21 years of evolution of primary hepatic neuroendocrine neoplasm in a patient with primary sclerosing cholangitis: A case report.

INTRODUCTION AND IMPORTANCE: Primary hepatic neuroendocrine neoplasms(PHNEN) are among the rarest primitive neuroendocrine neoplasms. Main prognostic factor is histological. We report an unusual case of a PHNEN with 21 years of evolution in the setting of a primary sclerosing cholangitis(PSC). CASE PRESENTATION: A 40 year old man presented in 2001 with clinical signs of obstructive jaundice. CT-scan and MRI showed a 4 cm large hypervascular proximal hepatic mass evoking hepatocellular carcinoma(HCC) or cholangiocarcinoma. Exploratory laparotomy found an aspect of advanced chronic liver disease of the left lobe. Extemporaneous biopsy of a suspicious nodule showed signs of cholangitis. Left lobectomy was performed and postoperatively the patient received ursodeoxycholic-acid and biliary stenting. After 11 years of follow-up, jaundice reappeared with a stable hepatic lesion.A percutaneous liver biopsy was done. Pathology showed a G1 neuroendocrine tumor. Endoscopy, imagery and Octreoscan were normal, supporting the diagnosis of PHNEN. PSC was diagnosed on tumor-free parenchyma. The patient is on liver transplantation waiting list. CLINICAL DISCUSSION: PHNENs are exceptional. Pathology findings, endoscopy and imagery are necessary to rule out an extra hepatic NEN with liver metastasis. While G1 NEN are known for their slow evolution, this 21 year latency is extremely rare. The presence of PSC adds to the complexity of our case. Surgical resection is recommended when possible. CONCLUSION: This case showcases the extreme latency of some PHNEN as well as possible overlap with PSC. Surgery is the most recognized treatment. Liver transplantation seems to be necessary for us, as the rest of the liver shows signs of PSC.

A rare case of chronic penile suppuration associated with fibromatosis.

Spontaneous penile abscess is rare. Without effective treatment, penile abscesses could evolve into a chronic form with fatal consequences.A subtotal penectomy was performed for a 51-year-old man with no medical history who presented a chronic penile suppuration mimicking tumor.

[Pyoderma gangrenosum and systemic lupus erythematosus: A rare association]. / Pyoderma gangrenosum et lupus érythémateux systémique : une association rare.

INTRODUCTION: Pyoderma gangrenosum (PG) is a neutrophilic dermatosis that is traditionally associated with systemic disorders such as chronic inflammatory bowel diseases, rheumatoid arthritis and malignant hematologic disorders. Its association with systemic lupus erythematosus (SLE) is rare and not well known. We report a case of this association with a review of the literature. CASE REPORT: A 43-year-old female patient, followed for 4 years for SLE, presented a deep ulceration of the anterior face of the left thigh with inflammatory borders, an ulcerated nodule of the right shoulder and four small ulcerations of the back of the right hand. The biopsy of the ulceration of the left thigh concluded to PG. The patient was treated by corticosteroids with complete healing of lesions. CONCLUSION: The prognosis of lupus does not seem to be aggravated by PG and the treatments of a SLE flare are usually enough for treating associated PG.

[Ulcerations of the extremities: An unusual manifestation of Behçet's disease]. / Ulcérations des extrémités : une manifestation inhabituelle de la maladie de Behçet.

BACKGROUND: Although cutaneous and mucosal involvement is a major manifestation in Behçet's disease, ulcerated lesions of the extremities are exceptional and poorly known. CASE REPORT: A 57-year-old male patient was diagnosed 7 years ago with Behçet's disease. This diagnosis was made in the presence of recurrent bipolar aphtous ulcers, pseudofolliculitis lesions and retinal vasculitis. After having been lost to follow up for two years, during which his treatment was discontinued, he presented centimetric necrotic ulcerations of the fingers of the hand and of the right big toe. The biopsy revealed leucocytoclastic and necrotizing vasculitis. The patient improved with antibiotic, oral corticosteroids, colchicine and local care. CONCLUSION: Linking extremity ulcers with Behçet's disease, though sometimes difficult, is essential for proper management.

