant soft tissue group of tumors. It has recently been described as highly aggressive soft tissue tumors of children and young adults sharing similar morphological features with the Ewing sarcoma. The digestive localization is exceptional., Case Presentation: A 14-year-old male presented with a history of abdominal pain for 1 year, which increased in intensity over the last 2 months. Imaging findings showed a large heterogeneous mesenteric mass on the left flank of the abdomen. Exploratory laparotomy was performed and revealed a large cystic hypervascularized mass depending on the transverse colon and mesocolon. A wide excision of the lesion was performed with segmental colectomy. No postoperative complications were noted. The microscopic examination revealed a vaguely nodular growth of undifferentiated small round cells, arranged in solid sheets separated by thin fibrous septa with a scarce stroma. After an uncomplicated post-operative course, the patient was referred for chemotherapy. The patient died 2 months later with a peritoneal and pleural progression., Conclusions: The CIC-rearranged sarcoma is an aggressive tumor. There is no standard therapy for this rare disease. Their treatment includes surgery and chemotherapy. Resistance to chemotherapy is common. Further publications and studies will help to determine a standard therapy for this rare disease.

Study Design: A case report., Objectives: To report an unusual case of multiple spinal metastases from an undiagnosed well-differentiated liposarcoma (WDLPS) of the iliac wing and to stress the need of a meticulous clinical examination and further screening of patients with chronic and asymptomatic bony lesions., Setting: University of medicine of Monastir, Department of neurological surgery, Fattouma Bourguiba University Hospital, Monastir, Tunisia and University of Medicine of Tunis EL Manar, Department of neurological surgery, Tunisian National Institute of Neurology, Tunis, Tunisia., Methods: A 39-year-old man presented with signs of spinal cord compression for the past 2 weeks. His medical history was consistent for an asymptomatic right iliac wing mass that appeared 3 years ago and for which he has not consulted. Magnetic resonance imaging revealed multiple bony lesions of the thoraco-lumbar spine associated with a 6-cm right paravertebral mass at the T4 level extending posteriorly through the intervertebral foramina to the spinal canal causing major spinal cord compression. An emergent T2-T6 laminectomy allowed for a complete resection of the epidural mass. Pathological examination confirmed the diagnosis of well-differentiated liposarcoma. Adjunctive radiation therapy was administered., Results: The patient's neurological status improved remarkably under an intensive care and rehabilitation program. He was ambulatory without assistance in the second postoperative week., Conclusion: The case reported in this paper represents a genuine example of the possible metastatic potential of WDLPSs of the bone and underscores the importance of examining patients thoroughly, especially when they have chronic and asymptomatic lesions.

Sarcoidosis is a multisystem disease of unknown cause and with a worldwide distribution. Involvement of the central nervous system occurs in a relatively small number of patients with sarcoidosis. Isolated neurosarcoidosis without signs of systemic disease however is a rare. In this report, we present an unusual case of neurosarcoidosis with intra cranial mass mimicking radiologically a glioma. Pathological examination revealed intraparenchymatous necrotising granulomatous lesions. After clinicopathological correlation, the diagnosis of a necrotizing cerebral granulomatosis (neurosarcoidosis) with atypical systemic involvement was made. Because of its non-specific clinical presentation and neuroradiological imaging characteristics, intracranial neurosarcoidosis remains a very difficult diagnosis, particularly in the absence of systemic signs of the disease.

Ectopic breast tissue is defined as glands located outside of the breast. Ectopic breast tissue should be excised because it may develop benign (fibroadenoma) or malignant pathologic processes. Less than forty cases of fibroadenomas have been reported in the literature. Although lactation changes can occur, lactating adenoma in the vulva are extremely rare. Only four cases have been reported. We report a case of a young woman who presented with vulvar mass during her lactation. The mass was excised, and histology confirmed vulvar lactating adenoma associated with fibroadenoma. This is the first case of vulvar heterotopic breast lesion associating lactating adenoma and fibroadenoma.

