| Abstract|| |
Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma, mostly occurring in the proximal extremities and limb girdle. Majority of the patients are in fifth and sixth decades of life with male preponderance. We report here a case of primary extraskeletal myxoid chondrosarcoma of the uterine adnexa involving the broad ligament and fallopian tube synchronously without any evidence of uterine/ovarian involvement in a young multiparous female of 27 years. After the histopathological diagnosis, re-excision of the tumor bed with wide local margins was recommended. Since the tumor has an aggressive course, with propensity for late recurrence and metastases to lungs, the patient must be considered for long-term follow-up.
Keywords: Extraskeletal myxoid chondrosarcoma, soft tissue sarcoma, uterine adnexa
|How to cite this article:|
Goyal S, Dev G, Mahajan S, Sharma S. Multicentric extraskeletal myxoid chondrosarcoma of uterine adnexa in a young female: An unusual presentation. Indian J Pathol Microbiol 2012;55:80-2
|How to cite this URL:|
Goyal S, Dev G, Mahajan S, Sharma S. Multicentric extraskeletal myxoid chondrosarcoma of uterine adnexa in a young female: An unusual presentation. Indian J Pathol Microbiol [serial online] 2012 [cited 2016 Oct 27];55:80-2. Available from: http://www.ijpmonline.org/text.asp?2012/55/1/80/94863
| Introduction|| |
Pelvic sarcomas are aggressive lesions with high propensity of local spread and metastases. Uterine adnexa is a rare site for the origin of these lesions. These warrant meticulous management, distinct from that of the usual carcinomas of female genitourinary tract. Rare sarcomas that have been specifically reported in adnexal region include malignant fibrous histiocytoma, embryonal rhabdomyosarcoma, alveolar soft part sarcoma, and hyalinizing spindle cell sarcoma with giant rosettes.
Extraskeletal myxoid chondrosarcoma (EMC) is a rare aggressive soft tissue neoplasm. It is most commonly seen in deep soft tissues of proximal extremities and limb girdle, especially thigh and popliteal fossa.  Of the female genital tract, EMC has been reported only in vulva.  We hereby report a case of EMC presenting as adnexal mass involving the broad ligament and Fallopian tube More Details synchronously, in a young female of 27 years, thereby suggesting a multicentric origin. To the best of our knowledge, this unusual site and age of presentation of primary EMC has not been described before.
| Case Report|| |
A 27-year-old multiparous female presented with pain and lump in the left lower abdomen for 2 months. The clinical possibility of fibroid was considered. MRI revealed two separate soft tissue masses, the larger one in relation to the broad ligament, on left side, and a smaller nodule in the left fallopian tube. These imaging findings were confirmed at laparotomy. Enucleation of the left broad ligament mass and left salpingectomy were carried out. Gross examination revealed soft, globular to nodular mass measuring 7 × 6 × 6 cm and a small gelatinous nodule measuring 1.5 × 1 × 1 cm within the fallopian tube wall [Figure 1]. Cut surface showed lobulated tumor mass with multiple, small nodular gelatinous gray-white areas and focal areas of cystic degeneration. Microscopy revealed a well-circumscribed tumor mass with a distinct multinodular pattern [Figure 2]. The tumor consisted predominantly of myxoid areas with few cellular areas. Individual cells were arranged in short anastomosing cords, strands, and pseudoacinar formations, creating a lace-like pattern in variable amount of myxoid matrix [Figure 3]a. The cells were round to slightly elongated, of uniform size and shape with a rim of moderate amount of deeply eosinophilic cytoplasm and indistinct cell borders. Nuclei were oval to slightly irregular, hyperchromatic with nuclear invaginations and grooving, features reminiscent of chondroblasts [Figure 3]b. Foci of cellular areas were composed of sheets of large round to epithelioid cells with vesicular chromatin, conspicuous to prominent nucleoli and deeply eosinophilic cytoplasm [Figure 3]c and d. No well-differentiated cartilage was identified. The tumor nodule limited within the fallopian tube wall showed similar morphology [Figure 4].
