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Recurrent cranial fibroblastic neoplasm in a male adolescent: Case report and review of the literature
Journal of Plastic, Reconstructive & Aesthetic Surgery (2011) 64, 949e951
CASE REPORT
Recurrent cranial fibroblastic neoplasm in a male adolescent: Case report and review of the literature S. Riml a,*, Z. Jasarevic b, L. Larcher a, P. Kompatscher a a b
Department for Plastic, Aesthetic and Reconstructive Surgery, University Academic Hospital Feldkirch, Feldkirch, Austria Institute for Pathology, University Academic Hospital Feldkirch, Feldkirch, Austria
Received 1 July 2010; accepted 25 November 2010
KEYWORDS Fibroblastic neoplasm; Cranial fasciitis; Myxoid spindle cell neoplasm; Childhood tumours
Summary Paediatric fibroblastic tumours are rare neoplasms, of which cranial fasciitis is the most common. We present a case of a male 7-year-old suffering from a cranial tumour preceded by a mild trauma. The tumour recurred despite radical resection within 8 months. Histologically, neither tumour could be classified as any published pathological entity. Both lesions were described as cellular fibroblastic neoplasms; in addition, the recurrent tumour featured a prominent myxoid matrix. In the 12 months following resection of the second tumour, no further disease recurrence has occurred. ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
A 7-year-old boy developed an indolent, slowly growing subcutaneous tumour after a bagatelle trauma in the parietal region. A biopsy was taken, followed by a radical resection, including all soft-tissue layers except the calvarian periosteum, and coverage by a transposition flap. Eight months later, a tumour of similar clinical appearance, again preceded by a bagatelle trauma, was observed in the frontal region 1.7 cm away from the primary resection
* Corresponding author. Tel: þ43 5522 303 1800; fax: þ43 5522 303 7524. E-mail address: [email protected] (S. Riml).
margin (Figure 1). In analogy, a two-stage tumour excision was performed. In both cases, the tumour appeared clinically as a sharply circumscribed, movable mass in the subcutaneous layer. Therefore, preoperative radiological imaging was not considered necessary.1 The biopsy confirmed the preoperative evaluation, and macroscopic infiltration of neighbouring structures was not detectable. During radical resection, residual tumour tissue could not be distinguished macroscopically from scar tissue, which was present all the way to the aponeurosis of the frontalis muscle. No further recurrence occurred during 12 months of follow-up. According to our radiotherapy department, adjuvant radiotherapy is not indicated in this case.
1748-6815/$ - see front matter ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2010.11.021
950
S. Riml et al.
Besides a mild ventricular parasystole, the patient’s medical history, including family history, is unremarkable.
The tumours feature less collagenous stroma and a less well-developed fascicular architecture than cranial fasciitis, the most feasible differential diagnose (Figure 2). Furthermore, in contrast to cranial fasciitis, the fibroblastlike cells observed here are relatively monomorph and plump, and inflammation as well as extravasated erythrocytes are lacking. We also considered fibromatosis, neurofibromatosis and a malign mesenchymal tumour, but our examined tumours differ substantially from these differential diagnoses. Furthermore, immunohistochemistry failed to show any signs of differentiation. Both tumours show similar histomorphology; however, the second tumour is characterised by a prominent myxoid matrix. The first tumour showed signs of a cellular fibroblastic neoplasm, the second a myxoid spindle cell neoplasm. On the one hand, the tumours show signs of malignancy such as high mitotic activity (Figure 2(D)) and focal pseudoinfiltration of the aponeurosis and the frontalis muscle (Figure 2(C)); on the other hand, atypical mitotic figures are not present.
Histology
Literature review and discussion
The histopathological examination was performed by three pathology institutes (Institute for Pathology, Academic Teaching Hospital Feldkirch, Austria; head: Prof. Offner; Institute for Pathology, Medical University of Graz, Austria; head: Prof. Beham; Harvard Medical School, Boston, USA; head: Prof. Fletcher). Despite extensive histopathological examination including immunostaining, neither tumour could be classified as a published tumour entity.
Despite vigorous literature research, we failed to find a similar case. Of course, the medical history strongly resembles cranial fasciitis of childhood, described by Lauer and Enzinger for the first time in 1980.2 The patients’ age, the localisation and the association with a past trauma are key features of cranial fasciitis. Nevertheless, the histological findings of both tumours are considerably different from cranial fasciitis.
Figure 1 First (1) and second (2) tumour bed in the frontoparietal region of the patient.
Figure 2 Histologic sections of the cranial fibroblastic neoplasm: A) Overview of the subcutaneous and circumscribed tumour (<) (Original magnification 40), B) Tumour architecture (Original magnification 400), C) Pseudoinfiltration of the frontalis muscle (<) and tumour-free resection margins (black ink) (Original magnification 200), D) High mitotic activity shown by Ki67 immunostaining (Original magnification 400).
