Paraneoplastic scleroderma in Kaposi's sarcoma: Report of two cases.

Kaposi's sarcoma (KS) is a proliferative and multifocal disease with a double vascular and fibroblastic cell component, of mucocutaneous and visceral expression. It is a multifocal tumoral process, hyperplastic in nature without metastatic potential, induced by the human herpes virus 8 (HHV8). Paraneoplastic syndromes (PS) in KS are rare, with only a small number of cases reported and we have found no previous descriptions of a paraneoplastic scleroderma in KS in the literature. We report the cases of two patients with this atypical PS.

Atypical cutaneous tuberculosis with an unusual course.

Cutaneous tuberculosis represents the fifth extrapulmonary form in Morocco after pleural, lymph node, urogenital, and intestinal tuberculosis. It is recognized to form a continuous immunopathologic spectrum, ranging from a high intensity to a low intensity of cell-mediated immunity, which explains the multiplicity and heterogeneity of anatomo-clinical forms. Association of multiple forms in the same patient is really rare. In this regard, we report a case of scrofuloderma on axillary tuberculosis adenitis associated to a lupus vulgaris in an immunocompetent patient, which was confirmed by histology, QuantiFERON-TB Gold test, and polymerase chain reaction. He received an antituberculous therapy with clinical regression of the lesions. In conclusion, cutaneous tuberculosis is still endemic in developing countries.The diagnosis is difficult because of its clinical polymorphism. That's why it's should be suspected clinically in the presence of any destructive or verrucous skin lesion evolving without healing for a long period and confirmed by bacteriological examinations and histology.

[Bladder AA amyloidosis: A rare location. About a case]. / Amylose AA vésicale : une localisation rare. À propos d'un cas.

INTRODUCTION: Bladder localization of AA amyloidosis is rare. It can be responsible for massive and recurrent hematuria. We report a case of bladder AA amyloidosis secondary to Crohn's disease in a renal transplant patient. CLINICAL OBSERVATION: A 62-year-old man, suffering from Crohn's disease since 1991 complicated by renal AA amyloidosis. He received a kidney transplant since 20 years from an HLA identical donor. After an 18-year period of clinical remission, the patient was admitted for a flare-up of his Crohn's disease in the form of intermittent diarrhoea. Treatment with corticosteroids allowed a good evolution. A year later, he was rehospitalized for massive macroscopic haematuria. Histological examination of the bladder biopsy revealed AA amyloidosis. The patient fully recovered but died 6 weeks later from septic shock of urinary origin. CONCLUSION: The treatment of bladder localization of AA amyloidosis is based on treating the cause. Hematuria is sometimes massive, exceptionally requiring emergency cystectomy for haemostasis.

Pubic and ischial sarcoidosis: a single bone lesion.

Bone sarcoidosis is very rarely indicative of the disease. When bone lesion is associated with lung and lymph node involvement, diagnosis can be made based on clinical and imaging features. When bone lesion is isolated, it is difficult to differentiate it from bone metastases because they both have similar appearance in imaging: in this case, the diagnosis is made by bone biopsy with histological study. We report the case of a 61-year-old male with a lytic lesion of the right ischio pubic ramus which appears to be aggressive whose biopsy revealed bone sarcoidosis.

Thyroid metastasis revealing a lung adenocarcinoma: A case report and review of the literature.

INTRODUCTION: Metastatic lung adenocarcinoma in the thyroid is very rare. The clinical presentation and the radiological findings for metastasis carcinoma are nonspecific and do not allow the distinction between metastatic lung carcinoma and primary thyroid tumor. CASE PRESENTATION: We report the case of a pulmonary papillary adenocarcinoma revealed by a thyroid metastasis in a 62-year-old and non-smoker patient with no history of cancer. DISCUSSION: Thyroid metastasis revealing a primary adenocarcinoma of lung is extremely rare. In the absence of a history of lung cancer, the histological appearance of a papillary adenocarcinoma localized in the thyroid can be misdiagnosed as a primary thyroid cancer given the non-specificity of the clinical, radiological and histological presentations. Immunohistochemical analysis and molecular studies are the gold standards for establishing the diagnosis of the primary site. CONCLUSION: In this report we aim to discuss the histological and immunohistochemical features of lung adenocarcinoma metastazing in thyroid gland through a literature review. We are also targeting to highlight the essential role of immunohistochemistry and molecular study for the confirmation of the primary pulmonary origin and to discuss therapy for patients with lung cancer metastatic in the thyroid [17].