Facteurs prédictifs de malignité des tumeurs oncocytaires de la thyroïde

INTRODUCTION : Les tumeurs oncocytaires (TO) de la thyroïde regroupent les adénomes et les carcinomes. La distinction entre malignité et bénignité constitue une difficulté de taille sur le plan histologique. De cet aspect découlent des contraintes dans l'attitude thérapeutique à adopter. L'objectif du travail était de déterminer les particularités cliniques, pathologiques et pronostiques des TO de la thyroïde et dégager les facteurs prédictifs de malignité. MÉTHODES : Etude descriptive, rétrospective portant sur 35 cas de TO de la thyroïde opérés entre 2007 et 2015. Les prélèvements ont été étudiés au service d'anatomopathologie. Une étude statistique a été réalisée à la recherche des facteurs prédictifs de mali¬gnité.RÉSULTATS : L'âge moyen des patients était de 53 ans. Le sex-ratio a été de 0,09. Le traitement était chirurgical dans tous les cas. L'examen anatomopathologique définitif a confirmé le diagnostic de 26 adénomes oncocytaires, six carcinomes papillaires on-cocytaires et trois carcinomes vésiculaires oncocytaires. La taille moyenne de la tumeur était de 38,1 mm (10-120). Une irathérapie post-opératoire a été proposée pour les carcinomes. En analyse univariée, les facteurs prédictifs de malignité étaient : l'âge supé¬rieur à 55 ans, la consistance dure, les limites floues, la double vascularisation anarchique, le caractère hypoéchogène du nodule, les microcalcifications, la colloïde sombre et dense, la capsule épaisse et la thyroïdite associée. En analyse multivariée, aucun de ces facteurs n'était prédictif de malignité.CONCLUSION : Les tumeurs oncocytaires de la thyroïde représentent une entité anatomo-clinique distincte. La détermination des facteurs prédictifs de malignité est essentielle pour adapter la prise en charge thérapeutique

[Kaposi sarcoma in a patient with ANCA vasculitis requiring hemodialysis]. / Sarcome de kaposi compliquant une vascularite à ANCA en hémodialyse chronique.

INTRODUCTION: Iatrogenic Kaposi's sarcoma is widely reported after transplantation. Less commonly, it occurs in patients receiving immunosuppressive therapy for ANCA associated vasculitis. We report here the rare association of Kaposi's sarcoma, prurigo nodularis and ANCA associated vasculitis in a hemodialysis patient. CASE REPORT: We describe a 58-year-old woman who presented granulomatosis with polyangeiitis with alveolar hemorrhage and renal failure requiring hemodialysis. She developed cutaneous Kaposi's sarcoma seven weeks after the beginning of immunosuppressive therapy. Biological tests showed negative HHV8 virus infection. Lesions of Kaposi's sarcoma responded to a discontinuation of immunosuppressive drugs and a decreasing dosage of corticosteroids. CONCLUSION: Our case showed that the immunosuppressed state related to multiple factors such as underlying disease, immunosuppressive therapy and hemodialysis may all have contributed to the development of this neoplastic disorder in our patient.

[About a rare tumor of the kidney: Malignant mixed epithelial and stromal tumor]. / À propos d'une tumeur rarissime du rein: la tumeur mixte épithéliale et stromale maligne.

Mixed epithelial and stromal tumor of the kidney is a recently described neoplasm that predominantly affects perimenopausal women. Few cases with malignant features have been reported. Here, we report the case of malignant mixed epithelial and stromal tumor of the kidney with sarcomatous transformation arising in a 27-year-old female. She presented with abdominal discomfort. Computed tomography of the abdomen revealed a large compressive mass arisen from the left kidney with solid and focal cystic components. The patient underwent left radical nephrectomy. Histologic sections showed benign and malignant components. The benign component consisted of multiple tubules and variably sized cysts lined by benign epithelium. The malignant component was composed of undifferentiated cellular spindle cell sarcoma. By immunohistochemical studies, the epithelial component was positive for cytokeratins and epithelial membrane antigen (EMA). The stromal component displayed strong immunohistochemical expression of vimentin, CD99, bcl2; and was negative for cytokeratins, desmin, SMA, S-100, estrogen receptor (ER) and progesterone receptor (PR). Analysis by reverse transcriptase polymerase chain reaction (RT-PCR) failed to identify the SYT-SSX1 or SYT-SSX2 fusion transcripts characteristic of synovial sarcoma.

[Paraganglioma of spermatic cord]. / Paragangliome du cordon spermatique.

Paragangliomas can exist anywhere in the distribution of neural crest derived cells. In the spermatic cord, they are exceedingly rare. We report a case of histologically discovery, occurring in a man of 50 years who had a para-testicular mass, painless, firm without other signs associated. In view of this observation and a review of the literature, we propose to establish the epidemiological profile and study the prognostic and the clinicopathologic features of this tumor.

[Primary cutaneous osteosarcoma]. / Ostéosarcome cutané primitif.

