UNDIFFERENTIATED SARCOMA ARISING IN AN IMMATURE TERATOMA OF THE OVARY: A case report.

INTRODUCTION: The immature teratomas present less than 1 % of ovarian cancers and affect preferentially young women (1). We report a rare case of an ovarian teratoma harboring undifferentiated sarcoma highlighting the clinical and pathological characteristics. CASE PRESENTATION: The patient was a 24-year-old female who consulted for an abdominal mass. Pelvic ultrasound and magnetic resonance imaging (MRI) revealed an ovarian teratoma measuring 22 cm with no evidence of malignancy. She underwent an exploratory laparotomy. She had a unilateral adnexectomy as the ovarian parenchyma was damaged and the fallopian tube was involved in the mass. The final anatomopathological examination revealed the presence of a teratoma with a predominant component of undifferentiated sarcomatous. This component made the prognosis worse and led to a rapid unfavorable evolution with numerous metastases (lung, liver, and peritoneum). She refused all treatment and died within a week of diagnosis. DISCUSSION: Immature teratoma is a malignant tumor composed of tissues derived from the three embryonic cell lineages at different stages of maturation. This fact makes the determination of its histologic grade by radiologic examination very difficult (1) and consequently raises a challenge in the workup as the prognosis of immature teratomas is closely related to their histologic grade (16). Improved chemotherapy after surgery has increased the 5-year survival rate for these malignancies from 90 % to 100 % (18). CONCLUSION: Young women are most commonly affected by immature teratomas. The prognosis and histologic grade are interrelated. To protect fertility, the trend now is treat them more frequently with conservative surgery.

[Primary benign schwannoma of the pleura: Report of 2 cases]. / Schwannome bénin primitif de la plèvre : à propos de deux cas opérés.

The preferred thoracic location of a schwannoma is the posterior mediastinum. A pleural location is very rare. To date there have been fewer than 20 cases in the literature. We report two operated cases of primary benign pleural schwannoma. The first occurred in a 52-year-old woman who presented with right-sided chest pain and chronic cough. The radiological appearance suggested a hydatid cyst of the lower right lobe. The second case concerned a 37-year-old, asymptomatic man with no past history, who presented with a left posterior mediastinal mass, discovered incidentally on imaging. Both patients underwent complete surgical resection via a posterolateral thoracotomy. The final anatomo-pathological investigation revealed two primary benign schwannomas of the pleura.

A rare case of squamous inclusion cyst in cervical lymph node.

A 38-year-old man, with no history of malignancy, was found to have a 2 cm jugular lymph node, for which a lymph node tuberculosis was suspected. The specimen revealed a cystic structure lined by mature keratinizing squamous epithelium with a prominent granular cell layer consistent with a squamous inclusion cyst in a lymph node, but a metastatic squamous cell carcinoma could not be excluded. This is the first case report of a rapidly enlarging squamous inclusion cyst in a jugular lymph node. Our case demonstrates the diagnostic challenges related to a squamous inclusion cyst in cervical lymph node and serves to inform the readers to consider this lesion in the differential diagnosis for similar situations.

[Clinical and pathological profile of the pleural malignant mesothelioma: A retrospective study about 30 cases]. / Le profil anatomoclinique du mésothéliome pleural malin : une étude rétrospective à propos de 30 cas.

