Teratocarcinosarcoma An Enigmatic Entity
SV Kane, AA Karpate. Tata Memorial Hospital, Mumbai, Maharashtra, India
Background: Teratocarcinosarcoma (TCS) is an unusual and highly aggressive clinicopathologic entity. It has variegated histomorphology characterized by epithelial, mesenchymal and neuroectodermal components. The morphologic diversity, a histologic hallmark, is responsible for diagnostic errors. This study is aimed at analyzing entire morphologic spectrum in excision specimen and evaluating diagnostic criteria in biopsy.
Design: This is a single institutional retrospective study comprising 25 cases diagnosed as TCS on review of HE slides. Performed pertinent immunohistochemistry. Clinical, follow up data recorded & correlated with histology.
Results: There were 20 males & 5 females with age spanning 10-65 years. The primary sites were nasal cavity, maxillary sinus, ethmoid sinus, oropharynx & oral cavity. Presence of all 3 elements in the first biopsy enabled a confident diagnosis of TCS in 28 % cases. In remaining cases, diagnoses of olfactory neuroblastoma, lymphoma, rhabdomyosarcoma, poorly differentiated carcinoma & inflammatory polyp were entertained depending on the predominant element in biopsy TCS exhibited intermingling of multiple tissues of varying maturity derived from 3 elements. The epithelial element comprised either of poorly differentiated carcinoma or benign glands mixed with fetal squamous epithelium. The glands showed squamous morules & were surrounded by smooth muscles imparting an organoid pattern. Fetal squamous epithelium was the characteristic diagnostic feature[10 cases] The mesenchymal elements ranged from fasciculated spindle cell sarcoma, rhabdomyosarcoma & chondrosarcoma to mature osteocartilagenous tissue, skeletal muscle tissue and myxoid matrix. The third & predominant element represented primitive neuroectodermal tissue with rosettes & neurofibrillary matrix Fifteen cases underwent excision of tumor followed by postoperative radiotherapy. Recurrence and nodal metastasis were observed in 3 patients each, bone metastasis in one. The post chemotherapy excision specimen exhibited extensive neural maturation in one case.
Conclusions: TCS exhibits heterogeneous tissues of variable maturity comprising 3 elements. Erroneous diagnoses on biopsy are common A high index of suspicion & adequate sampling are prerequisites for diagnosis. Though 3 elements are necessary for confident diagnosis on biopsy, combination of tissues derived from any 2 elements should lead to suspicion. Fetal squamous epithelium offers a diagnostic clue If diagnosed on biopsy, neoadjuvant chemotherapy can be offered to this aggressive tumor to induce maturation.
Category: Head & Neck
Monday, March 9, 2009 1:00 PM
Poster Session II # 156, Monday Afternoon