|Figure 1: Gross photograph shows cut open well-circumscribed nodular tumor measuring 7 × 6 × 6 cm. Inset: A small gelatinous nodule measuring 1.5 × 1 × 1 cm within the fallopian tube wall|
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|Figure 2: Low power view shows characteristic multinodular arrangement of the extraskeletal myxoid chondrosarcoma (H and E, ×40)|
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|Figure 3: Extraskeletal myxoid chondrosarcoma. (a) Tumor cells arranged in cords, strands, and pseudoacini in abundant myxoid background (H and E, ×100). (b) Higher magnification showing relatively monomorphic round to ovoid cells with a rim of deeply eosinophilic cytoplasm and hyperchromatic nuclei (H and E, ×200). (c) Cellular areas - epithelioid cells in sheets with little myxoid stroma (H and E, ×100). (d) High power showing vesicular nuclei with prominent nucleoli (H and E, ×400)|
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|Figure 4: Photomicrograph shows well-circumscribed myxoid tumor with pseudocapsule limited within the fallopian tube wall. (a) Luminal aspect. (b) Serosal aspect (H and E, ×40)|
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The abundant extracellular myxoid stroma stained deeply with alcian blue at pH 2.5 and 1.0 (inhibited by pretreatment with hyaluronidase). The myxoid tumor matrix stained metachromatically with toluidine blue at pH 4.0 and 1.0 and was positive for mucicarmine. PAS positive diastase sensitive material was seen within the vacuolated cytoplasm of tumor cells as in chondroblasts. On immunohistochemistry, the tumor cells showed diffuse immunoreactivity with vimentin and were negative for S-100, keratin, and EMA.
In the differential diagnosis, extraskeletal myxoid chondroma must be considered; however, this tumor is relatively hypocellular and has better differentiated cartilage. Diagnosis of chordoma is also unlikely due to absence of characteristic physaliphorous cells. Diagnosis of EMC was considered based on characteristic morphology, histochemistry, and IHC staining pattern, thereby excluding the other possibilities. EMC has characteristic multinodular pattern with round to elongated tumor cells aligned in short anastomosing cords, pseodoacini, and strands in variable amounts of myxoid matrix. Chondroblast-like cells having small hyperchromatic nuclei, occasional nuclear grooving, and a narrow rim of deeply eosinophilic cytoplasm, as seen in our case, are diagnostic. In literature, well-differentiated cartilage has been reported in about one third of tumors. Like chondrosarcoma of bone, EMC produces myxoid matrix rich in chondroitin-4-sulfate, chondroitin-6-sulfate, and keratin sulfate. Histochemistry supported the presence of sulfated glycosaminoglycans in tumor matrix in our case which distinguishes EMC from myxoma, myxoid liposarcoma, and myxoid variant of fibrohistiocytic neoplasms. On immunohistochemistry, the cells of EMC stain strongly with vimentin, while S-100 protein immunoreactivity has been reported in only 17% of cases.
As no evidence of contiguous/metastatic tumor spread was found, we suggest multicentric origin of this tumor. There has been only one prior case report of multicentric EMC described in synovial tissue. 
| Discussion|| |
EMC is a rare slow growing tumor that may recur and/or metastasize to lungs. Peak incidence is in fifth and sixth decades with few cases encountered in children and adolescents. Males are affected more frequently. Ours is the first case of primary EMC involving the broad ligament and fallopian tube in a young female without any evidence of uterine/ovarian origin. Studies have shown most patients of EMC have 5 year survival rate of 75% despite of high recurrence and metastasis.  Few patients have an aggressive course with 30-50% mortality within 3-5 years. Recurrence has been reported up to 8 years after the initial diagnosis. 
After the histopathological diagnosis of EMC was given, re- excision of the tumor bed with wider margins was recommended. The patient has been on regular clinical and imaging follow up for 2 years and has shown no evidence of tumor recurrence or metastasis.
It is important to distinguish this rare entity from the usual tumors presenting as adnexal mass including leiomyoma, metastatic sarcomas, and fallopian tube malignancies. Early and accurate diagnosis of EMC is crucial so as to plan for wide local excision of the tumor and adjuvant radiotherapy with a long-term follow-up.
| References|| |
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|2.||Santacruz MR, Proctor L, Thomas DB, Gehrig PA. Extraskeletal myxoid chondrosarcoma: A report of a gynaecologic case. Gynaecol Oncol 2005;98:498-501. |
|3.||Kindblom LG, Angervall L. Myxoid chondrosarcoma of the synovial tissue A clinicopathologic, histochemical and ultrastructural analysis. Cancer 1983;52:1886-95. |
|4.||Oliveira AM, Sebo TJ, Mc Grory LE, Gaffey TA, Rock GM, Nascimento GA. Extraskeletal myxoid chondrosarcoma: A clinicopathological, immunohistochemical and ploidy analysis of 23 cases. Mod Pathol 2000;13:900-8. |
Department of Pathology, Room 78, Gents Hostel 8, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
[Figure 1], [Figure 2], [Figure 3], [Figure 4]