[Immunohistochemical analysis of mismatch repair proteins in colorectal adenomas]

The deficiency of mismatch repair system is one of the main pathways in colorectal cancer. This system consists mainly of four proteins: MLH1, MSH2, MSH6 and PMS2. Colorectal cancer develops in the majority of cases from precancerous lesions called adenomas. Only few studies have reported on the deficiencies of these proteins in adenomas. In this study we used immunohistochemistry staining in colorectal adenomas to assay functional status of MLH1, MSH2, MSH6, and PMS2 proteins. 102 adenomas from 93 patients were collected in our institution during six years [2007-2012]. The immunohistochemical technique was performed with 4 antibodies: MLH1, MSH2, MSH6 and PMS2. The loss of expression was retained if adenomatous cells were not stained with positive internal control. Staining was considered as abnormal if nucleus of adenomatous cells showed low nuclear staining and / or heterogeneous one, while positive internal control had normal staining. Loss of expression of MSH2 and MSH6 in adenomatous cells was found in only 1 case which was a tubular adenoma 3mm high-grade dysplasia. Abnormal staining of the adenomatous cells was noted in 23 cases [22.5%] for MSH2 and in 8 cases [7.8%] for MSH6. No cases showed loss of expression of MLH1 and PMS2. Abnormal expression of MSH2 and MSH6 was not correlated with sex of patients, the location of the adenoma, its grade of dysplasia and its histological type. Loss of Mismatch repair proteins expression is a rare event in adenomas. However, the abnormal expression levels are higher in our study compared to those reported in the literature. This could reflect a higher rate of microsatellite instability in our patients. Multicenter and larger studies with molecular biology techniques are needed

Primary testicular non-Hodgkin lymphoma. A 10-year Tunisian single-institute experience

Primary testicular non-Hodgkin lymphoma [NHL] is an uncommon extra nodal presentation, accounting for 1% of all NHL and 1 to 9% of testicular neoplasms. Median age at time of presentation is 60 years old. Anthracycline based chemotherapies are most frequently used. To analyze baseline characteristics, treatment modalities and survival of six cases of primary testicular non-Hodgkin lymphoma. We screened 46 testicular neoplasm cases registered from January 1999 to January 2009 and found six primary testicular lymphoma patients. These six cases were analyzed for baseline clinical features, investigations, treatment and outcome variables. Median age was 50 years old and median duration of symptoms was 4 months. All patients had testicular swelling. Four patients had abdominal lymphadenopathy. Most patients had diffuse large B-cell histology. All patients underwent high inguinal orchidectomy and five were treated with anthracycline based chemotherapy. Four patients completed therapy and one of them relapsed two years later. Primary testicular NHL is an uncommon entity and with current combined modality treatment, the outcome may be as good as nodal NHL

Prognostic value of pheochromocytoma of the adrenal gland scaled score [PASS score] tests to separate benign from malignant neoplasms

Differentiating malignant from benign pheochromocytoma has been challenging when based on histologic features. This is due to the definition of malignant pheochromocytoma which are defined by the presence of metastases. A PASS score was developed and according to many authors, a PASS score> =4 identified potentially malignant tumors. To assess the prognostic value of PASS score in differentiating benign pheochromocytomas from malignant ones. The records of 11 patients with tumors diagnosed as "pheochromocytoma" were identified from 1970 to 2010 in the files of the pathology, intern medicine and biochemistry departments of the Charles Nicolle hospital and Pasteur Institute. Receiver operating characteristics [ROC] curve analysis was performed to evaluate the diagnostic performance of PASS. The logistic model was developed using the 11 predictive variables. Its performance was evaluated by calculating the area under the ROC curve and comparing it with that of the PASS. In benign tumors, The PASS score was <4 in 3 cases and >/= 4 in 6 cases. In malignant tumors, the PASS score was >/= 4 in both cases. According to the ROC curve analysis, a PASS equal or superior to 4 identifies malignant pheochromocytoma with a sensitivity of 50% and a specificity of 45%. I think that PASS score, despite its low sensitivity, may help to reserve the more aggressive treatment and narrow follow up for potentially malignant tumors. Widespread of this called score with complete clinical data will help to validate these findings and to add other prognostic factors of value that could be a part of this scaled score such as immunohistochemical findings

Giant trichoblastoma of the scalp

Trichoblastoma is a rare and benign adnexial tumor with characteristic histological features. It occurs on any hair folliclebearing location, and usually presents as a solitary lesion most often less than 2 cm in size. Giant trichoblastoma has been rarely reported in the literature. To report a new case of giant trichoblastoma, misleading for malignancy. A 57-year-old woman presented with a 5 cm-solitary asymptomatic nodular lesion of the scalp, of 28 years. It had been previously excised with recurrence and progressive regrowth. On examination, it was a dome-shaped, erythematous, firm, papillomatous, non infiltrated nodule. Full body work up revealed no metastases. Cutaneous biopsy concluded to trichoblastoma but failed to eliminate malignancy. After excision with secondary skin graft, histological examination confirmed the benignity with clear margins. There was no evidence of recurrence after a 5 year-follow-up period. This case illustrates a rare clinical variant of trichoblastoma with an unusual important size. This can be misleading for malignancy, but the slowly progressive course of the tumour in our patient, together with histological benignity led to the correct diagnosis. This tumour is considered as a distinct entity by some authors

