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Case 88

Retroperitoneal paracoccidioidomycosis brasiliensis

Epigastric mass

Tulio A A Macedo, M.D.

A forty-one year-old male Brazilian resident presented with epigastric pain lasting 2 months. He lost 10 kg through this period and had two episodes of fever (39°C) daily. His physical examination showed a large mass in the epigastric area.

Ultrasound revealed a mass in the head of the pancreas, which was further assessed by C.T.

Findings

Irregular, partially cystic mass at the pancreas head on computed tomography. There was enhancement following injection of contrast media.

Diagnosis

Retroperitoneal paracoccidioidomycosis brasiliensis.

Differential

Adenocarcinoma of the pancreas head.
Tuberculosis

Discussion

This was a rare case found in the Amazon region of Brazil. It is caused by fungi called Paracoccidioidis braziliensis. The most common presentation is pulmonary, but it may be present in any organ such as bone, nervous and gastrointestinal systems, and others.

Paracoccidioidomycosis (South American Blastomycosis)

Epidemiology: Humans are the most important natural hosts of Paracoccidioides brasiliensis. It is a disease that is restricted to the Americas, occurring from Mexico to Argentina. It is homogeneously distributed in all Americas and high incidence areas are found near low incidence ones. Countries where paracoccidioidomycosis occurs more frequently are Brazil, Colombia and Venezuela (Amazon area), but a few cases have been described in the United States, in Europe, Africa, Asia and in the Middle East, probably autochtonous (all patients acquired paracoccidioidomycosis in Latin America, where they lived or travelled in the past). Probably P. brasiliensis lives in humid, protein-rich soil, where temperature variation is minimal. It produces conidia, which is the form the fungus disperses through air. If inhaled, it reaches the pulmonary alveoli.

Clinical Findings: The most important mode of transmission is through inhalation of aerial forms of P. brasiliensis. Paracoccidioidomycosis may remain dormant for long periods. Pulmonary involvement is characterized by a low frequency of respiratory symptoms, in spite of the extensive radiological involvement. Lymph node are compromised more importantly than is the pulmonary parenchyma.. The x-ray usually shows interstitial or alveolar-interstitial involvement. Pleural involvement is not common. Lymphatic involvement is common and it may actually be the only complaint, mostly in the juvenile form. Cephalic lymphs are more involved. The upper respiratory tract may be involved and it may allow collection of diagnostic material. Cutaneous lesions are important because of its frequency as well as its diagnosis value. It occurs predominantly in the juvenile form. Facial lesions are more common. As the general characteristics of paracoccidioidomycosis, also the cutaneous lesions are polymorphic, various characteristics may be seen in the cutaneous lesions, although ulcerated lesions are, by far, the commonest of all. Other organs involved may be the eyes, adrenal glands, thyroid, urogenital tract, heart, spleen and striated muscles.

Diagnosis: The direct observation is the fastest and easiest diagnosis method, using KOH mount, which reveals around 95% of the patients. Its characteristics during this exam make diagnosis easy: multiple budding, refraction capacity and double wall pattern. Biopsy may be diagnostic. Culture should be performed, since it may prove the activity of the process. Serologic tests are useful for the diagnosis of P. brasiliensis.

Chest X-ray appearances: This chest x-ray is from a different patient, showing a more typical pulmonary presentation.

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