Adenoid cystic carcinoma of Bartholin's gland, a case report with genomic data and literature review.

Adenoid cystic carcinoma of the Bartholin's gland (ACCBG) is a rare, slowly but aggressive malignancy. We reported the case of a 31-year-old woman who was treated by local excision and then hemi-vulvectomy, with positive margins and perineural invasion. Radiation therapy (RT) was then performed delivering 45Gy in 25 fractions in bilateral inguinal lymph nodes and 64.8Gy in 36 fractions on the vulvar area. After 30 months, there was no local relapse (LR) but the patient presented a histologically documented lung recurrence. Genomic profiling of the tumor showed a MYB-NFIB fusion transcript and a somatic mutation of PLCG1. A treatment by Lenvatinib was started. We conducted a literature review of 100 published cases. Patients were mainly treated by radical vulvectomy (30%), hemi-vulvectomy (17%), wide or local excision (21% and 24%, respectively) or other. Forty-four percent of patients received postoperative RT, more frequently in case of positive margin (71.9% versus 29.5%). RT may reduce the risk of LR regardless of margin status, with 15.4% vs. 41.9% of LR with or without RT, respectively, in patients with negative margins, and 13% vs. 33.3% of LR with or without RT, respectively, in patients with positive margins. The risk of relapse of any type was 40.9% in patients who received adjuvant RT vs. 48.2% in patients who did not. Median time to relapse was 24 months (range 6-156 months). The most frequent metastatic sites were lung (76.7%) and bone (26.7%). Optimal treatment for ACCBG is still not clearly defined but pooling the data from published case report help us better understand this rare disease and help in the therapeutic decision.

Cumulative radiation dose after lung transplantation in patients with cystic fibrosis.

PURPOSE: The purpose of this study was first to evaluate the imaging-related cumulative post-transplantation radiation dose in cystic fibrosis (CF) lung transplantation (LT) recipients and second, to identify the occurrence and type of malignancies observed after LT. MATERIALS AND METHODS: A total of 52 patients with CF who underwent LT at our institution between January 2001 and December 2006 with at least 3 years of survival were retrospectively included. There were 27 men and 25 women with a mean age of 24.4±9.2 (SD) years (range: 7.6-52.9 years) at the time of LT. Calculation of cumulative effective and organ doses after LT were based on dosimetry information and acquisition parameters of each examination. Cumulative radiation doses were calculated until June 2016, but stopped at the time of de novomalignancy diagnosis, for patients developing the condition. RESULTS: Patients received a mean cumulative effective dose of 110.0±51.6 (SD) mSv (range: 13-261.3 mSv) over a mean follow-up of 8.1±3.6 (SD) years (range: 0.5-13.5 years), with more than 100mSv in 5 years in 19/52 patients (37%). Chest CT accounted for 73% of the cumulative effective dose. Mean doses to the lung, breast and thyroid were 152.8±61.1 (SD) mGy (range: 21.2-331.6 mGy), 106.5±43.2 (SD) mGy (range: 11.9-221.4 mGy) and 72.7±31.8 (SD) mGy (range: 9.5-165.0 mGy), respectively. Nine out of 52 patients (17%) developed a total of 10 de novo malignancies, all but one attributable to immunosuppression after a mean post-transplantation follow-up period of 11.1±3.5 (SD) years (range: 3.7-16.3 years). Six-month cumulative effective dose was not greater in patients with de novomalignancies than in those without de novomalignancies (28.9±14.5 (SD) mGy (range: 13.0-53.4) vs 25.6±15.3 (range: 5.0-69.7), respectively, P>0.05). CONCLUSION: The cumulative effective dose exceeded 100 mSv in 5 years in 37% of LT recipients, the reason why continuous efforts should be made to optimize chest CT acquisitions accounting for 73% of the radiation dose.

[Bronchiectasis imaging]. / Imagerie des dilatations des bronches.