[Rosacea: 244 Tunisian cases]

Rosacea is a common, chronic facial dermatosis of uncertain etiology, several well-defined types and variable progression. There is a paucity of epidemiologic studies from North African countries especially in Tunisia. To determine epidemiological, clinical, histological features, treatment and outcome of rosacea in a Tunisian study. A tretrospective study of all rosacea cases diagnosed in the outpatient Dermatology Department of Charles Nicolle hospital of Tunis was conducted between 1990, January and 2003, May. Our study included 244 patients. The diagnosis of rosacea was made on symptoms and clinical features in 185 cases and on histological findings in 59 patients. The hospital prevalence of rosacea was 0.2%. The sex ratio M/F was 0,4. Patient's Mean age was of 49 years. Triggered factors mentioned included most often: sun exposure [64%] and thermal stimuli [25%]. Mean duration between the onset of symptoms and the first consultation was about 20 months. The rosacea subtypes were: erythematotelangiectatic type [12%], papulopustular rosacea [69%], and rhinophyma [3.7%]. Granulomatous rasacea was diagnosed in 8 patients and steroid rosacea in 28 cases. Ocular rosacea was observed in 41 cases. Treatment modalities included: topical treatment [1.6%], systemic treatment [85%], systemic and topical treatment [5%] and surgical procedures [1.2%]. Rosacea seems to be a frequent dermatosis in Tunisia where most of the population is phenotype IV-V. It affects mostly middle-aged women. Most of our patients present with papulopustular rosacea [69%]. More epidemiological and clinical studies in North African countries should be conducted to emphasize these results

[ case of cutaneous endometriosis developed on abdominal scar]

Endometriosis is characterized by the presence of an ectopic endometrial tissue. It affects between 1% and 5% of women in reproductive age. Its main clinical symptoms are dysmenorrhoea and infertility. Among women having had abdominal surgery for any gynecological reason, the prevalence of endometriosis is between 15% and 50%. We report the case of a 15 year-old patient who underwent surgery twice: once for appendicitis two years before and once for acute salpingitis and tubo-ovarian abscesses one year before. Laparotomy was performed by Pfannenstiel incision. The post operative course was uneventful. The patient reported the gradual emergence of two bluish nodes on the abdominal scar. These nodules became painful and turgid during the menstrual period. She also reported the issue of blood from the abdominal scar during menstruation. The abdominal examination, performed during the menstrual period, showed a scar of good quality and two regular shiny and gluish cutaneous nodules measuring 1 cm in size. These elements were firm and painless. An endometriosis node on the scar of laparotomy was suspected. Surgery was performed in order to remove both nodules. At surgery the depth of nodules reached the top of the abdominal fascia of the major rights. The content of these nodules was chocolate brown. The histo-pathological examination confirmed that these nodules contained ectopic endometrial tissue. The etiopathogeny of endometriosis is still a subject of debate. This case illustrates the possibility of occurrence of endometriosis on abdominal scars after laparotomy: an unusual location

utility of alpha-methyl CoA racemase [P504S] expression as a marker of renal cell carcinomas

Renal cell tumours are numerous and heterogeneous. Because of their clinicopathological heterogeneity, their accurate diagnosis may be challenging. In case of an equivocal diagnosis, immunohistochemistry may be a useful mean of diagnosis. Recently, alpha-methyl CoA racemase has been identified as a useful marker in kidney cancers. Our objectives are to highlight the role of alpha-methyl CoA racemase [AMACR] as a diagnostic marker in papillary renal carcinoma and to assess its utility in the other tumour types. A retrospective review was performed on 62 patients who were treated for renal tumours between January 1994 and November 2005. Immunoreactivity was evaluated with a qualitative manner. Positive AMACR staining was defined as a coarse dense cytoplasmic granularity. The 62 renal tumours were diagnosed as papillary tumours in 22 cases, clear cell tumours in 18 cases, chromophobe carcinoma in 12 cases and oncocytoma in 10 cases among the 22 cases of papillary tumours, all the cases [100%] showed cytoplasmic immunoreactivity staining. 4 cases between the 18 clear cell carcinomas [22%] showed positivity with AMACR. The 12 cases of chromophobe carcinoma didn't express AMACR by immunohistochemistry. Only one case between the oncocytomas [1%] expressed AMACR. This study confirms the high sensitivity of AMACR for papillary renal cell carcinomas but we must keep in mind that weak focal AMACR staining could be present in other renal cell carcinomas

[ exceptional hepatic tumor]

Hepatic localization of non Hodgkin's lymphoma is generally secondary. Primary localizations are rare.To report a rare case of primary hepatic lymphoma particular by its association with dermatopolymyositis. A 55-year-old woman with a past medical history of dermatopolymyositis diagnosed one year before presented with abdominal pain and fever. Laboratory tests showed pancytopenia. Radiologic examination revealed multiple hepatic masses. Surgical biopsy revealed a large B cell hepatic lymphoma. No other localizations were found so the diagnosis of primary hepatic lymphoma was retained. The patient died after a few days due to a severe sepsis. Primary hepatic lymphoma is a rare tumor with a bad prognosis. Its diagnosis is based on histologic examination. Treatment of these tumors remains non consensual

