Abstract
Objective
To describe a difficult case of a large extra-abdominal desmoid fibroma of the posterior neck and back; to discuss the pathologic findings and treatment options of this case and to review the current literature for a rare presentation of this disease.
Method
A case report and review of the current relevant English literature, carried out using PubMed Medline, are presented.
Results
We present a challenging case in which a locally invasive desmoid of the posterior neck and back had grown to such an extent that complete surgical excision in one procedure was not possible.
Conclusion
Extra-abdominal desmoid fibromas are rare tumors with multiple treatment options. The literature supports incomplete surgical resection when necessary to reduce postoperative morbidity. Further options described for residual or recurrent disease include repeat surgical excision, radiation therapy, and possible chemotherapy. For particularly large tumors, close observation and a planned second stage procedure are an appropriate choice.
1
Introduction
Desmoid tumors, or deep-type musculoaponeurotic fibromatoses, are rare infiltrative fibroblastic tumors that present complex management considerations. Wide local excision with clear margins has long been the gold standard treatment , but despite its classification as a histologically benign neoplasm without metastatic potential, desmoid fibromatoses are poorly encapsulated and locally invasive, making complete surgical excision difficult and often functionally morbid. Their propensity for recurrence poses an additional treatment dilemma.
Multimodality approaches such as radiation therapy, hormonal therapy, or cytotoxic chemotherapy are increasingly employed as primary treatments or as adjuvant therapies in incomplete resection or recurrence , along with a growing focus on more conservative and individualized strategies. Extra-abdominal desmoid tumors of the head and neck in particular commonly infiltrate vital neurovascular structures including the brachial plexus , emphasizing the importance of function-sparing surgery. In light of these trends, we report a case of a large extra-abdominal desmoid tumor causing significant morbidity and discuss the literature around treatment strategies.
2
Case report
A 26-year-old male presented with a five-year history of progressively enlarging posterior neck mass ( Fig. 1 ) causing significant pain and impaired neck range of motion. A magnetic resonance imaging (MRI) of the tumor showed a 27 cm by 17 cm mass of the paraspinal muscles bilaterally ( Fig. 2 ), extending from the posterior cervical neck to the upper back. The patient underwent surgical excision by a combined otolaryngology and neurosurgery team. The tumor was poorly encapsulated and found to have an extensive vascular supply. The mass was not excised in its entirety due to excessive intraoperative blood loss and the potential morbidity of complete surgical resection.
The excised cervical mass was 9 cm by 7 cm by 4.5 cm, weighing 0.15 kg; the specimen from the upper back measured 18 cm by 15 cm by 7.5 cm, weighing 1.62 kg. The histopathology of the mass revealed poorly circumscribed tumor growth pattern composed of proliferating stellate to spindle cells arranged in long fascicles or whorling patterns with bland nuclear features and dense keloid-like collagen in areas ( Fig. 3 ). The cells did not show nuclear atypia or hyperchromasia. The tumor cells infiltrated into surrounding soft tissue and skeletal muscle bundles. Immunohistochemistry stains from SMA, CD34, MIB-1, and C-kit were negative, while elastic tissue and collagen stains were positive. A diagnosis of extra-abdominal desmoid fibroma was rendered. The patient did very well post-operatively ( Figs. 4 and 5 ). Unfortunately, he was lost to follow-up after the 2010 earthquake in Haiti.
2
Case report
A 26-year-old male presented with a five-year history of progressively enlarging posterior neck mass ( Fig. 1 ) causing significant pain and impaired neck range of motion. A magnetic resonance imaging (MRI) of the tumor showed a 27 cm by 17 cm mass of the paraspinal muscles bilaterally ( Fig. 2 ), extending from the posterior cervical neck to the upper back. The patient underwent surgical excision by a combined otolaryngology and neurosurgery team. The tumor was poorly encapsulated and found to have an extensive vascular supply. The mass was not excised in its entirety due to excessive intraoperative blood loss and the potential morbidity of complete surgical resection.
