Recurrent subcutaneous metastasis of a convexity meningioma: a case report and literature review.

Subcutaneous metastasis in the operative route after intracranial meningioma resection is extremely rare. Here we describe the case of a 69-year-old male who was operated on in our department for a convexity meningioma that spread a few years after surgery in the soft tissues next to the craniotomy site. Twenty-one other cases have been found in the medical literature. In this article, we discuss mechanisms of growth, presentation and management of subcutaneous meningiomas.

[An unusual brain lesion]. / Un lésion cérébrale déroutante.

A 31-year-old man, with no past medical history, presented with headaches, sudden loss of vision, right exophtalmia, bilateral papilledema, and fever. Brain imaging noted a right basi-temporal lesion. Excision of the lesion was performed. The histological examination noted a glial tissue with acute inflammatory changes and multinucleated giant cells. Within this infiltrate there were septate and branched hyphae consistent with aspergillosis. These filaments were stained with PAS. The patient died post-operatively.

[Transient thickening of the pituitary stalk followed by a supra-pituitary Langerhansian histiocytosis : consequence or mere coincidence ?] / Cas clinique. Épaississement transitoire de la tige pituitaire suivi d'une histiocytose langerhansienne supra-hypophysaire : conséquence ou simple coïncidence ?

One of the difficult challenges in endocrinology is the etiological diagnosis of isolated thickened pituitary stalk (PS). We report the case of a woman in whom a thickened PS was diagnosed following the onset of central diabetes insipidus revealed by polyuria-polydypsia syndrome of late pregnancy and postpartum. The pituitary exploration showed panhypopituitarism with disconnecting hyperprolactinemia. An etiological investigation for an inflammatory, granulomatous or tumour cause was carried out, but was negative. Postpartum lymphocytic hypophysitis was then retained. However, the course was puzzling with a control pituitary MRI showing disappearance of the PS thickening with paradoxical appearance of a supra-pituitary tumour, the biopsy of which concluded of being a Langerhansian histiocytosis. This paradoxical sequence is unusual and has not been reported before. It called into question the autoimmune lymphocytic origin of the thickened PS, initially considered, and raised the likelihood of a causal relationship between this PS thickening and Langerhansian histiocytosis.

Le diagnostic étiologique d'un épaississement isolé de la tige pituitaire (TP) constitue l'un des grands défis en endocrinologie. Nous rapportons le cas d'une patiente chez qui un épaississement de la TP a été diagnostiqué suite à la survenue d'un diabète insipide central révélé par un syndrome polyuro-polydypsique de fin de grossesse et du post-partum. Le bilan hypophysaire a montré un panhypopituitarisme avec une hyperprolactinémie de déconnexion. Une enquête étiologique à la recherche d'une cause inflammatoire, granulomateuse ou tumorale a été menée et s'est avérée négative. Une hypophysite lymphocytaire du post-partum a alors été retenue. Cependant, l'évolution a été déroutante avec, à l'IRM hypophysaire de contrôle, la disparition de l'épaississement de la TP et l'apparition paradoxale d'une tumeur suprahypophysaire dont la biopsie a conclu à une histiocytose langerhansienne. Cette évolution paradoxale est inhabituelle et n'a pas été rapportée auparavant. Elle a remis en question l'origine lymphocytaire auto-immune de l'épaississement de la TP, retenue initialement, et a soulevé la possibilité d'une relation de cause à effet entre cet épaississement de la TP et l'histiocytose langerhansienne.

Tumor budding is a prognostic factor linked to epithelial mesenchymal transition in pancreatic ductal adenocarcinoma. Study report and literature review.

