Extranodal and nodal lymphoma

Case contributed by Dr Ibrahim M. Jubarah

Presentation

History of long-standing non-improved cellulitis over the left suprapubic area, with a recent diagnosis of chronic deep venous thrombosis of the left lower limb, presented with neck, left breast and left suprapubic lumps.

Patient Data

Age: 50 years
Gender: Male
  • Multi-compartmental enhancing enlarged matted pathological lymph nodes, some of them show cystic change; the para-aortic group of them are encasing the abdominal aorta (and displacing it anteriorly), both renal arteries, inferior mesenteric artery, iliac arteries, both ureters (without definite obstruction or hydroureteronephrosis) and, with extraluminal compression over, the inferior vena cava and iliac veins, along with abutting both sides of the urinary bladder resulting in the pear-shape of it.
  • A cortical cyst is seen in the upper pole of left kidney.
  • Multiple cutaneous soft tissue mass lesions in the lower abdomen, the largest one is in the left pubic/suprapubic region.
  • Diffuse thickening of the lower abdominal skin with subcutaneous edema down through both thighs more on the left.
  • Soft tissue mass lesion in the left breast. 
  • Mild pelvic free fluid.

Case Discussion

Lymph nodes' features, in this case, are most likely suggesting lymphoma, manifested by the relatively soft consistency lesions with:

  • matting with surrounding, encasing, and slight push rather than discreteness and marked push (as would it be in other causes of lymphadenopathy) nor pulling adjacent structures (in contrast to, for example, retroperitoneal fibrosis)
    and
  • mild mass-effects relative to their size with venous sluggish flow and stasis (resulting in chronic deep venous thrombosis of the left lower limb here)

The pear-shaped urinary bladder is a classical radiologic sign indicating extraluminal pelvic mass lesion.

The subcutaneous fat stranding/edema of the lower abdomen and lower limbs, in this case, could be due to chronic venous thrombosis; however, the presence of overlying skin thickening raises the possibility of neoplastic involvement and/or cellulitis.

In this context, the cutaneous thickening and masses may suggest primary cutaneous lymphoma with differential diagnosis including CD30 positive primary cutaneous lymphoma or mycosis fungoides, among others.

Biopsies were subsequently taken from a cervical lymph node and the left breast mass and revealed consistency with anaplastic large cell lymphoma-ALK-negative, with strong positivity for CD30, CD3-positivity of some cells, and negative CK for breast mass (excluding lobular carcinoma).

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