BACKGROUND: The malignant pleural mesothelioma (MPM) is a rare tumour usually associated to asbestos exposure. The delay between the exposure and the occurrence of the cancer can reach 40 years. This caused the pick of incidence described in many countries including Tunisia. The diagnosis is suspected based on clinical features but positive diagnosis is microscopic. Our aim was to describe the clinical and microscopic features of MPM through a single institution experience. PATIENTS AND METHODS: We conducted a retrospective study about 30 MPM diagnosed over a 20-year-period (1995-2015). We included only patients with complete records including clinical, radiologic and microscopic features. All the microscopic diagnoses were reviewed by 2 pathologists. A mean of 12 slides per case was reviewed. The diagnosis was based on the 2015 WHO classification. RESULTS: The mean age of the patients was 61 years, average 22 to 80 years. The sex ratio was 6,5. An asbetose exposition was reported in 21 cases. The most frequent symptoms was chest pain reported in 25 cases. Physical exam was normal in 9 cases. It revealed pleural syndorm in most patients (60 %). Imaging findings consisted mainly in diffuse pleural thickening in 17 cases. Twelve tumours were classified as stage I, 3 stage II, 14 stage III et 1 stage IV. Pleural biopsy was performed using needle in 18 cases, through thoracoscopy in 16 cases, thoracotomy in 3 cases and allowed the diagnosis in respectively 7 cases/18, 16 cases/16 and 3 cases/3. A lymph node biopsy was performed through mediastinoscopy in one case and yelded the diagnosis. The diagnosis was performed on surgical specimen in 2 patients: one bullectomy and one right upper lobectomy. The microscopic exam concluded to an EM in 17 cases, sarcomatoid mesothelioma (SM) in 4 cases and biphasic mesothelioma (BM) in 9 cases. Pan-cytokeratin antibody was used in all cases in association with 2 antibodies with positive diagnostic value and 2 antibodies with negative diagnostic value. It was repeated in 15 cases and the most used antibodies were the anti-calretinin and the TTF1. This was due to the lack of fixation in one case and in order to reach a quality criteria in the other cases. Surgical resection was possible in 2 patients. 15 patients were lost of view after a mean follow-up period of 3 months. Thirteen patients died before or during the follow-up. CONCLUSION: This work was about a Tunisian experience in the diagnosis and management of MPM. The major limits faced were the incomplete databases, the small number of patients included. Microsocpic positive diagnosis necessitates a degree of expertise and every laboratory has to determine the most valuable antibodies through its experience in order to optimize the diagnosis and to reduce the delay of diagnosis.

Unexpected infant death secondary to a pulmonary infiltration due to acute myelocytic leukaemia.

Acute myeloid leukaemia (AML) often presents with non-specific symptoms such as fatigue, anaemia or infection. Pulmonary involvement is uncommon in AML during the course of the disease and is usually caused by infection, haemorrhage, leukaemic pulmonary infiltrates and leukostasis. Lung localization of AML is very uncommon and potentially life threatening if not diagnosed and treated rapidly. The authors describe the sudden death of an asymptomatic five-month-infant because of a misdiagnosed lung localization of AML. Autopsy examination followed by histopathological studies showed an extensive leukostasis and extramedullary leukaemic infiltrating the lungs. Special stains and immunohistochemical studies revealed findings consistent with acute myelogenous leukaemia. This case suggests that underlying acute leukaemia should be considered as a cause of flu-like symptoms in infants. Medical personnel are urged to be alert to fever, sore throat, weakness and dyspnea that may be characteristic of serious systemic diseases.

[Place of the microscopic examination in the diagnosis of costal fibrous dysplasia]. / Place de l'examen anatomopathologique dans le diagnostic de la dysplasie fibreuse costale.

BACKGROUND: Fibrous dysplasia of bone is a rare benign lesion characterized by the coexistence of a fibrous tissue and an immature osteogenesis. Costal localization is rare and may be monostotic or polyostotic. The diagnosis may be suspected based on clinical and radiological findings. Facing the development of radiological investigations, we tried to highlight the diagnostic role of the microscopic examination through the experience of our department. METHODS: We describe a retrospective study about 12 costal fibrous dysplasias diagnosed over a 17-year-period. Clinical records were retrieved from the department of thoracic surgery of the same hospital. RESULTS: Costal fibrous dysplasia is equally observed in men and women with predominance in the third and fourth decades. Clinical symptoms consist mainly in chest pain. Physical examination was normal in almost all cases. Based on the radiological findings, the diagnosis was suspected in 33% of the cases. Microscopic examination highlighted the diagnosis in all cases but it was challenging in one case and necessitated a multi-disciplinary approach. The difficulties encountered were due to artifact decalcification. CONCLUSION: Costal fibrous dysplasia is a benign lesion which diagnosis is based on microscopic features. Radiologic investigations show nonspecific features but allow to rule out a malignant tumor. The outcome of the patients is generally good except in rare cases with a malignant transformation.

[Unusual location of an intrathoracic mesothelial cyst in the posterior and upper mediastinum]. / Une masse inhabituelle du médiastin supérieur et postérieur : le kyste cœlomique.

BACKGROUND: Intrathoracic mesothelial cysts are congenital lesions due to an abnormal development of the pericardial coelom. They are usually asymptomatic and found incidentally on chest radiography or computed tomography. As their classic anatomical location is in the cardiophrenic angle, they are also referred to pleuropericardial cysts. CASE REPORT: A 50-year-old male presented with a history of chest pain. Physical examination and chest X-ray were normal. Computed tomography (CT) scan revealed a cystic lesion in the posterior and upper mediastinum. The cyst was surgically removed through a posterolateral thoracotomy. Histopathological examination confirmed that it was a mesothelial cyst. The surgical resection of the cyst lead to relief of the thoracic pain over a three-year follow-up period. CT-scan showed an aberrant right subclavian artery or arteria lusoria, which is an anomaly of the aortic arch secondary to abnormal embryogenesis. We know no other report of concurrent ectopic coelomic cyst and aberrant right subclavian artery. CONCLUSION: Although the majority of coelomic cysts needs only radiological and clinical follow-up, surgical resection should be performed when the patient is symptomatic or when the diagnosis is uncertain.