BACKGROUND: Osteosarcoma is a malignant tumor of mesenchymatous origin that chiefly affects the metaphysis of long bones. The extraskeletal form of the disease is rare, and even rarer is a cutaneous site, whether metastatic or primary. Herein, we report a new case of primary cutaneous osteosarcoma. PATIENTS AND METHODS: A 54-year-old woman presented a hard subcutaneous nodular tumor on her left arm noted 1 year earlier. The diagnosis of cutaneous osteosarcoma was made on the basis of histological analysis of the lesion, which showed a sarcomatous dermal-hypodermal proliferation secreting osteoid. Clinical and radiological staging ruled out any extra-cutaneous spread, particularly to bone, thus confirming the primary cutaneous nature of the osteosarcoma. DISCUSSION: Primary cutaneous osteosarcoma is a rare tumor, diagnosis of which is normally based on histopathological features.

[Squamous cell carcinoma of the lip: a report of 30 cases]. / Carcinome épidermoïde des lèvres: A propos de 30 cas.

BACKGROUND: Squamous cell carcinoma (SCC) is one of the most common malignant tumors of the lips (90%). The prognosis of these SCC seems to be poor thus here periorificial localization. AIM: To present the epidemiological, clinical, pathological, therapeutic features and out come of SCC of the lips. METHODS: we conducted a retrospective study performed in the dermatology department of the La Rabta hospital of Tunis over a 11-year-period [2000-2010] recording patients with histologically confirmed SCC of lips. RESULTS: Thirty patients were included (26 men and 4 women) with an average age of 63 years. The most frequent risk factors were smoking and chronic sunlight exposure. The occurrence of the labial SCC on a precursor lesion was noted in 11 cases. It occurred more frequently on the lower lip (80%). Tumor was ulcero-vegetant in 21 patients. Twenty patients had a commune SCC, 19 of them were well-differentiated. Surgery was indicated in 18 cases and 10 patients were treated by exclusive radiotherapy. Lymph nodes metastases were noted in 2 cases. No visceral metastasis was observed. During the period of follow-up (20.12 months), two patients died. CONCLUSION: The diagnosis of SCC of the lips is late and the treatment often mutilating. The improvement of the prognosis depends not only on the early diagnosis and the treatment of the precursors, but also on the photo protection and alcohol and smoking eviction.

[Sweet's syndrome: retrospective case series of 47 patients]. / Syndrome de Sweet : étude rétrospective de 47 cas.

PURPOSE: Also called acute febrile neutrophilic dermatosis, Sweet's syndrome is an inflammatory disorder with a prominent cutaneous expression. It is characterized by a variety of manifestations, clinical and histological findings. The objective of this study was to describe their clinical, pathological and therapeutic characteristics. METHODS: We report on a series of 47 patients who presented a Sweet's syndrome, collected in our institution in Tunis between 1997 and 2011. RESULTS: The patient population consisted of 11 men and 36 women. The mean age was 47 years with extreme ranging from 28 to 74 years. An associated disorder was observed in ten patients: inflammatory disease (three cases), inflammatory bowel disease (two cases), tuberculosis (three cases) and diabetes (three cases). One case of pregnancy was observed. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominantly of neutrophils. Fibrinoid necrosis and intramural inflammation were observed in eight cases. CONCLUSION: The skin disorder can precede, follow, or appear concurrent with the diagnosis of an associated disease which requires careful surveillance.

Acute generalized exanthematous pustulosis induced by the essential oil of Pistacia lentiscus.

Acute generalized exanthematous pustulosis (AGEP) is an uncommon pustular eruption characterized by small nonfollicular pustules on an erythematous background, sometimes associated with fever and neutrophilia. Over 90% of cases are drug-induced; however, it can be caused in rare cases by other agents. We report two cases of AGEP secondary to ingestion of Pistacia lentiscus essential oil, the first two such cases to our knowledge. The cutaneous morphology, disease course and histological findings were consistent with a definite diagnosis of AGEP, based on the criteria of the EuroSCAR study group. These two cases highlight the need to consider herbal extracts as a potential rare cause of AGEP and to ensure the safety of herbal medicines.

[Rosai-Dorfman disease: therapeutic issues in 2 cases]. / La maladie de Rosai - Dorfman : enjeux thérapeutiques à propos de 2 observations.

Rosai-Dorfman disease (RDD) is a benign lymphoproliferative disorder characterized by cervical lymph node enlargement with a consistent risk of airway compression and esthetic damage. Extranodal localizations are also described. There is no therapeutic consensus for pediatric forms of RDD. Through 2 pediatric cases with nodal involvement in 1 patient and a sinonasal and soft tissue localization in the other, we focus on the management problems of both nodal and extranodal RDD.

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.

Spindle cell oncocytoma of the adenohypophysis in a woman: a case report and review of the literature.

