Introduction.
With a 2.9% 5-year prevalence rate, kidney cancer ranks 18th in terms of incidence and mortality in Pakistan [1]. Kidney cancer is two times more prevalent in males than in females, and the usual age of presentation is between 55 and 74; presentation before the age of 45 is considered uncommon. In the beginning of the disease process, kidney cancers are mostly asymptomatic, but as they advance, the classic triad of haematuria (visible blood in urine), ipsilateral or affected site flank pain, and palpable abdominal mass becomes apparent with time [2]. Along with general symptoms of advanced disease such as fever, weight loss, chronic or persistent cough, and bone pain, the patient may also develop paraneoplastic syndrome in the form of hypertension, polycythaemia, and Cushing syndrome [3]. Kidney cancer most commonly metastasises to the lungs, followed by bone, brain, liver, adrenal glandand lymph nodes [4].
The clinical presentation may vary depending upon the type of kidney cancer and clinical stage. Main types are renal cell carcinoma (RCC), transitional cell carcinoma (TCC), and Wilms’ tumour [5]. RCC is the most common type among them, and it has many histological subtypes. Papillary RCC (PRCC) is the second most frequent renal cancer subtype, 10-15%, after clear cell renal carcinoma [6]. PRCC, a malignant tumour arising from renal tubular epithelium having tubulopapillary architecture, can further be classified on the basis of morphological features into PRCC type 1 and type 2 [7]. The exact cause of PRCC still remains unclear; possible risk factors include smoking, genetics, hereditary syndrome, obesity, family history, hypertension, and hepatitis C infection [8]. Regarding hereditary syndrome, patients with hereditary papillary renal cancer syndrome have a greater risk of type 1 PRCC, whereas those with hereditary leiomyomatosis and renal cell cancer (HLRCC) or Reed’s syndrome have an increased risk of type 2 PRCC [9]. The standard treatment offered is total, partial, or radical nephrectomy to eliminate all possible risk of metastasis, but surgical options depend upon cancer stage and patient factors.
We hereby report a case of a young 38-year-old patient who presented with symptoms of painless haematuria and lower back pain and ended up with a diagnosis of a rare papillary RCC type 2 along with multiple suspicious-looking pulmonary nodules. The case is unique owing to the young age of presentation, the absence of possible risk factors, and the uncommon prevalence of PRCC in our setup. This case report highlights a detailed account of step-by-step investigations that aided in deciding the final diagnosis and providing appropriate management to the patient. Along with the case description, it discusses the current literature regarding this topic to emphasise the rising incidents of renal cancer in young patients and the importance of early detection and public awareness. Moreover, it also discusses the cytogenetics, some under-researched risk factors, and pharmacological and non-pharmacological management options.
Case History and Examination.
A 38-year-old patient presented to the outpatient department with complaints of left flank pain, tenderness in the left lumbar region, painless haematuria for four months, and right flank pain and lower back pain for two months. The patient also lost significant weight, around six kg, during the same timeline. Past medical, surgical, and family history were all insignificant. On relevant examination, there was mild tenderness noted in the suprapubic and left lumbar regions, but no palpable mass was appreciated in the left lumbar area. The rest of the examination was unremarkable, including general physical, respiratory, cardiovascular, and abdominal. The patients’ vitals were also within normal range.