[Microcystic serous pancreatic cystadenoma]

The pancreatic cystic serous neoplasms are divided into two categories: benign serous cystadenoma [SCA] and malignant serous cystadenocarcinoma. Furthermore, based on the macroscopic appearances, SCAs are subdivided into serous microcystic adenomas [SMAs] and serous oligocystic or macrocystic adenomas. Report of a new case of SMA in which we emphasize on the diagnostic difficulties encountered against such tumor despite its relatively radiological easiness compared to the other variants. We report the case of 84 year-old-woman with a microcystic serous cystadenoma of the pancreas which was identified on pathologic examination of the surgical specimen, after unconclusive abdominal ultrasound and computed tomography [CT]-scan. This case describes this rare entity and emphasizes that the diagnosis of such entity is still based on pathological examination after tumor removal

Adreno-cortical oncocytoma: a case report

Adrenal oncocytoma is a very rare lesion, non functioning and benign in most cases. Only 46 cases have been reported in the medical literature. This study aimed to report a new case of adrenal oncocytic tumor with uncertain malignant potential. A 72 year-old- man, consulted for renal fossa pain. Ultrasonography and omputed tomography scan revealed a large mass in the right adrenal gland with extension to the right kidney. A right adrenalectomy and nephrectomy was performed. The diagnosis of adrenal oncocytoma with malignant potential was confirmed by pathology. Patient had a well recovery and left hospital on the fifth day post operatively. He was followed up for 8 months, no tumor recurrence detected. Adreno cortical oncocytoma is a rare tumor. The majority of reported cases had good prognosis

[Parotid basal cell adenoma of membranous type]

Basal cell adenoma [BCA] is a rare benign neoplasm characterized by the basaloid appearance of the tumour cells and the lack of myxochondroid stromal component present in pleomorphic adenoma. We report a case of basal cell adenoma of membranous type, highly suspected of malignancy because of the presence of mediastinal lymph nodes and pulmonary nodules which finally were related to an associated sarcoidosis. Our patient was an 80-year-old woman who presented a swelling of the right parotid two years ago. The clinical examination revealed a solid, indolent and mobile mass. A chest radiography noted mediastinal lymph nodes. The CT-scann confirmed the presence of mediastinal and tracheal lymph nodes with pulmonary nodules. So the diagnosis of metastatic malignant salivary gland tumor was suspected. Finally, the histological examination concluded to a basal cell adenoma of membranous type with sarcoidosis granulomas in the parotid and in the lymph nodes. The BCA is a benign tumor located generally in the parotid gland. When the malignancy is suspected, like in our case, this tumor must be differentiated from the basal cell enocarcinoma using histological criteria

[Multilocular cystic renal cell carcinoma]

Multilocular cystic renal cell carcinoma is an uncommon variant recently described of renal cell carcinoma. Analyze clinicopathological features, imaging findings and prognosis and therapeutic options of this tumor. The authors reported two cases of multilocular cystic renal cell carcinoma arising in 68 and 39 year old men. They were admitted with pain in the lumbar region and/or haematuria. The imaging findings were a renal neoplasm with cystic and solid areas. The pathologic findings confirm the diagnosis of multilocular cystic renal cell carcinoma. Multilocular cystic renal cell carcinoma, usually identified at earlier stages, had slower growth rate and was therefore associated with a better prognosis and longer survival than conventionnal renal cell carcinoma

[Chronic prurigo revealing an angio-immunoblastic T cell lymphoma]

Cutaneous manifestations in angio-immunoblastic T cell lymphoma [AITL] can be seen in almost 50% of patients. They are especially represented by maculo-papular pseudotoxic exanthema. The other manifestations, like nodular prurigo are rarely observed. We report a case of AITL diagnosed after an etiologic screening for chronic prurigo. The objective of our work is to stress on the possibility to diagnose a hemopathy in etiologic screening of chronic pruritus. A 45-year-old patient presented a chronic pruritus of 18 months associated with general manifestations [fever, night sweating and weight loss]. Physical examination showed diffuse adenomegalies. On histology, the lymph nodes were composed of polymorphous lymphoid proliferation made of middle to large sized cells with clusters of epithelioid cells and post-capillary veinules hyperplacia. Immunohistochemical study showed T cell phenotype [CD3+]. Large cells were CD30+. Latent Protein of EBV virus was not expressed. Molecular biological analysis of a lymph node showed a T cell clonal proliferation. Cutaneous biopsy showed a little dermic perivascular lymphocytic inflammatory infiltrate. The diagnosis of angio-immunoblastic T cell lymphoma was made. The abdomino-pelvian CT scanner showed multiple inter-aortico-cave lymph nodes and a splenomegaly. A polychemotherapy was initiated. In front of chronic prurigo with general manifestations, a careful etiologic screening should be done to detect internal disorders especially malignant hemopathies