Hepatoid Adenocarcinoma of the Lung: A Rare Form of Lung Cancer.

Hepatoid adenocarcinoma is a rare type of extrahepatic adenocarcinoma with glandular and hepatocyte differenciation. The tumor can occur in many organs, generally in the stomach, the location of the lung being extremely rare. Despite poor prognosis and few effective treatment options, a timely and accurate histopathological diagnosis is key to optimal clinical management for long-term survival. Given the few reports published to characterized hepatoid adenocarcinoma, the emergence of any new case will contribute to improve understanding of the disease. Very few reports have been published to characterize hepatoid adenocarcinoma cytologically or even histologically. The aim of this work is to focus on the anatomoclinical characteristics of this rare entity. We present a retrospective study of 2 cases of pulmonary hepatoid adenocarcinoma confirmed by histological and immunohistochemical study.

Dietary Fat Intake and KRAS Mutations in Colorectal Cancer in a Moroccan Population.

Epidemiologic data support an association between diet and mutations in the Kirsten-ras (KRAS) gene involved in colorectal cancer (CRC) development. This study aimed to explore the associations between fat intake and KRAS mutations in codons 12 and 13 in cases of CRC in the Moroccan population. A multicenter case-series study nested in a large-scale Moroccan CRC case-control study was conducted. Among all CRC cases recruited, 151 specimens were available for the DNA mutation analysis. Logistic regression was used to calculate odds ratios (ORs) and 95% confidence intervals (Cis) for KRAS mutation status according to the fat intake variables. A KRAS mutation was detected in the CRC tumor of 34.4% of the patients among whom 65.4% had a single mutation at codon 12 and 34.6% had a single mutation at codon 13. Compared to low levels of consumption, a positive association was observed between high polyunsaturated fatty acids (PUFA) consumption (>16.9 g/day) and prevalence of KRAS mutations (OR = 2.15, 95% CI = 1.01-4.59). No statistically significant associations were observed for total fat, monounsaturated fatty acids, saturated fatty acids and KRAS mutations. The results of this study suggest that PUFA may be relevant in the etiology of CRC, possibly through the generation of G > A transitions at the KRAS oncogene. Further studies are needed to verify and explain this finding.

[Sneddon-Wilkinson disease: a case report]. / Maladie de Sneddon-Wilkinson: à propos d'un cas.

Sneddon-Wilkinson disease is a benign amicrobial pustulosis belonging to the spectrum of neutrophilic dermatoses. It is characterized by evident stereotypic clinical features, including, in most cases, pustular lesions of the trunk and prominent skin folds. This pustulosis may be associated with other diseases (IgA monoclonal gammopathy, rheumatoid arthritis, neoplasms or other neutrophilic dermatoses) and therefore requires regular follow-up. This disease has a relapsing-remitting course. First line therapy is dapsone. We here report the case of a 49-year-old patient with amicrobial pustulosis (Sneddon-Wilkinson disease).

Concurrent Angiomyolipoma and Clear Cell Renal Cell Carcinoma in the Same Kidney: A Rare Finding in a Patient without Tuberous Sclerosis.

Synchronous renal cell carcinomas (RCC) and angiomyolipomas (AML) occurring in the same kidney are rare. Cases in the setting of tuberous sclerosis (TS) have been reported in the literature. However, the association of these tumors in the same kidney without TS is even more rare. We report here a case of a clear cell renal cell carcinoma (CCRCC) associated with an AML in the same kidney in a 42 years old female lacking the TS diagnostic criteria. The patient underwent a radical nephrectomy. Six months after surgery, the patient is healthy without signs of tumor recurrence or distant metastasis.