Bronchiectasis are defined as an irreversible focal or diffuse dilatation of the bronchi and can be associated with significant morbidity. The prevalence is currently increasing, probably due to an increased use of thoracic computed tomography (CT). Indeed, the diagnosis relies on imaging and chest CT is the gold standard technique. The main diagnosis criterion is an increased bronchial diameter as compared to that of the companion artery. However, false positives are possible when the artery diameter is decreased, which is called pseudo-bronchiectasis. Other features such as the lack of bronchial tapering, and visibility of bronchi within 1cm of the pleural surface are also diagnostic criteria, and other CT features of bronchial disease are commonly seen. Thoracic imaging also allows severity assessment and long-term monitoring of structural abnormalities. The distribution pattern and the presence of associated findings on chest CT help identifying specific causes of bronchiectasis. Lung MRI and ultra-low dose CT and are promising imaging modalities that may play a role in the future. The objectives of this review are to describe imaging features for the diagnosis and severity assessment of bronchiectasis, to review findings suggesting the cause of bronchiectasis, and to present the new developments in bronchiectasis imaging.

[New TNM classification of non-small cell lung cancer]. / Nouvelle classification TNM des cancers du poumon non à petites cellules.

Initial staging is a key part of the initial evaluation of non-small cell lung cancer. It relies on the 7th edition of the TNM classification. Proposals have been recently developed for the 8th edition of the classification, which is due to be enacted in early 2017. Among these proposals, the weight of tumor size has been increased and new N descriptors have been introduced to further describe N category depending on the number station involved. Regarding M descriptors, oligometastatic disease is distinguished from multiple distant extrathoracic metastases.

[Computed tomography imaging of non-small cell lung cancer]. / Imagerie par tomodensitométrie du cancer bronchique non à petites cellules.

Computed tomography (CT) plays a key role in the initial evaluation of non-small cell lung cancer. It allows initial staging and helps targeting lesions for pathological analysis. The aim of initial imaging work-up is to differentiate between localized disease, eligible to a local treatment, and advanced disease requiring medical treatment. CT is very useful for the assessment of local extension but is less accurate than positron emission tomography (PET)-CT for the assessment of lymphatic and metastatic spread. However, initial staging should include CT examination of the brain and upper abdomen, and PET-CT should be only be performed in patients eligible to a local treatment after initial CT assessment. Propositions for the 8th edition of lung cancer TNM bring several changes for T staging. In particular, the weight of lesion size is increased. Similarly, N1 and N2 stages are now divided in subgroups according the number of involved stations.

The immunophenotype of adult T acute lymphoblastic leukemia in Morocco.

BACKGROUND: There is paucity of detailed studies of adult T cell acute lymphoblastic leukemia (T-ALL) in developing countries reflecting the condition of these patients including clinical and biological features. OBJECTIVE: This study was carried out to analyze the immunophenotypic characteristics of 40 Moroccan patients with T-ALL and its association with biological and clinical features. PATIENTS AND METHODS: Between 2006 and 2009, 130 adult patients diagnosed with acute lymphoblastic leukemia (ALL) were immunophenotyped by 3-color flow cytometry using a panel of monoclonal antibodies. Cases presenting features of a T-lineage phenotype were subjected to detailed analysis including immunophenotypic, clinical and biological parameters. RESULTS: Proportion of T-ALL among ALL Moroccan patients was 31.0%. Median age of patients was 28 years. Twenty-nine patients were females and 11 were males. 45.0% of patients (18/40) had features of immature T-ALL stages (pro-T and pre-T ALL), 30.0% (12/40) of CD1a+ cortical T-ALL stage and 25.0% (10/40) had a characteristic phenotype of medullary T-ALL. The frequencies of progenitor cell markers CD10, CD34 and TdT expression were 14.0; 57.5% and 50.0% respectively. The aberrant expression of B lineage associated antigen CD79a were positive in 20.5% of the cases and the aberrant expression of myeloid antigens CD13 and/or CD33 was found in 22 (55.0%) cases. No significant association was encountered between TdT, CD34 or myeloid antigens positivity and high risk features at presentation as age, sex, and white blood cells. However, myeloid antigens (CD13 and/or CD33) was significantly associated with T-cell maturation stages (p = 0.009). CONCLUSION: To the best of our knowledge, this is the first report from North Africa of immunophenotypic study on adult T-ALL. Our findings indicate that the proportion of T-ALL among ALL in Morocco is similar to that reported in others Mediterranean countries like France and Italy and that myeloid-associated antigens expression is frequently associated with immature immunophenotype.

