Commonest manifestation of Grawitz's tumor in male
Ans. c (Hematuria) (Ref. Bailey and Love 26th/ pg. 1307)Adenocarcinoma of the kidney# Haematuria is the most common clinical symptom.# May be associated with PUO, polycythaemia, hypercalcaemia, anaemia and other paraneoplastic symptoms# Metastasises via the bloodstream to bone, liver and lung (cannonball secondaries)# Renal vein extension may embolise to the pulmonary circulation during nephrectomyHYPERNEPHROMA (Syn. GRAWITZ'S TUMOR)# This is an adenocarcinoma.# It is the most common neoplasm (75 per cent) of the kidney.# It arises from renal tubular cells.# most commonly the upper pole.# Spread : The tumor is prone to grow into the renal vein.# Highly vascular metastases may pulsate.# If the tumour extends beyond the renal capsule it is liable to metastasise via the lymph nodes in the hilum of the kidney to the para-aortic nodes and beyond.# CF: M:F = 2:1.# Hematuria is the most common symptom.# In men, a rapidly developing varicocele is a rare but impressive sign, occurring most often on the left side because the left gonadal vein is obstructed where it joins the left renal vein.# Atypical C/f: Hyperpyrexia, Extreme anemia, or Polycythemia (occurs in 4%of cases), Hypercalcemia, Nephrotic synd.# Investigation: US is generally initial or screening too of choice, while CECT demonstrate the extent of the lesion more clearly and will show whether there is hilar lymphadenopathy or renal vein involvement. MRV remains the best choice for detecting renal vein invasion.# Treatment- If the tumor is confined to the kidney, treatment is nephrectomy with removal of the perinephric fat.- Nephrectomy can be performed through a loin or a transverse or oblique upper abdominal incision. The transab- dominal approach has the advantage that the renal pedicle and the IVC can be widely exposed.- The vascular pedicle should be ligated before the kidney is mobilised because handling the tumor may cause malignant cells to be released into the circulation.- Adenocarcinoma of the kidney does not respond well to radiotherapy or conventional chemotherapy. There have been early promising results from clinical trials of the cytokine interleukin-2 in this condition.- Conventional surgical treatment is by nephroureterectomy.- Percutaneous resection of these cancers is controversial.Also know:WILMS' TUMOR (Syn. NEPHROBLASTOMA)# This is a mixed tumor containing epithelial and connective tissue elements arising from embryonic nephrogenic tissue.# The tumors are usually discovered during the first 4 years of life.# Abdominal tumor. An abdominal tumor appears which grows rapidly while the general well being of the child deteriorates.# Pyrexia, which is a feature in half of these patients, disappears when the tumor is removed.# Initial Imaging is by ultrasonography,however CECT is best for evaluation.# Metastasis occurs early, mainly to the lungs. Liver and bone metastases are rare; and brain metastases even more so.# Lymphatic spread is uncommon.# The presence of bone secondaries favours a diagnosis of nephroblastoma, another renal tumor of childhood.# Rx: Nephrectomy should be performed as soon as possible and followed by radiotherapy with or without chemo- therapy.- Partial nephrectomy may be possible in patients with bilateral disease.# Prognosis- Under 1 year of age 80 per cent survive for 5 years, but the prognosis is less good in older children.- Recurrences usually occur within a year, so a child surviving for 18 months or more is probably cured.- NATIONAL WILMS' TUMOR STUDY GROUP (NWTS) STAGING SYSTEM (post-chemotheraphy based staging system in Wilms tumor). It employs Roman numerals I through V (one through five).
Secondary deposits
Pathological fracture
Hematuria
Rapidly developing varicocele
Neoplasia