AP Conference

April 2012

42 y/o Female Mass right hip/thigh present for several months

Presenting Complaint

T2-weighted MRI

6 x 5.1 x 4.2 spheroid soft tissue Smooth glistening capsule-like layer of connective tissue Cut surface soft tan-white, glistening with a small amount of mucoid material

Gross

Juxta-articular Myxoma

Diagnosis

Soft tissue, right hip/thigh, excision: Juxta-articular Myxoma. Comment The mass was well-demarcated and surrounded by a thin layer of edematous fibrofatty tissue that was focally disrupted. The lesion appears to be completely excised. Histology is typical of benign myxoma. It is noted from the operative report that it was closely associated with the hip joint.

Diagnosis

Soft tissue, right hip/thigh, excision: Juxta-articular Myxoma. Comment The mass was well-demarcated and surrounded by a thin layer of edematous fibrofatty tissue that was focally disrupted. The lesion appears to be completely excised. Histology is typical of benign myxoma. It is noted from the operative report that it was closely associated with the hip joint.

Diagnosis

Concept of myxoma as a single entity

Stout AP. Myxoma, the tumor of primitive mesenchyme. Ann Surg. 1948 Rosai & Ackerman 2011 Differential Diagnosis in Surgical Pathology 2010

Myxoma: a single entity?

Myxoma: a single entity?

Allen PW. Myxoma is not a single entity: a review of the concept of myxoma. Ann Diagn Pathol. 2000 Markku Miettinen, eds. Modern Soft Tissue Pathology 2010

Myxoma: a single entity?

Myxomas encompass a heterogenous group of clinicopathologic entities, common to which is prominent content of extracellular mucin. Soft tissue myxomas are fibroblastic-myofibroblastic neoplasms, or in some cases possibly non-neoplastic proliferations.

Modern Soft Tissue Pathology 2010

Myxoma: a single entity?

Enzinger FM Am J Clinical Pathol 1965 Intramuscular myxoma Superficial angiomyxoma Juxta-articular myxoma

2F:1M McCune-Albright syndrome

Mx intramuscular myxomas, endocrine, polyostic fibrous dysplasia

1.5M:1F Carney complex

Cutaneous and cardiac myxomas

3M:1F

No recurrence Slow growing mass Somatic mutation Gs alpha gene. ACTH, TSH, LH & FSH also regulated by Gs protein.

Recurrence common Recurrent chromosomal changes reported including rearrangements of 12p1 and 17p1. Carney complex mutations of PRKAR1A gene (17q24).

Recurrence common Case report: t(8;22)(q1112;q12-13) and trisomy of ch. 7. No Gs mutation found.

Myxoma: a single entity?

Meis JM, Enzinger FM: Juxta-articular myxoma: a clinical and pathologic study of 65 cases. Hum Pathol 1992

Juxta-articular myxoma

60% between ages 20-50 y/o 3M:1F Most <5cm (case reports >10 cm) Recurrence common (esp. if excision incomplete) Associated with joint damage

Juxta-articular myxoma

After IV gadolinium chelate

T1-weighted MRI

T2-weighted MRI

T1

T2

Gadolinium

Myxoid neurofibroma Intramuscular myxoma Myxoid liposarcoma Low grade fibromyxoid sarcoma Low grade myxofibrosarcoma Cellular myxoma Nodular fasciitis

Differential

Myxofibrosarcoma Pleomorphism present More cellular

Intramuscular & Juxta-articular myxoma Uniformly small bland cells Extremely paucicellular

Mitotic figures maybe present

Arching vessels

Mitotic figures never present

Markedly hypovascular

Differential

Locations

Clinical features

Location

Histology Differential

Juxta-articular myxoma

Questions?

60 y/o Female with dyspnea CT imaging: large intrathoracic goiter compressing trachea and bilateral pulmonary nodules

Presenting complaint

A.

B.