Clinical importance of myeloid antigen expression in Moroccan patients with adult B-lineage acute lymphoblastic leukemia.

The prognostic significance of myeloid antigen (MyAg) expression in acute lymphoblastic leukemias (ALL), especially in adult patients, is still controversial. In the present report, frequency and clinical significance of MyAg (CD13 and/or CD33) in blast cells were assessed in 80 consecutive adult (≥18 years) patients with B-lineage acute lymphoblastic leukemia (B-ALL), representing 66.7% of 120 patients diagnosed as having ALL during the study period. Immunophenotyping was used to classify leukemic cells as Bor Tlymphoblasts and to identify the aberrant expression of myeloid-associated antigens. MyAg expression was documented in 52.5% of the 80 B-ALL cases analyzed. CD13 was the most commonly antigen expressed (36.3%) followed by CD33 (28.8%). No significant associations were found between the expression of MyAg and the presence of known adverse prognostic features (eg: age>30 years, male gender, high WBC count and Philadelphia positivity). Also, we failed to observe any statistically significant difference between MyAg-positive and MyAg-negative patients in terms of achievement of complete remission and overall survival at 3 years. This study demonstrates that the presence of MyAg on lymphoblastic cells lacks prognostic value In Moroccan patients with adult B-ALL.

[Factor VIII hyperactivity: an arterio-venous thrombogenic factor. Case report]. / Hyperactivité du facteur VIII: un facteur thrombogène artério-veineux. A propos d'un cas.

Thrombosis is the principal mechanism in vascular pathology, whether cerebral, coronary or peripheral. During the initial stages of infarction, coagulation contributes to vascular occlusion, the haemostatic factors playing a determining role in the development of atherothrombotic lesions. An increase in a coagulation protein, besides any lowering of anticoagulation protein levels, is a risk factor for thrombosis. Among these pro-coagulant factors, the pro-thrombogenic action of factor VIII has without doubt been studied the least. We report the case of a 62 year old patient with a personal and family history of many previous thrombotic episodes, both arterial and venous, in whom factor VIII hyperactivity was discovered after a myocardial infarction. This case underlines the association of the factor VIII complex with thrombosis, and its clinical repercussions, especially the incidence of coronary pathology.

[Hydatic kidney cyst: 90 case reports]. / Kyste hydatique du rein: à propos de 90 cas.

The hydatid cyst of kidney is rare, it ranks third among all visceral localisations. The authors report a series of 90 cases renal hydatid cyst from 1972 to 2000. The middle age is 36 years with female predominance. Renal hydatid cyst often has a suggestive clinical presentation; flank mass in 84%, pain in 74% and sometimes a specific presentation hydaturia in 29%. The hydatid serology is positive in 55% and preferring ultrasonography and computed tomography in diagnosis of renal hydatid cyst. Surgical treatment is now well defined. Conservative treatment occupes a predominant place 84% and resection of the proeminent dome is usually efficient. Total nephrectomy should only be considered in the case of a completely destroyed kidney (16%) of cases. Post-operative course is generally uneventful and reexpansion of renal parenchyma is observed in 93% indicating the benign nature of this disease.

[Bladder stone surrounding a foreign body: a case report]. / Lithiase vésicale englobant un corps étranger: à propos d'un cas.