Fibroblastic neoplasm in childhood Interestingly, the patient developed two separate, but adjacent, tumours. The tumours share similar histopathological features, but are not identical. The second tumour is distinguished by its prominent myxoid matrix. The proximity of both tumours indicates a local recurrence, although the first tumour was excised completely (Figure 2(C)). Theoretically, the tumours could have developed independently. Anamnestically, a trauma preceded both tumours. The first tumour was preceded by a surgically treated wound, the second only by a mild pat, incidents any lively boy will acquire during sport and play. The worst-case scenario would be metastatic disease. Fortunately, no further recurrence occurred during 12 months of follow-up. Notably, concerning cranial fasciitis, one comparable case has been published: Summers et al.3 report a patient developing multiple lesions of cranial fasciitis, in the cranial region as well as in the chest wall and the abdomen. Of course, the aggressiveness of treatment of our patient is debatable. As the precise tumour biology is unknown due to lack of comparable cases, we opted for a minimally invasive approach in the form of radical tumour resection without an additional security margin. Assuming the second tumour presented a local recurrence, a security margin of at least 2 cm would have been necessary, which would have caused a substantial aesthetic problem. The same arguments led our department for radiotherapy to refrain from adjuvant radiotherapy.4 In summary, we are confronted with a tumour of unknown tumour biology in a radiosensitive region of a young patient. Further follow-
951 up is necessary for accurate assessment of our therapy strategy.
Acknowledgement We thank Dr. W. Sterlacci for editing the article and A. Piontke for excellent photodocumentation.
Conflict of interest None.
Funding None.
References 1. Johnson KJ, Dannenbaum MJ, Bhattacharjee MB, et al. Diagnosing cranial fasciitis based on distinguishing radiological features. J Neurosurg Pediatrics 2008;2:370e4. 2. Lauer DH, Enzinger FM. Cranial fasciitis of childhood. Cancer 1980 Jan15;45(2):401e6. 3. Summers LE, Florez L, Berberian ZJ, Bhattacharjee M, Walsh JW. Postoperative cranial fasciitis. Report of two cases and review of the literature. J Neurosurg 2007;106(6):1080e5. 4. Hussein MR. Cranial fasciitis of childhood: a case report and review of literature. J Cutan Pathol 2008 Feb;35(2):212e4.
CASE REPORT
Recurrent cranial fibroblastic neoplasm in a male adolescent: Case report and review of the literature S. Riml a,*, Z. Jasarevic b, L. Larcher a, P. Kompatscher a a b
Department for Plastic, Aesthetic and Reconstructive Surgery, University Academic Hospital Feldkirch, Feldkirch, Austria Institute for Pathology, University Academic Hospital Feldkirch, Feldkirch, Austria
Received 1 July 2010; accepted 25 November 2010
KEYWORDS Fibroblastic neoplasm; Cranial fasciitis; Myxoid spindle cell neoplasm; Childhood tumours
Summary Paediatric fibroblastic tumours are rare neoplasms, of which cranial fasciitis is the most common. We present a case of a male 7-year-old suffering from a cranial tumour preceded by a mild trauma. The tumour recurred despite radical resection within 8 months. Histologically, neither tumour could be classified as any published pathological entity. Both lesions were described as cellular fibroblastic neoplasms; in addition, the recurrent tumour featured a prominent myxoid matrix. In the 12 months following resection of the second tumour, no further disease recurrence has occurred. ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.
A 7-year-old boy developed an indolent, slowly growing subcutaneous tumour after a bagatelle trauma in the parietal region. A biopsy was taken, followed by a radical resection, including all soft-tissue layers except the calvarian periosteum, and coverage by a transposition flap. Eight months later, a tumour of similar clinical appearance, again preceded by a bagatelle trauma, was observed in the frontal region 1.7 cm away from the primary resection
* Corresponding author. Tel: þ43 5522 303 1800; fax: þ43 5522 303 7524. E-mail address: [email protected] (S. Riml).
margin (Figure 1). In analogy, a two-stage tumour excision was performed. In both cases, the tumour appeared clinically as a sharply circumscribed, movable mass in the subcutaneous layer. Therefore, preoperative radiological imaging was not considered necessary.1 The biopsy confirmed the preoperative evaluation, and macroscopic infiltration of neighbouring structures was not detectable. During radical resection, residual tumour tissue could not be distinguished macroscopically from scar tissue, which was present all the way to the aponeurosis of the frontalis muscle. No further recurrence occurred during 12 months of follow-up. According to our radiotherapy department, adjuvant radiotherapy is not indicated in this case.
