Xanthogranuloma in the External Auditory Canal in Childhood, an Unexpected Lesion.

Pedunculated lesions in the external auditory canal present a broad differential diagnosis. It is crucial to rule out malignant neoplasms and consider the possibility of xanthogranulomas, which are very rare in this location. Management is determined by associated complications, and otolaryngologists must consider this for appropriate treatment.

Rare primary cardiac myxofibrosarcoma in adolescence: Diagnostic challenges and surgical insights.

This article presents a unique case of primary myxofibrosarcoma, an exceptionally rare cardiac neoplasm, in a 16-year-old girl. With fewer than 40 documented cases, this tumor's occurrence in adolescence is particularly uncommon. The patient presented with right-sided paresthesia and subsequent right hemiparesis and dysarthria, along with bilateral pleural effusion. Imaging tests revealed a cerebral ischemic lesion and the presence of a left atrial mass. Initially suspected as a myxoma, the tumor exhibited significant infiltration. Despite prompt excision and cardiac intervention, the patient experienced rapid decompensation and succumbed to heart failure 2 days later. Histopathological analysis revealed an intermediate grade myxofibrosarcoma. This case underscores the diagnostic complexity, emphasizing the need for early identification given the grim prognosis associated with cardiac myxofibrosarcomas, requiring detailed imaging, surgical, and histopathological insights.

Unusual presentation of biliopancreatic cancer as primary retroperitoneal tumor.

Primary retroperitoneal tumors are little-known neoplasias and consequently, difficult to diagnose. We report an extremely unusual case of a biliopancreatic adenocarcinoma with retroperitoneal localization simulating a primary retroperitoneal tumor. As far as we know, there are no similar cases published up to date.

Unusual presentation of jejunal adenocarcinoma and ovarian metastasis.

It is rare to find a small bowel tumour presenting as intestinal obstruction. This type of cancer is an extremely unusual condition often misdiagnosed until late stages. We report the case of a patient with persistent vomiting secondary to an obstructing jejunal adenocarcinoma not related to intestinal bowel disease. After resection and chemotherapy treatment a huge mass was detected in the left ovary. The anatomopathological findings confirmed a metastatic cancer consequent to the jejunal adenocarcinoma previously resected. This case illustrates a successful outcome of a jejunal adenocarcinoma with very poor prognosis after a extremely unusual ovarian metastasis. It is highly important to suspect other causes than intestinal bowel disease when doing a differential diagnosis in a young patient presenting with clinical symptoms of intestinal obstruction.

"Deciduoid" Change in Uterine Leiomyomas in Pregnancy: Aberrant Expression of Sex Cord Markers Inhibin and Calretinin.

Leiomyomas are common hormone-responsive uterine neoplasms which can exhibit a variety of morphologic changes secondary to hormonal agents such as progestogens. They may increase in size during pregnancy as a result of hormonal stimulation but surprisingly the morphologic features of leiomyomas in pregnancy are not well described in the literature. In this report, we describe the morphologic features of a series of 29 uterine leiomyomas in pregnancy. The features include in decreasing order of frequency infarct-type necrosis, decidualization of the serosal surface, hyalinization, myxoid alteration of the stroma, edema (sometimes with cyst formation), and dystrophic calcification. We also report a feature which we term "deciduoid" change (seen in 10 of 29 leiomyomas) which takes the form of altered smooth muscle cells with an epithelioid morphology with abundant eosinophilic or clear cytoplasm. Furthermore, we show that the "deciduoid" cells commonly exhibit expression of sex cord markers inhibin and calretinin. We speculate on the pathogenesis of the "deciduoid" change which together with its "aberrant" immunophenotype may result in diagnostic problems and consideration of other neoplasms.

Machine Learning Quantification of Intraepithelial Tumor-Infiltrating Lymphocytes as a Significant Prognostic Factor in High-Grade Serous Ovarian Carcinomas.