The bladder can be the site of various foreign bodies. We report one case of bladder stone including a foreign body in a 24 years old man with a psychomotor deficiency who was admitted for pyuria, block miction and bladder symptoms. The pelvic X-Ray film showed a bladder stone including a sewing needle. We analysed the diagnosis, aspect and therapeutic management of this case.

[Local recurrence of paratesticular rhabdomyosarcoma]. / Récidive locale d'un rhabdomyosarcome paratesticulaire.

We report an uncommon case of scrotal recurrence of embryonal paratesticular rhabdomyosarcoma in 19 year old man after 3 years later. The diagnosis was suspected clinically and confirmed by histopathology study after resection of the scrotal tumor. About this case, the authors discuss the diagnosis and the management of this tumor.

[Pheochromocytoma and von Recklinghausen's disease]. / Phéochromocytome et maladie de von Recklinghausen.

The association between von Recklinghausen's disease and pheochromocytoma is present about 10% of cases. We report a case of 49 years old women who presented with elevated blood pressure and von Recklinghausen's neurofibromatosis. Laboratory examination showed a marked level in the urinary excretion of cathecholamine. The computed tomography showed a right adrenal tumor suggesting a pheochromocytoma. The adrenalectomy was realised by transabdominal approach and the histological examination confirmed a benign pheochromocytoma. The authors discuss the pathogenetic hypothesis of this rare pathological association, the diagnostic methods and the therapeutic procedure.

[Anatomoradiologic correlations in prostate benign hypertrophy. Report of a series of 220 cases]. / Corrélations anatomoradiologiques dans l'hypertrophie bénigne de la prostate. A propos d'une série de 220 cas.

In our daily practice, the reliability of ultrasonography (US) and digital rectal examination (DRE) is limited by many factors. The aim of our study is to correlate between the prostatic volume assessed by the DRE, the pre operative US results and the actual weight of the enucleated or endoscopic resection benign prostatic hyperplasia (BPH). We report a serie of 220 patients with BPH treated by endoscopic resection or by open surgery. The mean age is 65 years (40-90 years old). Ultrasonography was performed in 92% of the cases, and transrectal ultrasound only in 8%. All our patients either underwent endoscopic resection (49.6%) or adenomectomy (50.4%). We found a high correlation between the prostatic volume assessed by DRE and by ultrasonography (r = 0.79; P < 0.0001) and the weight of the piece of surgical adenomectomy (r = 0.701, P < 0.0001). However, the volume of benign prostatic hyperplasia assessed by US did not correlate with the piece of endoscopic resection (r = 0.214, P < 0.05). This study and a review of recent literature enabled us to notice the various anatomical and radiological factors implicated in this correlation.

[Retrovesical pseudocyst : difficult diagnosis of one case and literature review]. / Pseudokyste rétrovésical: difficulté diagnostique à propos d'un cas et revue de la littérature.

Retrovesical localization of a cystic mass is rare. The authors report one observation of hematic retrovesical pseudocystic formation having a difficult diagnosis. In the light of this observation, different diagnostic patterns of cystic and pseudocystic retrovesical formation are discussed.

[Intradiverticular bladder tumors. Three case reports]. / Les tumeurs de vessie intradiverticulaires. A propos de trois cas.

The bladder tumours in vesical diverticula is rare, and the poor prognosis, because it was often with early invasion. We reported three cases of bladder tumours in vesical diverticula, with delay of diagnosis two, eight and twelve months respectively. The radiology exploration suspected the diagnosis and the histology biopsy confirmed a diagnosis of primary transitional cell carcinoma in two cases: PTa GI and T2 GII, and in an other case it was a invasive epidermoid carcinoma. The first patient was dead by urethral resection of the bladder tumour. The second required a cytoprototectomy and the last patient. The treatment consisted of radiotherapy and chemotherapy. We insisted of the particularity diagnosis, histology and therapeutic for bladder tumour in vesical diverticula and the early diagnosis in order to have a good prognosis.