Kaposi sarcoma of the adrenal gland: A report of two cases with a review of the literature.

Kaposi sarcoma (KS) is a low-grade vascular neoplasm commonly involving mucocutaneous sites, while adrenal gland involvement is exceptional. The anaplastic variant is a rare entity characterized by marked cellular pleomorphism, increased mitosis, worse prognosis, and an increased metastatic potential. The diagnosis remains on histology and immunohistochemistry. We describe two cases of primary adrenal KS to report on this exceptional presentation of KS: the first case is a 67-year-old female with anaplastic KS wit fatal outcome. The second case is a 56-year-old male who presented a classic KS of the adrenal gland. Until now, no standard efficient treatment regimens have been clearly identified. There is a need for a further understanding of anaplastic KS's biology, and collecting a sizable patient cohort remains essential to review treatment outcome.

Leiomyosarcoma of the Renal Vein Mimicking a Primitive Renal Cell Carcinoma: Case Report of an Unusual Presentation.

Primary leiomyosarcomas (LMS) of vascular origin are rare tumors, and more than half of the cases arise in the inferior vena cava (IVC). Primary LMS of the renal vein are extremely rare tumors with only a few cases reported in the literature. Their diagnosis is made only by pathological features. Histologically, they are made of atypical spindle-shaped cells arranged in long intersecting fascicles. Tumor cells stain positive for myogenic markers in immunohistochemistry. Standard treatment consists of radical nephrectomy followed by chemotherapy and/or radiotherapy. Because of insufficient histological data and follow-up, the prognosis factors are not well identified. Overall prognosis of renal vein LMS is poor. We report here an exceptional case of a huge LMS of the right renal vein mimicking a primitive renal cell carcinoma, occurring in a 56-year-old male patient.

Primary Hodgkin lymphoma of the ulnar nerve: the first case report with review of the literature.

Hodgkin lymphoma is a lymphoid malignancy characterized by minority population of neoplastic cells (Reed-Sternberg cells and its variants) within a reactive inflammatory background. It encompasses two entities: classical HL (∼95% of cases) and nodular lymphocyte predominant HL (∼5% of cases). Primary lymphoma of peripheral nerves (PLPN) represent a very rare condition, since only 19 cases have been reported in the English literature to date, all of which are of a non-HL phenotype. A 20-year-old female presented an intramural mass of the ulnar nerve. Histological analysis revealed a Classical Hodgkin lymphoma. Further investigations failed to reveal nodal or extranodal involvement. PLPN is a very rare entity. There is a need for further understanding of this unusual lymphoma presentation.

A polymorphous cutaneous tuberculosis.

Cutaneous tuberculosis is uncommon, comprising 1%-2% of all extrapulmonary manifestations. It has a wide clinical spectrum. The clinical presentation depends on the route of the infection, the pathogenicity of the bacteria, and the immune status of the host. Association of multiple forms in a same patient is uncommon. We report a case of disseminated tuberculosis with polymorphous cutaneous lesions in an immunocompetent woman, combining scrofuloderma, gummas, and sporotrichoid lesions, along with tuberculous adenitis and bone tuberculosis.

Retroperitoneal leiomyoma of gynecologic type: a case report and review of the litterature.

Retroperitoneal leiomyomas are rare benign tumors with smooth muscle differentiation, with only ~1001 cases reported in the English literature to date. Because of its scarcity and non-specific presentation, the preoperative diagnosis might be challenging. On histology, these neoplasms share the same macroscopic, morphological and phenotypic features with uterine leiomyoma; thus, they are referred to as leiomyoma of gynecologic type. Herein, we describe a case of a voluminous retroperitoneal leiomyoma in a 51-year-old woman with a history of total hysterectomy to raise awareness about this condition, as its differential diagnosis might be challenging.