British Journal of Radiology 74 (2001),452-454 © 2001 The British Institute of Radiology
Calcifying fibrous pseudotumour of the adrenal gland
F Eftekhari, MD
1
J L Ater, MD
2
A G Ayala, MD
3 and
B A Czerniak, MD, PhD
3
1 Division of Diagnostic Imaging, Box 057
2 Division of Pediatrics, Box 087
3 Division of Pathology and Laboratory Medicine, Box 085, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Boulevard, Houston, TX 77030, USA
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Abstract
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Calcifying fibrous pseudotumour is a distinct pathological entity usually occurring in the soft tissue of the extremities, trunk, axilla, pleura, mediastinum and peritoneum. This report describes the hitherto unreported occurrence of this tumour of the adrenal gland in a 10-year-old girl whose imaging findings closely resembled a neuroblastoma. This entity is a potential pitfall in diagnosing adrenal neuroblastoma.
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Introduction
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Calcifying fibrous pseudotumour (CFPT) is a distinct clinical and pathological entity, which usually orginates from the soft tissues of the extremities in children, but may also arise in the soft tissues of the trunk, neck, axilla and pleura, and may also occur in adults.
The clinical presentation is usually a soft tissue mass of several weeks or months duration without any constitutional symptoms. It shows either laminated or amorphous calcifications on plain radiography. Treatment is surgical and recurrence is rare.
This communication reports yet another site of occurrence of CFPT in a 10-year-old girl, namely the adrenal gland.
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Case report
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A 10-year-old girl, previously in good health, had a 3-month history of intermittent frontal headaches occurring every other day, at no particular time of day. The headaches were very severe at times and were alleviated by ibuprofen. According to the child's mother, her face flushed during one of these episodes but no blood pressure measurements were obtained. There were no other symptoms. The headaches gradually increased in severity. She was seen by a paediatrician, who discovered a large abdominal mass. This was confirmed by CT (Figure 1
), which localized the tumour to the left suprarenal region. The interpreting radiologist diagnosed the tumour as an adrenal neuroblastoma. The patient was then referred to our institution.
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Figure 1. Calcifying fibrous pseudotumour of the left adrenal gland. (a) Contrast enhanced CT showing a large solid mass (black arrowheads) displacing the spleen (S) anteriorly. The lateral segment of the liver was seen at a higher level. Notice coarse calcifications (white open arrow) and also the enlarged retroperitoneal nodes (black open arrows). (b) Contrast enhanced CT showing the caudal extent of the calcified mass (white arrowheads) compressing the left kidney. Notice left paraaortic nodes (large tapered black arrow) and incidental renal cyst.
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No abnormalities were detected on physical examination apart from fullness in the upper abdomen on deep palpation. The mass appeared to be adherent to adjacent structures. The child was normotensive. Further work-up, including a bone marrow aspiration, showed no metastatic disease. Urinary vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels were normal.
The CT from the outside institution showed a solid retroperitoneal mass containing coarse calcifications (Figure 1
). The mass measured 15 cm x 12 cm in size and displaced the left kidney, spleen and pancreas. Several enlarged paraaortic lymph nodes were present. While the appearance of the mass was not quite typical, in the absence of other differential possibilities we concurred with the diagnosis of adrenal neuroblastoma. At laparotomy, a large left suprarenal mass was found, which was distinctly separate from the left kidney. A frozen section was interpreted as a spindle cell neoplasm. The mass and retroperitoneal nodes were resected in toto (Figure 2). Permanent sections (Figure 3) showed a spindle cell neoplasm forming irregular bundles and fascicles without microscopic atypia. There were abundant collagen fibres and foci of dystrophic calcifications. Chronic lymphoplasmocytic inflammatory cells forming prominent focal aggregates were also present. These findings were consistent with CFPT. Residual foci of adrenal tissue were found at the periphery of the mass, as well as a separate focus of normal adrenal tissue. Three regional lymph nodes, nerves and ganglia were all free of tumour.