[Kidney cancer. Report of 170 cases]. / Le cancer du rein. A propos de 170 cas.

PURPOSE: The goal of this study is the analysis of the diagnostic and therapeutic aspects of this affection which is usually discovered at a delayed stage in our context. MATERIAL AND METHODS: A retrospective study of 170 renal cancer, collected during a period of 15 years (1985-2000). Ninety-two men and 78 women were studied, their mean-age was 50 years (15-81 years). The diagnosis was relied on clinical, radiological and histological arguments. RESULTS: Lumbar pain, hematuria and enormous mass were present in 85 cases (50%). The ultrasound and computed exams permitted the diagnosis and also evaluated the venous and locoregional extension. The treatment consisted on large nephrectomy practiced in 114 patients (67%), abstention in 56 cases (33%) due to the advanced stage of the tumor. The histological exam concludes to clear cell adenocarcinoma in 91.2% of cases. CONCLUSION: The authors deplore the delayed diagnosis of renal cancer in our context which is responsible of a high rate of abstention (33%); and underline the interest of the early diagnosis for an adequate management.

[Extra-adrenal pheochromocytoma discovered peroperatively]. / Phéochromocytome extrasurrénalien découvert en peropératoire.

In this study, an uncommon case has been reported of an ectopic pheochromocytoma without the presence of any clinical symptoms. The radiological investigations showed a right retroperitoneal tumor without any kidney involvement. The diagnosis was established by biopsy and subsequent histological findings. In the course of surgery as the large tumor mass was being removed, tachycardia was observed which caused the resection to be performed as rapidly as possible. Once the tumor had been removed, bradycardia occurred, followed by cardiac arrest: although the latter was stabilized after cardiac massage, the patient died one hour after the operative field had been closed. In addition to this case report, the diagnosis, therapeutic strategy and prognosis regarding an ectopically located pheochromocytoma have been discussed.

[Phenotype B primitive adrenal lymphoma, diagnosed by percutaneous aspiration biopsy]. / Lymphome primitif de la surrénale de phénotype B, diagnostiqué à la ponction-biopsie percutanée.

The authors report a case of primary adrenal lymphoma in a 30-year old-female who complained of lumbar pain and was in poor general condition. Ultrasonography and CT scan revealed a heterogeneous mass with necrosis in the left adrenal gland. Non-Hodgkin's lymphoma of B-cell origin was determined by ultrasound-guided aspiration biopsy of the adrenal mass. Taking this case and the findings in the literature into consideration, the features of this disease have been reviewed and the problem of diagnosis, treatment and prognosis have been examined.

[Leiomyoma of the urinary tract]. / Les léiomyomes de la voie excrétrice urinaire.

Leiomyomas of the urinary tract are benign and uncommon forms of tumor. In the present study, two cases have been described of leiomyomas situated in the bladder. Following this description, the pathological characteristics and the diagnostic and therapeutic aspects of these lesions have been examined. The clinical symptomatology depends on the tumor site, and this type of lesion is more frequently found in women.. Treatment mainly consists of endoscopic resection, but may involve cystectomy. The prognosis for patients with this type of tumor is invariably favorable.

[Giant scrotal condyloma acuminata: a case report]. / Condylome acuminé scrotal géant: à propos d'un cas.

In this study, the case has been reported of a scrotal tumor in a 50-year old male admitted with a large warty lesion at the base of the scrotum which resembled a giant condyloma accuminatum (Buschk-Lowenstein tumor). The HIV serology was negative, but the papillomavirus findings were positive. A complete resection of the tumor was performed. The histological findings confirmed the diagnosis of condyloma accuminatum without evidence of degenerative development into a squamous cell carcinoma. The clinical course was favorable, and no local recurrence was observed at 16 months follow-up. The problems that are frequently encountered in the diagnosis and therapy connected with this type of tumor have also been discussed.