The major prognostic factors of thymomas: about a Tunisian study of 100 cases.

AIM: Thymomas are characterised by their rarity, histologic variability and peculiar patterns of recurrence. Herein, we present the experience of a single institution and aim to highlight the major prognostic factors of these tumours. MATERIALS AND METHODS: We present a retrospective study on 100 thymomas diagnosed between 1994 and 2011. Statistical analyses were performed using version 18.0 SPSS. The Kaplan Meier method was used to estimate survival, and survival curves were compared using the Log-Rank test. A p < 0.05 was considered statistically significant. RESULTS: 50 men and 50 women underwent surgical resection for thymoma. Radiologic findings highlighted a diagnosis of thymoma in 51% of cases. The thymomas were classified as stage I in 25 cases, stage II in 47 cases, stage III in 25 cases and stage IV in 3 cases. According to the WHO classification, tumours were classified as type A in 14 cases, type AB in 24 cases, type B1 in 17 cases, type B2 in 20 cases, type B3 in 8 cases, B1/B2 in 8 cases and B2/B3 in 9 cases. The mean survival of patients was 136 months. Age, sex, tumour size, WHO classification and Masaoka stage were evaluated as prognostic factors. Univariate analysis showed that the major prognostic factors were WHO classification (p = 0.019) and Masaoka Stage (p = 0.0001). CONCLUSION: Our results place emphasis on the prognostic value of WHO classification and Masaoka stage in thymomas; in addition, the necessity of improving reproducibility of microscopic classification to avoid discrepancies among prognostic groups is highlighted.

[Tracheal tumor treated as asthma]. / Une tumeur trachéale traitée comme un asthme.

Primary tumors of the trachea are very rare. In adults, the majority of them are malignant. Schwannomas are exceedingly rare benign tumors in the tracheobronchial tree. We report a case of a 37-year-old man who was hospitalized for increasing dyspnea. He had been treated for bronchial asthma for the last 4 years with no benefit. The CT scan of the chest and bronchoscopy identified a tracheal mass that was prolapsed in the left stem bronchus. The patient did not remain free of disease after endoscopic laser resection. So, surgical resection was made. The tumor was excised at its base. A segment of the left stem bronchus was removed and primary anastomosis was performed. The histopathologic diagnosis was of a benign schwannoma without malignant elements. There was no recurrence during the follow-up period. This case demonstrates that intratracheal masses should be considered in patients with dyspnea or in patients with asthma refractory to conventional therapy.

Fibroadenoma in an ectopic vulvar breast gland: a common neoplasm in an uncommon site.

Ectopic breast tissue is defined as glands located outside of the breast. It can be found anywhere along the milk line extending from the axilla to the groin, and can occur in the vulva. Ectopic breast tissue should be excised because it may develop benign or malignant pathologic processes. Less than 40 cases of fibroadenoma in the vulva have been reported in the literature. We report a case of a 37-year-old woman presenting a solitary vulvar mass. The mass was excised completely, and histology demonstrated an ectopic breast fibroadenoma. This is one of the few reports on the benign pathologies of vulvar mammary glands.

[Treatment and prognosis of advanced stage non-small-cell lung cancer]. / Traitement et pronostic du cancer bronchique non à petites cellules au stade avancé.

INTRODUCTION: Lung cancer is the main cause of death from cancer in both men and women worldwide. In 70 to 80% of cases, the diagnosis is made at an advanced stage. Although the management of non-small-cell lung cancer (NSCLC) has continued to improve over the last 5 years, the prognosis remains poor with a 5-year survival rate of about 16%. The aim of this study was to evaluate the management of locally advanced or metastatic NSCLC in our patients and to analyze overall survival (OS) and prognostic factors at these stages. MATERIALS AND METHODS: A retrospective study, including cases of locally advanced and metastatic NSCLC diagnosed in our department between 2008 and 2011. RESULTS: We included 150 patients with a mean age of 60.2 years. The cancer was at stage IIIA in 21% of cases, IIIB in 14% of cases and IV in 65% of cases. Thoracic surgery was performed in 5 patients; 61.4% of patients received chemotherapy and chemo-radiotherapy was given in 21% of patients. Overall survival was 6 months. Better survival was observed in patients aged less than 60 years, having better performance status (PS), having no metastatic mediastinal lymph nodes and patients who received specific anti-tumor treatment. CONCLUSIONS: The prognostic factors in locally advanced and metastatic NSCLC in our patients were: age, PS, status of mediastinal lymph nodes at diagnosis and treatment. These factors should be considered by physicians when treating patients with advanced stage NSCLC.

