Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma

Presentación de casos clínicos RESUMENEl adamantinoma es un tumor primario de los huesos largos, que afecta principalmente la diáfisis de la tibia, y es extremadamente raro en pediatría. Se presenta a partir de la segunda década de vida, con un ligero predominio en el sexo masculino. Se trata de un tumor de bajo grado, con alta agresividad a nivel local y bajo índice de metástasis y recurrencia una vez resecado en forma completa. Su diagnóstico resulta difícil, no solo por tratarse de una patología poco frecuente en pediatría, sino también por la dificultad para el diagnóstico diferencial con otras lesiones benignas. Presentamos el caso de un paciente de 15 años, con una tumoración indolora de larga evolución en la tibia distal, cuyo diagnóstico fue confirmado histológicamente con la pieza de amputación, ya que los estudios complementarios y las dos biopsias iniciales no fueron concluyentes. Aunque la mayoría de la bibliografía publicada consta de reportes de casos, y muy pocos en pacientes pediátricos, es de común acuerdo la dificultad para arribar al diagnóstico de adamantinoma. Palabras clave: adamantinoma, tumor óseo, tumor de bajo grado, pediatría.ABSTRACT Adamantinoma is a primary tumor of long bones, which affects mainly the shaft of the tibia, and is extremely rare in pediatrics. It frequently presents during the second decade of life, with a slight predominance in males. It is a low grade tumor, with local aggressiveness and low rate of metastasis and recurrence once it is completely removed. Its diagnosis is difficult, not only because it is a rare disease in children, but also because of the difficulty in the differential diagnosis with other benign lesions. We report the case of a 15-year-old patient with a painless swelling of the distal tibia, whose diagnosis was confirmed with the piece of amputation, as imaging features and both initial biopsies were not enough to achieve diagnosis. Though most of the literature consists of case reports, and very few in pediatric patients, they all agree on the difficulty in achieving the diagnosis of adamantinoma.

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“…5,6 Se manifiesta con una tumoración, que puede ser indolora, y en algunos casos, ocasiona fracturas patológicas.…”

Section: Discussionunclassified

“…6 La resonancia magnética nuclear permite evaluar mejor la extensión tumoral y el compromiso de partes blandas. En la secuencia T1, el tumor muestra una señal isointensa al músculo y es hiperintenso y heterogéneo en T2.…”

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Adamantinoma en pediatría. Presentación de caso

et al. 2015

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“…It most commonly affects the tibia followed by the fibula. Histologically, OFD is characterized by an osteofibrous stroma replacing normal bone, showing trabeculae which, unlike in FD, are surrounded by a regular rim of prominent cubic osteoblasts, mature lamellar bone and zonal segmentation with newly formed trabeculae mostly in the cortical region (Campanacci 1976) with radiological features similar to those of adamantinoma (Unni 1996;Kahn 2003).…”

Section: Histological Features Of Adamantinoma and Osteofibrous Dysplmentioning

confidence: 99%

“…Some cases of OFD have been demonstrated by immunohistochemical studies to contain cytokeratinpositive epithelial cells (Kahn 2003). In OFD, however, the epithelial-like component was much smaller than in adamantinoma and was composed of a few cells positive for cytokeratin (Benassi et al 1994).…”

Section: Correlation Between Adamantinoma and Osteofibrous Dysplasia Imentioning

confidence: 99%

“…More recently, a third group of cases with clinical and radiological features similar to those of OFD has been proposed and this group of the lesion is associated with more overt strands of epithelial cells within a fibro-osseous background and have been categorized as "differentiated," "regressive," "juvenile intracortical" or "osteofibrous dysplasia-like" adamantinoma (Kahn 2003). Czerniak et al (1989) in proposing a different correlation between OFD and adamantinoma, suggesting that adamantinoma can be divided into two distinct groups.…”

Section: "Osteofibrous Dysplasia-like" Adamantinomamentioning

confidence: 99%

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A Classic Adamantinoma Arising from Osteofibrous Dysplasia-Like Adamantinoma in the Lower Leg: A Case Report and Review of the Literature

Hatori

Watanabe

Hosaka

et al. 2006

Tohoku J. Exp. Med.

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Adamantinoma is known as a low-grade malignant biphasic tumor. Classic adamantinoma is in general characterized by admixture of both epithelial and osteofibrous components that are associated with various proportions and differentiation patterns. Osteofibrous dysplasia (OFD) is a self-limited benign fibro-osseous lesion of bone during infancy and childhood. OFD-like adamantinoma is characterized by predominance of osteofibrous tissues, in which small groups of epithelial cells are only detected by careful search or immunohistochemistry. There have been controversies as to the potential correlation among OFD, OFD-like adamantinoma and classic adamantinoma. We report an unusual case of adamantinoma arising in the tibia, with an extensive review of the literature. The present findings suggest a direct correlation between OFD-like adamantinoma and classic adamantinoma. At the age of 12 years, the tibial biopsy lesion was diagnosed as OFD. At the age of 23, the lesion became larger and more destructive on X-ray films. The biopsy lesion was diagnosed as classic adamantinoma. Wide excision was performed. The primary lesion was retrospectively diagnosed as OFD-like adamantinoma because of presence of keratin-positive epithelial cells within the stroma. At five years after surgery, the patient was free from recurrence or metastasis. The retrospective histological findings of OFD-like adamantinoma in the original biopsy and of a classic adamantinoma in all sections of the later resection specimen raised the possibility of an unusual progression of OFD-like adamantinoma to a classic adamantinoma. The present case and the literature review suggest that an OFD-like adamantinoma may be a precursor lesion of classic adamantinoma. Therefore, the possibility of progression of OFD-like adamantinoma to a classic adamantinoma should be kept in mind, particularly when the destructive changes are seen radiologically.

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Adamantinoma of bone: Long‐term follow‐up of 46 consecutive patients

Houdek

Sherman

Inwards

et al. 2018

Journal of Surgical Oncology

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Background Adamantinomas are rare bone tumors, commonly affecting the tibia. Due to the rare nature of disease, previous studies are small or from multiple centers. The purpose of this study is to investigate outcomes of patients with adamantinoma treated in a single institution. Methods Forty‐six histological confirmed adamantinomas of the extremities were reviewed at our institution between 1939 and 2012. Follow‐up data included clinical and radiographical information focusing on complications, local recurrence, metastasis, and overall survival after the treatment. The mean follow‐up was 16 years (range 2‐42 years). Results The most common location was the tibia (n = 31). Patients commonly presented with pain and swelling. The mean age was 24 years (7‐79 years). Thirty‐seven patients were treated with limb salvage. The 39% of patients required a reoperation. The 10‐year disease specific‐ and recurrence free survival was 92% and 72%, with three patients having a recurrence over 15 years postoperative. Older (> 20 years) patients and males were at increased risk of local recurrence (P < 0.05). Conclusion Treatment of adamantinoma of the long bone consists of limb‐salvage surgery. Male patients should be cautioned on their increased risk of disease recurrence, and advocate for continued surveillance of patients even greater than 15‐years postoperatively due to late tumor recurrence.

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Adamantinoma, osteofibrous dysplasia and differentiated adamantinoma

Cited by 136 publications

References 48 publications

Adamantinoma en pediatría. Presentación de caso

Adamantinoma en pediatría. Presentación de caso

A Classic Adamantinoma Arising from Osteofibrous Dysplasia-Like Adamantinoma in the Lower Leg: A Case Report and Review of the Literature

Adamantinoma of bone: Long‐term follow‐up of 46 consecutive patients

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