BACKGROUND: Pancreatic ductal adenocarcinoma (PDAC) has a devastatingly poor prognosis. Surgical resection is undertaken in only 20% of patients. Most of well-known prognostic factors reflect tumor stage more than its biology. So it is important to identify new biological indicators related to survival in order to develop new therapies. OBJECTIVE: To determine the relation between tumor budding and Epithelial Mesenchymal Transition (EMT) and to evaluate their impact on survival for patients after resection of PDAC. METHODS: We herein report a retrospective study of 50 patients with resected PDAC. Tumor budding, immunohistochemical expression of vimentin and other standard factors were correlated with survival using the Kaplan-Meier method and Cox multivariable survival analysis. For tumor budding assessment, an inter-observer variability study was performed using 100 images of tumor slides stained with Hematoxylin & Eosin and Pan-Cytokeratin. RESULTS: Tumor budding was present in all tumors. A substantial agreement between six pathologists was established in distinguishing high-grade from low-grade budding (κ = 0.6 and 0.73 for H&E and PCK images respectively). High-grade budding was identified in 56% of tumors (28/50). It was an adverse prognostic factor independent of tumor size, resection margins status, nodal status and vascular invasion (p = 0.008). Tumor budding was significantly associated with vimentin expression (p = 0.002). CONCLUSIONS: The association of tumor budding with vimentin expression supported the idea that EMT is a key process in PDAC responsible for progression and drug resistance. Consequently, the elucidation of EMT molecular biology and development of new targeted therapy may improve disease outcome.

Embryonal tumor with multilayered rosettes: illustrative case and review of the literature.

BACKGROUND: Embryonal tumor with multilayered rosettes (ETMR) is a very rare entity and has seldom been reported. It has been newly defined tumor entity included in the latest update (revised fourth edition) of WHO 2016 Classification of Tumors of the Central Nervous System which portends a uniform dismal prognosis and survival even with the best of multimodality approaches. ILLUSTRATIVE CASE: This report documents the presentation of a 2-year-old girl with voluminous intracranial ETMR in the right parieto-occipital region. We describe clinical diagnosis, histological aspects, radiological features, and current management of this very aggressive tumor. CONCLUSION: Pediatric intracranial ETMR is a highly aggressive neoplasm, and it should be considered in the differential diagnosis of pediatric brain tumors.

Papillary thyroid tumors: Diagnostic value of CD56 and Cytokeratin 19.

INTRODUCTION: Noninvasive Follicular Thyroid Neoplasm With Papillary-like Nuclear Features (NIFTP) is a tumor composed exclusively of follicles lined by cells having nuclear characteristics of papillary carcinoma. Morphological diagnosis is often difficult especially in the cases in which these nuclear abnormalities are focal. AIM: To investigate the contribution of the immunohistochemical study with anti CD56 and anti cytokeratin 19 in the positive diagnosis of NIFTP. METHODS: This is a diagnostive, retrospective study of 40 thyroid specimens including 15 NIFTP, 10 classical form papillary carcinoma (CPC) and 15 vesicular adenomas (AV), collected over a period of eighteen months (December 2013-May 2015). RESULTS: Negativity with anti-CD56 was noted in 16 cases: 9 cases of NIFTP and 7 cases of CPC. A diffuse staining was noted in 14 cases of AV. Positivity with anti-Cytokeratin19 was noted in 38 cases: 14 NIFTP, 10 CPC and 14 AV. For the diagnosis of NIFTP, the sensitivity of CD56 was 60% and specificity of 100%. The sensitivity of the Cytokeratin 19 was 93,3 % and specificity of 33,3%. CONCLUSIONS: Considering the good sensitivity and specificity of the CD56, it is possible to apply immunohistochemistry for definitive diagnosis of NIFTP and to differentiate it from adenoma. The cytokeratin 19 does not have a big contribution to distinguish between benign and malignant lesions.

Intracranial grade I meningiomas: Confrontation between histological examination and magnetic resonance imaging.