Primary mediastinal angiosarcoma: a rare observation in a patient with 8-year-survival.

BACKGROUND: Vascular tumours of the mediastinum are rare, accounting for 1-2% of all mediastinal tumours in this location. Angiosarcomas are most often encountered as sporadic lesions, typically in the scalp or face of elderly patients. However, they can occur in any anatomic site. Mediastinal angiosarcomas (MA) are very rare with less than 50 cases reported. CASE REPORT: The authors describe the case of a 38-year-old woman whose past medical history was consistent for a MA that was diagnosed in 2003. This tumour was treated by complete surgical resection followed by radiation therapy and chemotherapy. Diagnosis was based on histologic examination. In 2011, the patient presented a pleural localisation of the angiosarcoma and died one month after admission, 8 years after diagnosis of the MA. CONCLUSION: MA is a very rare tumour causing a diagnostic dilemma. Clinical and radiologic findings are non-specific, and final diagnosis is based on histologic examination. The case described is unusual considering the long period of survival, which may be explained by the treatment modalities associating complete surgical resection, chemotherapy and radiation therapy.

About a challenging tumour, elastofibroma dorsi: an eight-case study.

Elastofibroma dorsi is an uncommon benign soft tissue pseudotumour usually located at the lower pole of the scapula, deep to the serratus anterior and often attached to the periosteum of the ribs. This lesion is usually seen in patients over the age of 50 years and is commonly misdiagnosed as a malignant tumour because of its size and deep location. We describe 8 cases of elastofibroma dorsi diagnosed over a 10-year-period. Our study contained 5 females and 3 males with a mean age at diagnosis of 62.5 years (range 47-75 years). We aim to highlight the clinical and radiologic presentation of elastofibroma dorsi in order to increase awareness of its existence and management.

Diagnosis and clinical course of cardiac myxoma.

Cardiac myxomas are the most common benign tumours of the heart. In spite of their benign nature, these tumours may induce metastasis or recurrences. Their diagnosis is challenging because of the lack of specific signs, and positive diagnosis is based on microscopic findings. We report a case series of 6 patients documented by radiologic and microscopic findings. In addition, one case was unique due to its location in the right atrium. Tumours were detected by trans-oesophageal ultra-sound examination in all cases. They were located in the left atrium in five cases and in the right side in one case. All patients underwent a successful surgical excision with en-bloc removal of the tumour. The outcome was fatal in one patient because of atrial arrhythmia.

About the necessity of improving the current nodal classification of non-small cell lung carcinoma.

BACKGROUND: The current classification of lymph node status in non-small cell lung carcinoma has not been revised since 1997. This fact has prompted many authors to point out the limits of this classification. METHODS: We tried to explore the prognostic relevance of the current TNM classification in comparison with the nodal classification based on the ratio of metastatic lymph nodes (LNR) and the nodal classification based on the number of metastatic LNs (nLN). Additionally, we tried to explore the recommended number of resected LNs. This was done through a retrospective study of 39 cases. We compared the survival curves of patients using the current, RLN and nLN classifications. In the nLN classification, we grouped patients into three categories: nNO (no metastatic LNs), nN1 (1 to 2 metastatic LNs) and nN2 (> 2 metastatic LN). In the LNR classification, we grouped patients into three categories: rNO (0%), rN1 (< or = 12) and rN2 (> 12). Concerning the total number of the resected LNs, patients were categorized into two groups according to the number of LNs: < 10 versus > or = 10 and < 15 versus > or = 15. RESULTS: Our results showed that the LNR classification highlighted a difference in prognosis between the rN1 and rN2 groups. Moreover, survival of patients seemed to be better when the number of the resected LNs was higher. CONCLUSION: The ratio of metastatic LNs seems to be an important prognostic factor, but further studies are necessary to standardize this classification.

[A rare pulmonary lesion association]. / Une association lésionnelle pulmonaire rare.

