Violaine Yvorel

Intussusception of the Meckel’s diverticulum within its own lumen: Unknown complication

Highlights • It is important to differentiate this rare pathological feature of MD from other entities as the treatment is surgical rather than medical.• No pathognomonic clinical symptoms indicating MD has been reported.• Diagnosis usually is by a technetium Tc 99m-pertechnetate scanning.• The treatment of symptomatic MD is surgical resection (wedge resection of the MD or resection of ileum).• The heterotopic tissue is: gastric, pancreatic, jejuna, duodenal, colonic or hepatobiliary tissue.

PD-L1 expression in pleomorphic, spindle cell and giant cell carcinoma of the lung is related to TTF-1, p40 expression and might indicate a worse prognosis

Lung sarcomatoid carcinoma of the lung is a rare tumor with a poor prognosis. More than 90% of them are pleomorphic, spindle cell and giant cell carcinoma (PSCGCC). This rare subtype of lung cancer is thought to be more resistant to chemotherapy, and a small subset of them seems to exhibit targetable mutations. Immunotherapy against PD1/PDL-1 is a new emerging treatment, and might be of interest in PSGSCC because they frequently express PD-L1. The aim of our work is to evaluate PD1 and PDL-1 expression in a surgical series of lung PSCGCC and their relationship with morphological and immunohistochemical parameters and prognosis. Thirty-six patients who underwent surgical resection of a PSGSCC were included. PD-L1 (E1L3N) expression on tumor cells and PD1 (NAT105) expression by tumor infiltrating lymphocytes (TILs) were performed by immunohistochemistry. Results were compared to immunohistochemistry tests of TTF1, Napsin A, p40 and to molecular study of EGFR, KRAS, BRAF and HER2. Seventy-five % of PSCGCC were considered as positive for PD-L1.PD-L1 expression in PSGSCC is associated with TTF-1 and/or Napsin A expression (47.2%, p = 0.039). Few p40 positive PSCGCC expressed PD-L1 (8.3%, p = 0.013). PD1 expression was not related to TTF-1 and/or Napsin A expression (p = 0.47), p40 expression (p = 0.68) or survival (p = 0.14). PD-L1 or PD1 expression were not related to the age, gender, pT, pN, stage, visceral pleura invasion, histopathological subtype, the presence of giant cell component, the predominance of sarcomatoid component, and the presence of EGFR or BRAF or HER2 or PIK3CA mutation (p>0.05). PD-L1 expression was correlated with a worse overall survival in PSCGCC (p = 0.045). PD-L1 expression is frequent in PSCGCC and might be associated with the expression of adenocarcinoma markers (TTF-1, Napsin A) or the lack of expression of squamous cell carcinoma marker (p40).

Mediastinal Mature Teratoma With Malignant Carcinomatous Transformation (Somatic-Type Malignancy) With Metastatic Course

We report the case of a 76-year-old patient presenting with an anterior mediastinal heterogeneous mass. Surgical biopsy revealed a solid and cystic lesion filled with hair. Pathological examination showed an atypical papillary epithelial lining without other germ cell tumor or immature teratoma associated. The final diagnosis was a mature teratoma of the mediastinum with somatic-type malignancy (carcinomatous transformation). After 8-month follow-up, subcutaneous and lymph node metastatic lesions of the carcinomatous component were identified. Subtyping of the malignant component within germ cell tumors is an important challenge for therapeutic options and prognosis.

First Characterization with Ultrasound Contrast Agent of a Fibrovascular Polyp Before Its Endoscopic Resection: A Case Report (with Videos)

We described for the first time the contrast enhancement of a giant fibrovascular esophageal polyp using ultrasound contrast agent, Sonovue® (Bracco, Milan, Italy) during echoendoscopy. Fine Doppler was unsuccessful in showing vascularization due to the mobile characteristic of the tumor. In contrast, via Sonovue®, tissue microcirculation was highlighted inside the entire head of the polyp, leading to better appreciate the risk of bleeding related to its resection. In a second part, we showed the feasibility of classic polypectomy for this giant polyp (5×5 cm) without complication and results of control endoscopy at 3 months. The present case is summarized in a video.