INTRODUCTION: Meningiomas are tumors derived from arachnoid cells. More than 90% of cases have a benign clinical course and are classified as grade I according to the World Health Organization. A confrontation between radiologic findings and pathological examination is necessary to predict the grading of meningiomas. OBJECTIVES: To study the radiological presentation by magnetic resonance imaging (MRI) and pathological features of intracranial meningiomas grade I. METHODS: This was a retrospective descriptive study of a series of 35 cases of grade I meningiomas. A review of MRI images was performed in this study. RESULTS: Our series consisted of 25 female and 10 male patients with a mean age of 49.2 years.The tumor was localized at the base of the skull in 20 cases (57.14%). The average size was 49 mm. At MRI, all meningiomas were solid showing enhancement after injection of contrast agent. This enhancement was homogeneous in 21 cases and heterogeneous in 14 cases. The edema was broad and extended in 15 cases, reduced in 8 cases and absent in 12 cases. The mean minimum apparent diffusion coefficient was 0.77 and the mean maximum average diffusion coefficient was 0,8. On histological examination, the meningioma was of meningotheliomatous type in 23 cases (65.7%), fibroblastic in 8 cases (22.9%), transitional 3 cases (8.6%) and angiomatous in one case (2,8%). CONCLUSION: Although the final diagnosis of meningioma is mainly based on pathological examination, comparison with imaging is also important to orient the pathologist.

Encapsulating sclerosing peritonitis : two rare observations and literature review.

Sclerosing encapsulating peritonitis (SEP) is a rare and little known pathological entity. It is a chronic fibro-inflammatory disease of the peritoneum, resulting in the formation of a thick fibrous membrane, which engages partially or totally the abdominal organs. Clinical and radiological signs make the diagnosis difficult to establish preoperatively. We present two original observations of SEP illustrating different clinical presentations, diagnostic and therapeutic means. His diagnosis requires a peritoneal biopsy. The treatment is not completely established. Surgical treatment is difficult, involving viscerolysis and multiple incisions of the fibrous membrane. Despite the current progress in therapeutic management, the prognosis remains pejorative, with significant mortality.

Neuroendocrine differentiation in basal cell carcinoma.

Basal cell carcinoma (BCC) is the prototypical basaloid tumor of the skin. It may show various patterns simulating other cutaneous tumors due to its pleomorphism. It may have an unusal pattern of differentiation such as squamous, sebaceous, apocrine, eccrine, pilar, and endocrine differentiation. In order to establish the relative frequency of neuroendocrine differentiation in BCC, we performed a retrospective study of 33 consecutive BCCs using conventional immunohistochemistry with two neuroendocrine antibodies: Chromogranine A and synaptophysine. The age of the patients ranged from 17-83 years with mean of 65 years. The male to female ratio was 16:17. In immunohistochimestry, Chromogranine A was seen in 72.2% (24/33) while Synaptophysine was positive in 9.09% (3/33). Their expression was cytoplasmic and membranous and was seen in the periphery of these tumors in the overlying cells. Positive staining of chromogranine A was high (75-100% of tumors cells) in 9%, intermediate (25-75% of tumors cells) in 33% of cases and relatively low (<25%) in 30.3% of cases.

Gastrointestinal neuro-endocrine tumors: retrospective study of 36 cases.

BACKGROUND: Neuroendocrine tumors (NETs) of the gastrointestinal tract are a heterogeneous group of tumors which have different malignant potential and evolution. The World Health Organization (WHO) has set up a new classification of gastrointestinal NETs in 2010. However, it does not evaluate the risk of malignancy for each tumor. AIM: To evaluate the prognostic impact of the WHO classification in 2010 by reclassifying the tumor according to new recommendations and to identify histoprognostic factors to better predict changes. METHODS: This is a descriptive retrospective study of 36 cases of gastrointestinal NETs, collected at the pathological anatomy department in Rabta hospital, over a period of 11 years from 2003 to 2013. RESULTS: Our series included 11 NETs of Appendix, nine of the pancreas, seven of the small bowel, four of the stomach, three of the rectum, one of the colon and one of the esophagus. The mean age of patients across all sites, was 50.3 years old and the sex ratio was 0,44. The tumors were classified according to the 2010 WHO classification. They were divided into: G1 in 27 cases (75%); G2 in three cases (8.3%); G3 small cell carcinoma in two cases (5.6%); G3 large cell carcinoma in one case (2.8%) and mixed adeno-neuroendocrine carcinoma in three cases (8.3%). CONCLUSION: Our study has shown limits of the latest 2010 WHO classification of NETs. The prognosis of these tumors could be better predicted by the evaluation of other histoprognostic factors and by the improvement of criteria defining histological degrees including tumor size.