The prognostic and predictive role of tumor-infiltrating lymphocytes (TILs) has been demonstrated in various neoplasms. The few publications that have addressed this topic in high-grade serous ovarian carcinoma (HGSOC) have approached TIL quantification from a semiquantitative standpoint. Clinical correlation studies, therefore, need to be conducted based on more accurate TIL quantification. We created a machine learning system based on H&E-stained sections using 76 molecularly and clinically well-characterized advanced HGSOC. This system enabled immune cell classification. These immune parameters were subsequently correlated with overall survival (OS) and progression-free survival (PFI). An intense colonization of the tumor cords by TILs was associated with a better prognosis. Moreover, the multivariate analysis showed that the intraephitelial (ie) TILs concentration was an independent and favorable prognostic factor both for OS (p = 0.02) and PFI (p = 0.001). A synergistic effect between complete surgical cytoreduction and high levels of ieTILs was evidenced, both in terms of OS (p = 0.0005) and PFI (p = 0.0008). We consider that digital analysis with machine learning provided a more accurate TIL quantification in HGSOC. It has been demonstrated that ieTILs quantification in H&E-stained slides is an independent prognostic parameter. It is possible that intraepithelial TIL quantification could help identify candidate patients for immunotherapy.

Primary malignant melanoma of the colon and the importance of an adequate anatomopathological analysis.

Primary malignant melanoma of the colon is an extremely rare tumor due to the absence of melanoblasts in this segment of the digestive tract. We report the case of a patient presenting an ulcerated lesion with a neoplastic appearance in the dentate line during a coloscopy. After chemotherapy and radiotherapy, surgical amputation was performed. The study of the surgical specimen described an invasive malignant melanoma located in the submucosa without involvement of the overlying epithelium or the muscularis propria, nor vascular or lymph node invasion, and with tumor free margins. The patient was presented to the melanoma committee, which determited follow up in consultations. This case strengthens the importance of a good anatomopathological study to prevent delays in diagnosis and appropriate treatment.

Pancreatic intraductal papillary mucinous neoplasm evolving into adenocarcinoma. Continual follow-up necessary.

We report a 62-year-old female with chronic pancreatitis after three episodes of severe acute pancreatitis. In 2008, an uneven main pancreatic duct (MPD) of 16 mm was found during an abdominal ultrasound. The follow-up was not continued until 2020, when a dilation of 27 mm of the MPD was discovered at magnetic resonance imaging (MRI). In 2022, another radiological test was carried out showing a dilation of the MPD of 40 mm with an intraductal tumoral lesion of 30 mm at the head of the pancreas). An echo-endoscopy was carried out and a pancreatic-duodenal tumour with a mixed pattern was discovered. Atypic cells were observed at the fine needle punction. A total duodenopancreatectomy was successfully carried out. In the anatomopathological macroscopic study a 14 cm-multi-cystic neoformation was seen and a solid tumour was discovered at the head of the pancreas that infiltrated the wall of the duodenum. In the longitudinal cross-section of the pancreatic body the MPD was seen to be severely dilated. A focal adenocarcinoma in a intraductal papillary mucinous neoplasm (IPMN) was finally diagnosed. Pancreatic lesions are usually asymptomatic and are detected by chance during other imaging tests. The IPMN rates of malignancy have been reported of between 19 to 30%. The management of pancreatic cystic lesions still represents a clinical challenge. As always, the multi-disciplinary management is mandatory in this type of tumour.

Refractory multisystemic sarcoidosis, a diagnosis and treatment challenge: a case report.

BACKGROUND: Sarcoidosis is a multisystemic granulomatous disease of unknown origin. It is characterized by abnormal activation of lymphocytes and macrophages with the formation of granulomas. Most cases have asymptomatic pulmonary involvement. In case of symptoms, they have an excellent response to glucocorticoid therapy. We present a case of sarcoidosis with multi-organ involvement, refractory to multiple treatments including biological. Partial remission was achieved in it. CASE PRESENTATION: We report an interesting case of a 38-years-old Spanish woman treated by Heerfordt's syndrome (uveitis, parotiditis, fever and facial palsy) plus pulmonary hiliar adenopathy. A sarcoidosis diagnosis was confirmed by lung biopsy. She was initially treated with an 8 weeks course of medium dose oral glucocorticoids and tapered over 8 weeks with improvement. After the suspension of glucocorticoids a relapse occurs with severe ocular involvement and suspicion of neurological involvement. The patient received multiple lines of treatment with poor response. Finally, after the combination of cyclophosphamide with infliximab, the uveitis resolved, improving the neurological symptoms. CONCLUSIONS: Sarcoidosis is a benign disease in most cases. In a small percentage of cases behaves aggressively, requiring early diagnosis and immunosuppressive treatment to avoid sequelae. An adequate immunosuppressive therapy based on Anti TNF drugs should be started to minimize damage and improve the quality of life.The choice of treatment depends on the type and severity of the disease.

