Multiple nodular foci in the liver associated with chronic hepatic porphyria after previous treatment of breast cancer

Abstract

More than 7 years after the diagnosis and treatment of breast cancer (T1N1aM0), multiple nodular foci were observed in the liver of a 40-year-old woman at ultrasonographic examination. The lesions were confirmed by CT scan, but CT-guided liver biopsy revealed only non-specific alterations. At subsequent peritoneoscopic examination, bluish-brown foci were indeed visible on the liver surface, but guided liver biopsies again failed to corroborate the suspected metastases. Instead, histology showed mild portal fibrosis, moderate steatosis and siderosis of hepatocytes, as before. Only the intense red fluorescence of part of the biopsy material under Wood's light suggested the diagnosis of chronic hepatic porphyria or porphyria cutanea tarda, here presumably as a consequence of prolonged alcohol consumption. Subsequent porphyrin studies in urine, faeces and plasma yielded the typical constellation of latent porphyria cutanea tarda (chronic hepatic porphyria type C). The activity of erythrocyte uroporphyrinogen decarboxylase was normal, which argued against a genetic predisposition. After 1 year of strict alcohol abstinence and low-dose chloroquine treatment the "nodular foci" in the liver were no longer visible on ultrasonogram and CT scan; only proton-weighted NMR imaging (SE 1500/30) still showed ill-defined areas of higher signal intensity. The renal excretion of porphyrins had decreased considerably. The levels are now consistent with the diagnosis of subclinical chronic hepatic porphyria type A. Modern non-invasive imaging techniques are tremendously useful, but they have their pitfalls. Focal liver lesions may present serious diagnostic problems, especially when they are found in a patient with a history of carcinoma at an extrahepatic primary site. A rare example is described.(ABSTRACT TRUNCATED AT 250 WORDS)

Multiple Leberrundherde bei chronisch hepatischer Porphyrie: Zwei sonographische Kasuistiken

“…Interessant ist die prinzipielle Reversibilität der Porphyrieherde. Bereits früh wurde eine vollständige Rückbildung unter strikter Alkoholkarenz oder auch Chloroquin-Therapie beobachtet [6,7]. Andererseits beschreibt Fengler unter fortgesetztem Alkoholkonsum eine Progredienz mit dann homogen verdichteter Leber und homogener Fluoreszenz in einer zweiten Leberbiopsie (initial nur herdförmige Fluoreszenz).…”

Photosensitivity and lymphoma

“…She developed a mild recurrence of the photosensitive rash during this period. The coproporphyrin and uroporphyrin levels were 0.45 and 3 iimol/24 h, respectively. A CT scan revealed a new hepatic lesion 4 cm in size (figure 2).…”

Multinodular fatty change in the liver in three patients with chronic hepatic porphyria: Contribution of sonography to the diagnosis

“…Chronic hepatic porphyria (CHP) is a relatively rare pathologic condition of the liver characterized by periportal inflammation, hemosiderin deposits, and fatty changes in hepatic parenchyma, induced mainly by acquired reduced activity of heme biosynthetic enzyme . It has been known for a long time through previous reports that some CHP cases present with multinodular fatty change (MNFC) in the liver, which mimics liver tumors on grayscale ultrasound (US) and CT . To discriminate MNFC from true liver tumors, US‐guided biopsy is usually performed to yield a definitive.…”