Prognostic value of Ki67: comparison of the proliferation index between meningiomas with and without infiltration of glial tissue.

BACKGROUND: Although generally considered benign, meningiomas can cause significant morbidity and mortality.  Histologic grade is the most useful morphologic predictor of recurrence. OBJECTIVES: To compare Ki67 labeling index between meningioma grade I and meningioma grade II with brain invasion. METHODS: We analyzed 20 primary meningioma, 10 of which were grade I and 10 of which were grade II. Ki67 proliferative indices were determined in all cases. RESULTS: The patient population consisted of 9 males and 11 females with mean age of 60 years. For meningioma grade I, the Ki67 labeling index varied between 1 and 15% with an average of 3.1%. A diagnosis of gradeII meningiomas was made solely on the basis of brain invasion. The immunohistochemical study noted that the Ki67 index varied between 1 and 20% with an average of 6,8%. CONCLUSION: The Ki67 labeling index shows a significant increase from grade I to grade II.  It may provide useful prognostic information.

Laparoscopic treatment of a cephalic pancreatic leiomyoma.

BACKGROUND: Leiomyoma of the pancreas is very rare. Symptoms and signs are not specific. It has the clinical presentation of a pancreatic mass. The preoperative clinical and radiological assessments are fundamental to establish a therapeutic schema. The curative treatment is surgical resection. A methodical histological examination is required to confirm the final diagnosis of Leiomyoma. CASE REPORT: A 52-year-old female patient presented with a mass of the head of the pancreas. After preoperative assessment, the patient had laparoscopic enucleation. Postoperative course was no remarkable for complications. Pathology examination concluded to leiomyoma. CONCLUSION: Preoperative diagnosis of pancreatic leiomyoma is difficult. It has the features of a pancreatic mass. The preoperative assessment aims to identify signs of malignancy. In its absence, laparoscopy is feasible and safe. Enucleation, if indicated, is a surgical option for a benign disease sparing the patient a pancreatic resection.

Agressive infection following a dental extraction in a diabetic patient :Rhinocerebral mucormycosis.

Mucormycosis is a rare and acute fungal infection which is frequently lethal, usually observed in non-controlled diabetic patients. The infection usually begins in the nose but it can invade the lung, the digestive tract, and the skin. Rhinocerebral mucormycosis accounts for 40 to 49% of mucormycosis cases. We report the case of a 44-year-old diabetic man, presenting with rhinocerebral mucormycosis. Our patient was treated by an association of amphotericin B and surgical debridement.

Malignant transformation of nasal inverted papilloma into sarcomatoid carcinoma.

A 57 year-old, male presented with a chronic unilateral nasal obstruction and epistaxis. Intranasal endoscopy showed multiple polypoid lesions. The computed tomography exam revealed a heterogeneous mass that occupied the right nasal cavity with osteolysis of the middle and lower cone causing fluid retention of the right maxillary sinus. He underwent resection of these lesions. Pathological examination revealed malignant transformation of nasal inverted papilloma into sarcomatoid carcinoma. This case report highlights the importance of considering malignant transformation in the differential diagnosis of polypoid lesions.

Fistulizing nodular lymphoid hyperplasia.