INTRODUCTION: Pulmonary amyloidoma or nodular amyloidosis is a localized form of amyloidosis, which can mimic a bronchopulmonary carcinoma. This form of amyloidosis may be associated to an infectious, a systemic disease or a lymphoma. OBSERVATION: We describe the case of a 36-year-old patient whose past medical history was consistent for a diabetes mellitus and a hypothyroidism treated by medical treatment. The patient presented a Gougerot-Sjögren syndrome and was explored for non-specific respiratory symptoms. Physical examination was normal. Laboratory tests revealed a monoclonal pic of immunoglobulin. Radiologic findings showed bilateral pulmonary nodules associated to mediastinal lymph nodes. A pulmonary biopsy was performed. Histologic examination revealed a tumoral nodule containing an abundant eosinophilic material, which was acellular and surrounded by a dense lymphomatous infiltrate destroying the pulmonary parenchyma. Histochemical and immunohistochemical study revealed an association of a nodular pulmonary amyloidosis with a MAT pulmonary lymphoma complicating a Gougerot-Sjögren syndrome. CONCLUSION: The association of MALT pulmonary lymphoma and localized amyloidosis is rarely observed in case of Gougerot-Sjögren syndrome. The pathogenesis of this association remains unknown and the management non-consensual because of the rarity of the cases reported. Whereas, it appears that localized amyloidosis associated to a MALT lymphoma seems to have a better prognosis than a disseminated amyloidosis.

The efficacy of bronchial washings in diagnosis of lung carcinoma.

BACKGROUND: The accurate diagnosis of lung carcinoma has become compulsory, especially after the introduction of new targeted therapies. The majority of these patients are non-operable, highlighting the importance of the cytology specimen. Our aim was to assess the diagnostic efficacy of bronchial washings in low income countries where this low cost technique is widely performed. MATERIAL AND METHODS: We conducted a study of 118 bronchial washings collected in the Department of Pathology. Bronchial washings were smeared on 5-6 clean slides. These were fixed in 95% ethyl alcohol for haematoxylin and eosin staining. Cases were retrospectively reviewed by two pathologists (FM and MM) together with the corresponding biopsies. False negative cases were reviewed twice, and the diagnosis was reassessed in one case. We calculated the sensitivity (Se), specificity (Sp), positive predictive value (PPV), negative predictive value (NPV) and Yoden index. CONCLUSIONS: Our study showed a sensitivity of 56%, specificity of 90%, PPV of 55%, NPV of 76% and a Yoden index of 0.45. These results emphasise the diagnostic efficacy of bronchial washings and the possibility of performing molecular tests on cytology specimens.

[A case of costal haemangioma]. / Un cas d'hémangiome costal.

UNLABELLED: Costal primary tumors are rare and dominated by malignant tumors. Haemangioma of the bone represents only 1% of bone tumors. Costal localization accounts only for 1% of the cases and only about fifty cases have been reported in the literature. AIM: The authors aim to describe a rare costal tumor, its histological features and the main differential diagnoses. OBSERVATION: The authors describe the case of a 46-year-old woman who presented with chest pain. Radiological findings did not permit a malignant tumor to be ruled out and the treatment consisted of a resection of the posterior arch of the rib. Microscopic examination concluded that the patient had a costal haemangioma and the patient didn't present any recurrence after a six-year follow-up. CONCLUSION: The costal haemangioma is a very rare tumor with a debated etiology. Some radiological features are specific such as the "soap bubble" or "honeycomb" aspect. However, the basis for diagnosis remains microscopic examination. These tumors have a good prognosis and no cases of recurrence have been reported following complete resection.

Angiolymphoid hyperplasia with eosinophilia: a study of 7 cases.

INTRODUCTION: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign vasoproliferative disease of undetermined origin. It is characterized by the presence of nodular pseudo-tumors corresponding microscopically to a vascular proliferation within an inflammatory infiltrate made up of lymphocytes, macrophages, and eosinophils. The authors describe 7 cases of ALHE. METHODS: The 7 cases were diagnosed over a period of 19 years (1990-2008). Clinical data and histological slides were brought from the departments of dermatology and pathology of the Rabta Hospital. RESULTS: The 7 patients were 4 women and 3 men with an average age of 34.5 years. The cephalic localization was the most frequent. Lesions were solitary or multiple and formed papules or plaques of variable color. The diagnosis was based in all cases on histological findings. DISCUSSION: The main disease in the differential diagnosis of ALHE is Kimura disease, but the 2 entities have several clinical and histological differences. The pathogenesis remains unclear and there is no consensus on the best treatment.