A 48-YEar-Old Male with a Pituitary Tumor

A 48-year-old patient without significant medical history or without bleeding disorder is addressed to our institution for acute headache and bitemporal hemianopsia. No hormonal secretion was detected, and the patient had a pituitary insufficiency with loss of libido, diffuse hair loss, asthenia and slowing. An MRI showed a 47 mm pituitary lesion occupying the sella turcica with central necrosis. This lesion was hypointense in T1 sequence, enhanced with gadolinium injection and hyperintense in T2 sequence (Figure 1A). Then, a transsphenoidal excision surgery was decided. A first surgery was performed, but the resection of the tumor lead to an extensive bleeding and the tumor could not be resected entirely. Three days later, because of incomplete resection a second excision procedure was decided leading to an extensive bleeding and a subtotal surgical resection. The visual symptoms of the patient improved quickly after the surgery.

Lésion multikystique du péritoine chez un enfant

Une enfant de 2 ans est adressée en consultation pour une altération progressive de l’état général avec une perte de poids de l’ordre de 1 kg en 3 mois. L’examen clinique rapporte une pâleur cutanéo-muqueuse sans notion d’extériorisation ni de fièvre. Le bilan biologique montre une anémie ferriprive, microcytaire sans syndrome inflammatoire biologique. L’échographie abdominale révèle des lésions kystiques multiples et cloisonnées au niveau de la cavité abdominopelvienne (Fig. 1a). La laparotomie exploratrice met en évidence plusieurs formations kystiques de siège sous-diaphragmatique droit, latéro-utérin gauche et au niveau des ligaments larges associées à des petits kystes péritonéaux. Le traitement chirurgical a consisté en une exérèse complète de l’épiploon et de toutes les lésions kystiques visibles. L’examen macroscopique objective des kystes multiples, à paroi fine et contenu séreux clair (Fig. 1b). L’examen microscopique met en évidence des cavités kystiques, séparées par des septa fibreux dépourvus d’amas lymphoïdes ou de fibre musculaire. Le revêtement kystique est souvent cubique, aplati, simple, sans stratification (Fig. 2a). Aucune atypie cytonucléaire ni mitose n’est notée. L’étude immunohistochimique montre un marquage des cellules bordant la cavité kystique par les marqueurs mésothéliaux (anti-HBME1, anti-calrétinine et anti-cytokératine 5/6) (Fig. 2b). Par ailleurs, ces cellules ne sont pas marquées par les anticorps anti-récepteurs hormonaux ni anti-actine musculaire lisse.