BACKGROUND: Nodular lymphoid hyperplasia (NLH) of the gastrointestinal (GI) tract is a rare condition in adults. It is usually asymptomatic. Few complications have been described. AIM:   We report an unusual clinical presentation of focal lymphoid hyperplasia of the GI. CASE REPORT: A 23-year-old female patient presented with a fistulizingdisease of the terminal ileum and the caecum complicated with an abscess of the lower right quadrant if the abdomen. CT-guided drainage with antibiotic therapy failed to control the abscess. Thus, surgery was undertaken and ileocaecal resection was performed. Focal lymphoid hyperplasia was confirmed by the pathology of the specimen. CONCLUSIONS: NLH is an uncommon condition in adults. To the best of our knowledge, no previous cases have been reported with fistulizing NLH. The management should follow the same algorithm as fistulizing ileitis. Surgery is indicated only in cases of complicated disease after the failure of medical treatment.

A Challenging Cutaneous T-Cell Lymphoma.

Cutaneous peripheral T-cell lymphomas not otherwise specified (CPTL-NOS) are rare neoplasms accounting for just 2% of cutaneous peripheral T-cell lymphomas (CPTL). Only very few case series have been reported. They represent a phenotypically and prognostically heterogenous group of CPTL that do not fit into any of CPTL well-defined subtypes. The authors report a case of a 64-year-old man with simultaneous plaque-like lesions and disseminated nodules growing rapidly on the face, trunk, and extremities over a 6-month period. There was no a history of preceding patches, erythematous plaques, rash, or pruritic lesions. These lesions were extending over 80% of the skin surface. Histopathologic analysis revealed dense diffuse infiltrates composed of mostly medium-sized to large lymphoid cells throughout the entire dermis without epidermotropism. Neoplastic cells were atypical with markedly pleomorphic nuclei. Immunohistochemistry showed that the tumor cells were positive for CD3, CD4, and CD5 with a loss of CD7. They were negative for CD20, CD8, CD56, CXCL13, PD1, TIA-1, granzyme-B, perforin, CD25, and CD30. The proliferative fraction was low, with MIB-1 labeling less than 10% of cells. The authors diagnosed the patient with primary CPTL-NOS. Despite the rarity of these tumors, clinicians as well as dermatopathologists and pathologists should be familiar with these rare CPTL especially because most of these lymphomas have an aggressive behavior and exhibit an unfavorable prognosis.

[Sarcomatoid carcinoma of the lung: retrospective study of 28 cases]. / Carcinomes sarcomatoïdes du poumon: étude rétrospective de 28 cas.

INTRODUCTION: Sarcomatoid carcinoma (SC) of the lung is defined by the World Health Organization as poorly differentiated non-small cell carcinoma that contains a component of sarcoma or sarcoma-like elements. It represents an overall continuum of epithelial and mesenchymal differentiation. Five subtypes are recognized: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma, and pulmonary blastoma. The diagnosis is pathological and requires a good sampling of the tumor. PATIENTS AND METHODS: Twenty-eight cases of primary sarcomatoid carcinoma, diagnosed between 1993 and 2010, were reviewed retrospectively, noting the clinicopathological characteristics. RESULTS: The patient population consisted of 25 males and 3 females with mean age of 62.9 years (48-75 years). The symptomatology was dominated by respiratory symptoms. Imaging features showed a pulmonary mass invading pleura or thoracic wall in 5 cases. The diagnosis was made in all cases on histological examination. These 28 tumors were divided as below: into 19 pleomorphic carcinomas, 4 giant cell carcinomas, 1 spindle cell carcinoma and 4 carcinosarcomas. Twenty-seven tumors were treated surgically. Associated treatments were neoadjuvant (3 cases) or adjuvant chemotherapy (1 case) and preoperative radiotherapy (5 cases). Deaths occurred in 7 patients. Twenty-two patients were lost to follow up. CONCLUSION: These tumors are frequently symptomatic, are locally advanced, and have higher rates of recurrence. Its prognosis is worse than that of other non-small cell lung cancer.