Introduction: Spindle cell oncocytoma of the adenohypophysis is a rare tumour recently reported by Roncaroli et al. in 2002. This tumour is considered a grade I tumour by the World Health Organization., Case Presentation: We describe what is, to the best of our knowledge, the 14th case of its kind in the literature. A 45-year-old African woman presented clinical and radiological findings related to a nonfunctioning pituitary adenoma. The diagnosis was made on the basis of histological and immunohistochemical findings., Conclusion: The purpose of this work is to report a rare pituitary tumour and to describe its histological and immunohistochemical features, which were characterized by the expression of thyroid transcription factor 1 antigen by tumour cells. This fact could support the theory of a possible common origin of these tumours in pituicytomas. In fact, thyroid transcription factor 1 is considered to be a specific marker of pituicytes.

Only 0.5 to 2% of hydatid cysts are localized in the skeleton and of these, 3 to 4% are found in the skull. In this paper, the authors report a case of primary hydatidosis involving the cranial vault revealed by a bulging mass of the forehead and symptoms of raised intracranial pressure that occurred in a 22-year-old woman who came from a rural area. Through this case and literature review, the authors analyse the epidemiological, clinical and radiological aspects of skull hydatidosis. They conclude that hydatid cyst should be considered in the differential diagnosis of any soft tissue swelling or osteolytic lesion in the scalp of patients living in endemic areas.

Sinus histiocytosis with massive lymphadenopathy (SHLM) disease is considered to be an indolent and self-limiting pathology. However, severe morbidity and mortality have been attributed to complications of SHLM. Lower respiratory tract involvement is rarely reported and is often unfavorable, and carries a particularly grave prognosis. A case of SHLM is reported, in which the patient had lower respiratory and pleural involvement.

Endometrial adenofibroma is an uncommon mullerian mixed tumor composed of benign epithelial and mesenchymal components. This tumor must be distinguished from other malignant lesions of the uterus, particularly adenosarcoma. The authors report three cases of endometrial adenofibroma and discuss their clinical and histopathologic features. The tumors were diagnosed in patients 31, 55 and 63 years of age. In all three cases polypoid lesions of 13, 2 and 5 cm, respectively, were found in the uterine cavity. A polypectomy was performed in two cases; one patient underwent hysterectomy. Follow-up was available for two patients who are today alive and well.

Adamantinoma-like Ewing sarcoma (ALES) is a rare malignant tumor characterized by EWSR1::FLI1 related fusions and complex epithelial differentiation. ALES poses a tremendous diagnostic challenge owing to its resemblance to a wide variety of common head and neck malignancies. We aimed to study the clinicopathologic spectrum of ALES diagnosed at our institute. A retrospective review of the clinical and pathologic features of all EWSR1-rearranged ALES cases was performed after confirming the diagnosis. The cases lacking EWSR1 rearrangement were excluded. A total of 7 patients were analyzed. The median age was 27 years (range 7-42 years). There were 4 males and 3 female patients. Tumors were distributed as follows: maxilla (n = 2), parotid (n = 2), nasal cavity (n = 1), ethmoid/maxilla (n = 1), and thyroid (n = 1). Tumor size ranged from 2.2 to 5.5 cm. On microscopy, tumors displayed nested-lobular architecture, monomorphic cells, and interlobular fibrotic stroma. Other features included: palisading (n = 5), squamous differentiation (n = 2), keratinization (n = 1), colonisation of salivary ducts (n = 1) and thyroid follicles (n = 1), follicle-like cysts (n = 3), calcification (n = 2), necrosis (n = 3). Mitotic rate was 4-15/2 mm 2 . On immunohistochemistry, cytokeratins (100%), p40 (100%), strong/diffuse membranous CD99 (100%), NKX2.2 (100%), Fli-1 (71%), and synaptophysin (71%) was positive. Patients received chemotherapy (n = 7) and radiotherapy (n = 4). Two patients developed recurrence at 6 and 10 months; 3 developed metastases at 0, 6, and 25 months. ALES is a rare and aggressive malignancy that mimics diverse neoplasms common in the head and neck region. Awareness of the morphologic and immunohistochemistry spectrum of this tumor is essential to avoid diagnostic errors., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)