B3 thymoma arising within thymolipoma

immunoprofile’ as benign giant cell tumours is a simplistic and inappropriate approach. Nevertheless, a diagnosis of giant cell tumour was adequately justified in our case, supported by accumulative clinicopathological and follow-up data. The tumour was mucosal based with no infiltrative growth, and despite extensive sampling and microscopic examination of the entire tumour, and comprehensive immunohistochemical studies on multiple blocks, no evidence of epithelial differentiation was affirmed. There was no radiological or clinical evidence of locoregional or metastatic disease at the time of diagnosis. Finally, on 20month follow-up to date, the patient is clinically well with no recurrence or metastasis. Spindle cell tumours rich in osteoclast-like giant cellswith low malignant potential are not limited to the gallbladder or biliary tree. The prototypical example is giant cell tumour of the bone and soft tissue, which is an indolent, locally recurrent neoplastic lesion with extremely low risk of metastasis. While giant cell tumours in the bone are generally considered neoplastic based on discovered genetic abnormalities and chromosomal aberrations, it has been argued that these lesions may represent an exuberant fibrohistiocytic reaction topersistent or recurrent haemorrhage in the bone owing to weak stromal support. This hypothetical reaction is mediated by plasma proteins and chemokines which result in activation and proliferation of stromal cells together with recruitment and conformation of monocytic cells into osteoclastic giant cells. Further gene expression modifications and enhanced telomerase activity in susceptible patients results in excessive neoplastic proliferation of mononuclear and multinucleated osteoclast-like giant cells. Synonymously in the ovary and peritoneum, rare occurrences of mucinous cystic tumours with spindle cell sarcoma-like mural nodules containing osteoclast-like giant cells have been reported. Although a subset of these nodules are actually confirmed to be sarcomas or anaplastic carcinomas on morphological, immunohistochemical and gene expression studies, benign spindle cell nodules of genuine reactive nature and indolent clinical behaviour have been reported. Exuberant proliferation of mesenchymal cells underlying the mucinous epithelium as a reactive response to haemorrhage or extruded mucin in the cyst wall, or alternatively a reaction to tumour related chemokines, have been suggested as the theoretical pathogenesis of these nodules. In contrast, the nodules of true sarcomatous or carcinomatous differentiation appear to be a clonal de-differentiation from the pre-existing mucinous tumour. In summary, we have described a rare case of giant cell tumour of the gallbladder, which appears to be a clinicopathologically distinct entity, analogous to the low-grade giant cell rich tumours of the other organs, in particular giant cell tumour of the bone. The exact pathogenesis and biological behaviour of this tumour is not clear due to its rarity. However analogous to giant cell rich lesions in the other organs, it may represent a local exuberant reactive response to persistent or recurrent local damage and haemorrhage in genetically predisposed individuals. Importantly, this tumour should be distinguished from undifferentiated carcinoma with osteoclast-like giant cells, which pursues a more aggressive clinical course. We believe this current case adds to the four previously reported cases of this unique entity.

A case of a Β3 thymoma arising from a “giant” mediastinal thymolipoma

Introduction: Thymolipoma is a rare benign neoplasm of the anterior mediastinum accounting for 2%-9% of thymic tumors. It mainly consists of adipose tissue comporting foci of thymic tissue. Neoplastic transformation of the epithelial component is extremely rare with four cases of thymoma and a case of thymic carcinoid previously reported. Aim: To report a case of thymolipoma associated with B3 thymoma. Case presentation: A 59 year-old woman treated for asthma presented with dyspnea. No mysthenia gravis was present. Imaging revealed a huge mediastinal tumor of fat density compressing the right lung. Surgical resection revealed a 2.8 kg and 39 cm tumor of mainly lipomatous aspect comporting a stellar solid focus of 3 cm. Microscopically, the tumor was a thymolipoma, with a focus (3 cm) of a B3 thymoma. No complementary treatment was applied and the patient is disease-free, two months after operation. Conclusion: This is the first case of a B3 thymoma arising inside a giant thymolipoma. Diagnosis of thymolipoma should be suspected in a fat-density tumor of the anterior mediastinum. Malignant transformation despite rare is possible and resection is mandatory.

Brain pathol case of the month – October 2015 case 2: A 48‐year old male with a pituitary tumor

A 48-year-old patient without significant medical history or without bleeding disorder is addressed to our institution for acute headache and bitemporal hemianopsia. No hormonal secretion was detected, and the patient had a pituitary insufficiency with loss of libido, diffuse hair loss, asthenia and slowing. An MRI showed a 47 mm pituitary lesion occupying the sella turcica with central necrosis. This lesion was hypointense in T1 sequence, enhanced with gadolinium injection and hyperintense in T2 sequence (Figure 1A). Then, a transsphenoidal excision surgery was decided. A first surgery was performed, but the resection of the tumor lead to an extensive bleeding and the tumor could not be resected entirely. Three days later, because of incomplete resection a second excision procedure was decided leading to an extensive bleeding and a subtotal surgical resection. The visual symptoms of the patient improved quickly after the surgery.