Cancerous leptomeningitis and familial congenital hypopituitarism.

People are at higher risk of cancer as they get older or have a strong family history of cancer. The potential influence of environmental and behavioral factors remains poorly understood. Earlier population and case control studies reported that upper quartile of circulating IGF-I is associated with a higher risk of developing cancer suggesting possible involvement of the growth hormone (GH)/IGF system in initiation or progression of cancer. Since GH therapy increases IGF-1 levels, there have been concerns that GH therapy in hypopituitarism might increase the risk of cancer. We report a 42-year-old female patient who presented with subacute onset of symptoms of meningitis and with the absence of fever which resulted in death 70 days after the onset of symptoms. The patient together with her younger brother was diagnosed at the age of 5 years with familial congenital hypopituitarism, due to homozygous mutation c.150delA in PROP1 gene. Due to evolving hypopituitarism, she was replaced with thyroxine (from age 5), hydrocortisone (from age 13), GH (from age 13 until 17), and sex steroids in adolescence and adulthood. Her consanguineous family has a prominent history of malignant diseases. Six close relatives had malignant disease including her late maternal aunt with breast cancer. BRCA 1 and BRCA 2 mutational analysis in the patient's mother was negative. Histology after autopsy disclosed advanced ovarian cancer with multiple metastases to the brain, leptomeninges, lungs, heart, and adrenals. Low circulating IGF-1 did not seem to protect this patient from cancer initiation and progression in the context of strong family history of malignancies.

Immunohistochemical expression of nestin in rhabdomyosarcoma: implications for clinicopathology and patient outcome.

Rhabdomyosarcoma (RMS) is a highly malignant cancer. Over the last two decades, prognosis for RMS patients has significantly improved, with the exception of those in the high-risk group. In order to identify new prognostic factors, we investigated the expression of nestin in RMS cells and its correlation with clinicopathological features and patient outcome. The analysis of overall survival for all patients (N = 30) revealed 1-, 2-, 3-, 4-, and 5-year survival rates of 93.3, 83.3, 66.7, 63.3, and 63.3%, respectively. Nestin overexpression significantly correlated with survival (P = 0.044). Survival of patients with ≤ 50% nestin-positive cells was 90, 70, and 40% after 1, 2, and 3 years, respectively, and remained unchanged until the end of the investigation period. The study group composed of patients exhibiting nestin expression in >50% of cells showed 1-, 2-, 3-, and 4-year survival rates of 95, 90, 80, and 75%, respectively, remaining stable at 75% for the fifth year of observation. A nestin-positive expression rate lower than 50% was observed more frequently in older patients (43.60 ± 27.58 years; P = 0.028). In addition, higher rates of nestin expression were observed in most embryonal RMS specimens and low-grade tumors, in early stages of the disease, and among younger patients. Our results lead us to propose nestin as possible positive prognostic factor in RMS.

Solitary lymph node metastasis without local recurrence of primary chordoma.

Chordoma is a malignant neoplasm believed to arise from notochord remnants. Its incidence is highest in the sixth decade and is generally regarded as a locally aggressive tumor with slow progression growth rate. Its metastatic incidence ranges from 5 to 40%, and it is generally believed that metastases without local recurrence of primary neoplasm are extremely rare. We report a case of a 38-year-old male patient with solitary inguinal lymph node metastasis without local recurrence of a previously surgically treated primary sacrococcygeal chordoma.

Verrucous carcinoma of the foot: a case report.

We present a case of verrucous carcinoma of the foot in a 34-year-old man. This is a rare, locally invasive, well-differentiated, low-grade squamous cell carcinoma, with human papilloma virus as a possible causative agent. It follows a chronic course and mimics a variety of skin lesions, delaying diagnosis by up to 15 years. The definitive diagnosis is made histologically, and treatment by wide local excision is recommended. Our patient underwent wide local excision and partial 5th metatarsal amputation because of invasive disease, local infection, and peripheral vascular disease. There were no postoperative complications. At the 10-year follow-up, there were no signs of tumour recurrence.

[Solitary bone cysts of the pelvis. A frequently misinterpreted entity]. / Solitäre Knochenzysten des Beckens. Eine häufig verkannte Entität.

Solitary bone cyst of the pelvis is a rare lesion. The diagnosis is relatively simple providing sufficient material is accumulated during biopsy or surgery. Calcifying forms often cause difficulties in terms of differential diagnosis. Radiographic imaging can be complex when examining large lesions of the pelvis. Biopsy is the method of choice for definitive diagnosis, which should be verified against material gathered surgically or during curettage. Bone tumors of the pelvis have to be considered in the differential diagnosis of the lesion. Large cystic lesions of the pelvis in particular are to be reviewed pathohistologically to avoid false diagnosis. The consequences for the patient in terms of quality of life and life expectancy can be serious. The need for interdisciplinary cooperation is greater than ever to ensure correct diagnosis and therapy of bone lesions.

Malignant intraosseous peripheral nerve sheath tumour of the proximal femur: a case report.

We report a rare case of an intraosseous malignant peripheral nerve sheath tumour of the femoral head and neck. The tumour presented as a well-defined radiolucent lesion on plain radiography. Computed tomography showed aggressive destruction of the bone with no involvement of the adjacent soft tissues. Magnetic resonance imaging revealed an isointense signal intensity on T1-weighted images, hyperintensity on T2-weighted images, and non-homogeneously increased signal intensity after administration of contrast media. The final diagnosis was based on pathohistologic analyses due to the non-specific nature of the lesion.

[Cytologic evaluation of solitary thyroid nodules]. / Citoloska evaluacija solitarnog nodusa stitaste zlezde.

Fine-needle aspiration is a low-cost diagnostic tool with principal value in determining which patients with thyroid nodules should undergo surgery. Team work and close cooperation among endocrinologists, surgeons, and pathologists are essential for success. Cytologic criteria for diagnosis of the most frequent conditions (benign cystic lesions), Hashimoto thyroiditis and malignancies found in thyroid aspirates have been provided. The unsolved problem of the so-called "follicular" or oxyphilic lesion or neoplasia will be investigated by immunocytochemistry.