INTRODUCTION: Spindle cell oncocytoma of the adenohypophysis is a rare tumour recently reported by Roncaroli et al. in 2002. This tumour is considered a grade I tumour by the World Health Organization. CASE PRESENTATION: We describe what is, to the best of our knowledge, the 14th case of its kind in the literature. A 45-year-old African woman presented clinical and radiological findings related to a nonfunctioning pituitary adenoma. The diagnosis was made on the basis of histological and immunohistochemical findings. CONCLUSION: The purpose of this work is to report a rare pituitary tumour and to describe its histological and immunohistochemical features, which were characterized by the expression of thyroid transcription factor 1 antigen by tumour cells. This fact could support the theory of a possible common origin of these tumours in pituicytomas. In fact, thyroid transcription factor 1 is considered to be a specific marker of pituicytes.

Pemphigus vegetans: a clinical, histological, immunopathological and prognostic study.

BACKGROUND: Pemphigus vegetans (P Veg) is a rare clinical form of pemphigus. Studies on P Veg are rare in the literature and none has so far evaluated the prognostic parameters. OBJECTIVE: In this retrospective study of P Veg, we aimed to analyse epidemiological, clinical, immunopathological and therapeutic data. Study of prognostic factors with accuracy of patient survival was also carried out. METHODS: This is a retrospective study (1981-2009) including 17 cases of P Veg. Statistical analysis was performed with chi-square and Fisher tests looking for a possible relationship between clinical data and prognostic factors. Follow-up time and disease-free survival time were estimated using Kaplan-Meier methods. Clinical data were evaluated in univariate analysis looking for a significant association with survival. Equality of survival distribution was studied using log rank test. RESULTS: The hospital prevalence of P Veg was 0.084‰ with a frequency of 9.1% among pemphigus. The mean age at onset was 47.6 years, with a sex-ratio (F/M) about 4.66. Neumann P Veg was the predominant clinical form (11/17). Clinically, the lesions were multifocal (16/17), prevailed on folds and mucous membranes. Under corticosteroids the mean period for healing was 24 ± 9 days. During the follow-up time, three patients died and 11 patients relapsed. Median of overall relapse-free survival was 13 ± 1.7 months. No significant association between clinical data and prognostic factors was found. LIMITATIONS: This study was a retrospective chart analysis and the number of patients was small. CONCLUSION: The P Veg seems to be more frequent in Tunisia with high rate of mortality.

Sweet's syndrome in a patient with rheumatoid arthritis, Sjögren's syndrome and lymph node tuberculosis.

Sweet's syndrome (SS) is an acute neutrophilic dermatosis characterised by abrupt onset of fever, leukocytosis and cutaneous eruption, with dermal neutrophilia on skin biopsy. Most cases are idiopathic but SS can be associated with various affections, especially neoplastic, inflammatory and infectious diseases. The authors report the case of an SS occurring in a patient with a known rheumatoid arthritis associated with a secondary Sjögren's syndrome, with incidental finding of concurrent lymph node tuberculosis. In case of SS, an associated disease (malignant, inflammatory or infectious diseases) must imperatively be searched for, knowing that two or more of these affections can coexist.

[Mycetoma in Tunisia: a 15-case series]. / Les mycétomes en Tunisie: 15 observations.

BACKGROUND: Mycetoma is a chronic infection of cutaneous and subcutaneous tissue that can be caused by fungi or bacteria. It is endemic in tropical and subtropical areas but rare in Tunisia. PURPOSE: The purpose of this report is to describe epidemioclinical features, treatment and outcomes in patients presenting mycetoma in Tunisia. PATIENTS AND METHODS: The files of all patients treated for mycetoma in the Dermatology Department of La Rabta Hospital in Tunisia from 1982 to 2006 were retrospectively reviewed. RESULTS: A total of 15 cases of mycetoma were recorded during the study period. There were 6 men and 9 women with a mean age of 53.2 years. The most common clinical presentation was infiltrated erythematous plaques with sinus tracts (fistulas). Lesions were located on the foot in 12 cases. Thirteen patients reported the presence of grains in fluid discharging from fistulas. The cause of mycetoma was actinomycetes, i.e., Actinomadura madurae, in 9 cases and fungus in 6 cases including 3 due to Madurella mycetomi and 2 to Pseudallesheria boydii. Treatment was based on oral antibiotics for actinomycetoma and oral antifungals for eumycetoma. CONCLUSIONS: Mycetoma in Tunisia is still uncommon with a slight female predominance. The foot is the most frequent location. Diagnosis can be accomplished by direct mycologic examination, culture, and histololgy. There is no consensus on treatment that is often prolonged with numerous relapses.