1748-6815/$ - see front matter ª 2010 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved. doi:10.1016/j.bjps.2010.11.021
950
S. Riml et al.
Besides a mild ventricular parasystole, the patient’s medical history, including family history, is unremarkable.
The tumours feature less collagenous stroma and a less well-developed fascicular architecture than cranial fasciitis, the most feasible differential diagnose (Figure 2). Furthermore, in contrast to cranial fasciitis, the fibroblastlike cells observed here are relatively monomorph and plump, and inflammation as well as extravasated erythrocytes are lacking. We also considered fibromatosis, neurofibromatosis and a malign mesenchymal tumour, but our examined tumours differ substantially from these differential diagnoses. Furthermore, immunohistochemistry failed to show any signs of differentiation. Both tumours show similar histomorphology; however, the second tumour is characterised by a prominent myxoid matrix. The first tumour showed signs of a cellular fibroblastic neoplasm, the second a myxoid spindle cell neoplasm. On the one hand, the tumours show signs of malignancy such as high mitotic activity (Figure 2(D)) and focal pseudoinfiltration of the aponeurosis and the frontalis muscle (Figure 2(C)); on the other hand, atypical mitotic figures are not present.
Histology
Literature review and discussion
The histopathological examination was performed by three pathology institutes (Institute for Pathology, Academic Teaching Hospital Feldkirch, Austria; head: Prof. Offner; Institute for Pathology, Medical University of Graz, Austria; head: Prof. Beham; Harvard Medical School, Boston, USA; head: Prof. Fletcher). Despite extensive histopathological examination including immunostaining, neither tumour could be classified as a published tumour entity.
Despite vigorous literature research, we failed to find a similar case. Of course, the medical history strongly resembles cranial fasciitis of childhood, described by Lauer and Enzinger for the first time in 1980.2 The patients’ age, the localisation and the association with a past trauma are key features of cranial fasciitis. Nevertheless, the histological findings of both tumours are considerably different from cranial fasciitis.
Figure 1 First (1) and second (2) tumour bed in the frontoparietal region of the patient.
Figure 2 Histologic sections of the cranial fibroblastic neoplasm: A) Overview of the subcutaneous and circumscribed tumour (<) (Original magnification 40), B) Tumour architecture (Original magnification 400), C) Pseudoinfiltration of the frontalis muscle (<) and tumour-free resection margins (black ink) (Original magnification 200), D) High mitotic activity shown by Ki67 immunostaining (Original magnification 400).
Fibroblastic neoplasm in childhood Interestingly, the patient developed two separate, but adjacent, tumours. The tumours share similar histopathological features, but are not identical. The second tumour is distinguished by its prominent myxoid matrix. The proximity of both tumours indicates a local recurrence, although the first tumour was excised completely (Figure 2(C)). Theoretically, the tumours could have developed independently. Anamnestically, a trauma preceded both tumours. The first tumour was preceded by a surgically treated wound, the second only by a mild pat, incidents any lively boy will acquire during sport and play. The worst-case scenario would be metastatic disease. Fortunately, no further recurrence occurred during 12 months of follow-up. Notably, concerning cranial fasciitis, one comparable case has been published: Summers et al.3 report a patient developing multiple lesions of cranial fasciitis, in the cranial region as well as in the chest wall and the abdomen. Of course, the aggressiveness of treatment of our patient is debatable. As the precise tumour biology is unknown due to lack of comparable cases, we opted for a minimally invasive approach in the form of radical tumour resection without an additional security margin. Assuming the second tumour presented a local recurrence, a security margin of at least 2 cm would have been necessary, which would have caused a substantial aesthetic problem. The same arguments led our department for radiotherapy to refrain from adjuvant radiotherapy.4 In summary, we are confronted with a tumour of unknown tumour biology in a radiosensitive region of a young patient. Further follow-
951 up is necessary for accurate assessment of our therapy strategy.
Acknowledgement We thank Dr. W. Sterlacci for editing the article and A. Piontke for excellent photodocumentation.
Conflict of interest None.
Funding None.
References 1. Johnson KJ, Dannenbaum MJ, Bhattacharjee MB, et al. Diagnosing cranial fasciitis based on distinguishing radiological features. J Neurosurg Pediatrics 2008;2:370e4. 2. Lauer DH, Enzinger FM. Cranial fasciitis of childhood. Cancer 1980 Jan15;45(2):401e6. 3. Summers LE, Florez L, Berberian ZJ, Bhattacharjee M, Walsh JW. Postoperative cranial fasciitis. Report of two cases and review of the literature. J Neurosurg 2007;106(6):1080e5. 4. Hussein MR. Cranial fasciitis of childhood: a case report and review of literature. J Cutan Pathol 2008 Feb;35(2):212e4.