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Figure 2. Surgical resection specimen of the mass showing a posteriorly located cleft (black arrows) corresponding to that shown on Figure 1.
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Figure 3. Microscopic features of calcifying fibrous pseudotumour. (A) Low power view showing spindle cell proliferation without atypia, coarse collagen fibres and focal dystrophic calcifications ( x 100). (B) Chronic lymphoplasmocytic inflammatory cell infiltrates scattered throughout the tumour and forming more prominent aggregates focally ( x 200).
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Discussion
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Pseudotumours are rare benign lesions of the pleura and soft tissues that may result from previous inflammatory processes. All CFPTs share the common features of other inflammatory pseudotumours, e.g. pulmonary hyalinizing granuloma and plasma cell granuloma [1–5]. These include dense hyalinization, fibrous proliferation and a lymphoplasmocytic cell infiltration. Unlike inflammatory pseudotumours, CFPTs show extensive concentric psammomatous or dystrophic calcifications [3–5].
Rosenthal and Abdul-Karim in 1988 [3] described two cases of CFPT in the thigh of a 2-year-old girl and the forearm of an 11-year-old girl, present for a period of 4 weeks and 6 months, respectively. Calcifications were identified radiographically only in the first patient. On histological examination, both tumours showed amorphous bands of collagen, uniform elongated fibroblasts and large number of microcalcifications. The authors termed this lesion "childhood fibrous tumour with psammoma bodies".
Reviewing the archives of the Armed Forces Institute of Pathology (AFIP), Fetsch et al in 1993 [4] collected only 10 definite cases of benign fibrous lesions characterized by abundant hyalinized collagen containing psammomatous or dystrophic calcifications and a lymphoplasmocytic infiltrate. These patients were between 1 year and 33 years of age and the tumours had been present for between 2 months and 10 years. They arose from the extremities, trunk, scrotum, groin, neck and axilla, and involved the subcutaneous and deep soft tissues. All lesions were treated by local excision. Local recurrence developed in one patient 7.5 years later and was treated by re-excision. These authors favoured the term "calcifying fibrous pseudotumour" to emphasize a broader age range and the fact that mineralization could also be of dystrophic type.
Radiographic features of pleural CFPT have been previously reported in three adult patients [5]. These cases showed thick, band-like or punctate calcifications, which were depicted on CT but not on conventional radiographs. Dumont et al [2] reported a case of CFPT in the mediastinum of a young woman treated by resection. Kocova et al [6] reported four cases of CFPT in the visceral peritoneum of small bowel and stomach, all occurring in adults. These authors recognized that their cases were distinctly different from all other lesions displaying hyaline collagenous deposits, i.e. inflammatory pseudotumour, desmoid, hyalinizing granuloma or fibrous plaques of peritoneum.
The case illustrated above is unique in its site of occurrence and presentation with headaches, and may be a potential pitfall for adrenal neuroblastoma. The patient's headaches subsided after resection of the mass. Whether they were coincidental or related to some circulating product of the mass is open to debate.
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Acknowledgments
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We thank Mary Carr for preparation of the manuscript and Kelly Duggan for photography.
Received for publication June 15, 2000.
Revision received January 29, 2001.
Accepted for publication February 16, 2001.
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References
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Dumont P, de Muret A, Skrobala D, Robin P, Toumieux B. Calcifying fibrous pseudotumor of the mediastinum. Ann Thorac Surg 1997;63:543–4.[Abstract/Free Full Text]
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Rosenthal NS, Abdul-Karim FW. Childhood fibrous tumor with psammoma bodies. Clinicopathologic feature in two cases. Arch Pathol Lab Med 1988;112:798–800.[Medline]
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Kocova L, Michal M, Sulc M, Zamecnik M. Calcifying fibrous pseudotumour of visceral peritoneum. Histopathology 1997;31:182–4.[Medline]
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Abstract
Introduction