A rare gastrointestinal tumor: primary gastric melanoma.

Primary gastric melanoma is an exceptional tumour with less than 20 cases described in the literature, as its origin is not entirely clear as the presence of melanocytes in the stomach has not been demonstrated. Symptomatology is non-specific, which prevents its early detection, and it is diagnosed in late stages. We present the case of a patient who was admitted to our hospital for vomiting in coffee grounds with analytical and haemodynamic repercussions. Urgent gastroscopy revealed a gastric lesion suspicious for malignancy, which was histologically confirmed as gastric melanoma. The therapeutic approach to these tumours is complex and they have a very poor prognosis.

Perianal Paget's disease.

The incidence of extramammary Paget's disease (EMPD) is very low. It is very important to distinguish between primary Paget's disease and secondary to another process. An 85-year-old man consulted for the presence of an erythematous plaque located in the anal and gluteal area, confirming Paget cells in the biopsy.

Tumor de células granulares de vulva: un raro tumor genital benigno / Granular cell tumor of vulva: a rare benign genital tumor

Resumen ANTECEDENTES: El tumor de células granulares de la vulva es poco común y de origen neurogénico. Afecta, principalmente, a mujeres entre 60 y 70 años y es más frecuente en la raza negra. CASO CLÍNICO: Paciente de 63 años, con una lesión vulvar indolora y no pruriginosa, en crecimiento. En la exploración se observó una lesión excrecente de 2.5 cm en la región superior del labio mayor izquierdo, dura, vascularizada y con infiltración a 2 cm de profundidad. No se palparon adenopatías sospechosas. Luego del reporte de la biopsia, sugerente de tumor de células granulares, se practicó una escisión completa, con márgenes libres. El estudio inmunohistoquímico se reportó positivo para CD68, S100 y TFE3 lo que confirmó el diagnóstico. Puesto que el índice proliferativo (Ki67) fue inferior al 5% y los márgenes quirúrgicos estaban libres, no se requirió tratamiento adyuvante. La paciente permanece en seguimiento y sin signos de recidiva. CONCLUSIÓN: Si bien los tumores de células granulares de la vulva son poco comunes y casi siempre benignos, deben incluirse en el diagnóstico diferencial de una tumoración vulvar. La inmunohistoquímica es la herramienta más útil para el diagnóstico preciso y su tratamiento de elección es la escisión local amplia, por su tendencia a la recurrencia local.

Abstract BACKGROUND: Granular cell tumor of the vulva is rare and neurogenic in origin. It mainly affects women between 60 and 70 years of age and is more frequent in black women. CLINICAL CASE: A 63-year-old woman with a painless, non-pruritic, growing vulvar lesion. Examination revealed a 2.5 cm excrescent lesion in the upper region of the left labium majus, hard, vascularized and infiltrated to a depth of 2 cm. No suspicious lymph nodes were palpated. After the biopsy report, suggestive of granular cell tumor, complete excision was performed, with free margins. The immunohistochemical study was positive for CD68, S100 and TFE3 which confirming the diagnosis. Since the proliferative index (Ki67) was less than 5% and the surgical margins were clear, adjuvant treatment was not required. The patient remains in follow-up with no signs of recurrence. CONCLUSION: Although granular cell tumors of the vulva are rare and almost always benign, they should be included in the differential diagnosis of a vulvar tumor. Immunohistochemistry is the most useful tool for accurate diagnosis and their treatment of choice is wide local excision because of their tendency for local recurrence.