Four pieces of white-tan tissue 1.5 1 0.4 cm in aggregate. Two pieces of firm pink-tan tissue 4 2.5 2 cm in aggregate. Outer surface irregular. Uniform, firm, pale white-yellow cut surface.

Gross

Dedifferentiated (anaplastic) thyroid carcinoma

Diagnosis

a. Tissue submitted as thyroid, biopsy:

Dedifferentiated (anaplastic) thyroid carcinoma (see comment)

b. Left thyroid lobe, wedge biopsy:

Dedifferentiated (anaplastic) thyroid carcinoma

Diagnosis

Comment Both specimens show similar features. A poorly differentiated carcinoma is present composed of relatively large pleomorphic cells which have elicited a desmoplastic stromal reaction. Invasion into skeletal muscle is present. Numerous mitotic figures are present and large areas of the tumor are necrotic. In some areas the neoplastic cells have squamoid features. In specimen B, this neoplasm can be seen evolving from a recognizable thyroid carcinoma which has a mixed histologic appearance, with areas of papillary, follicular, and Hurthle cell features. Immunohistochemical stains show that cells in the dedifferentiated areas are positive for P63 and cytokeratin 5/6 and are negative for TTF1. The better differentiated areas of the tumor do stain positively with TTF-1 and lack staining for cytokeratin 5/6, and P63. Cytokeratin 7 is diffusely positive. The histology staining pattern is diagnostic for a dedifferentiated (anaplastic) thyroid carcinoma arising from a previous thyroid carcinoma with mixed histologic features. All anaplastic carcinomas of the thyroid are T4 by definition. In the AJCC staging system, this is a pT4, Nx, MX lesion although pulmonary nodules are present which may represent metastatic disease.

M:F ratio 1:1.1 to 1:2 2-5% of all thyroid carcinomas 30-40% where goiter is endemic (Alpine region) Radiation 50% cases inoperable at presentation 5 year survival rate 3.6-10% Rare survivors usually <60 y/o and small tumor (<5-6 cm)

Clinical features

Recent rapid enlargement of thyroid in patient with longstanding goiter (commonest) Rapid growth of thyroid mass in patient without goiter Recent rapid growth in patient with recurrent well-differentiated thyroid carcinoma Regional or distant metastatic tumor

Clinical Presentations

Extensive replacement of thyroid parenchyma Frequent invasion of adjacent soft tissue and structures Fleshy white-tan cut surface with frequent necrosis and hemorrhage

Gross

Gross

Undifferentiated thyroid carcinomas (UTC) are highly malignant tumors that histologically appear wholly or partially composed of undifferentiated cells that exhibit immunohistochemical or ultrastructural features indicative of epithelial differentiation.
Katoh R, Sakamoto A, Kasai N et al. Squamous differentiation in thyroid carcinoma. With special reference to histogenesis of squamous cell carcinoma of the thyroid. Acta Pathol Jpn 39: 306312.

WHO Definition

Widely invasive Admixture of spindle cells, pleomorphic giant cells & epithelioid cells. Variable elements. 20-30% with squamoid features Mitotic figures frequent Necrosis, neutrophilic infiltrate Obliteration of vascular lumina common

Histology

Angiomatoid variant Osteoclastic variant Rhabdoid variant Lymphoepithelioma-like carcinoma Paucicellular variant Carcinosarcoma Adenosquamous carcinoma Squamous cell carcinoma

Morphologic variants

Osteoclastic variant

Cytokeratin most frequently expressed (40100%) Thyroglobulin almost invariably negative TTF-1 rarely expressed

Regulates thyroglobulin expression

TP53 typically strongly positive

Immunohistochemistry

Favor undifferentiated carcinoma when pleomorphic tumor appears to arise within thyroid, especially if patient is elderly and there is evidence of a residual differentiated thyroid tumor.

Differential

1.

2. 3.

4.
5. 6. 7. 8. 9.

Sarcoma. Primary sarcomas are rare; diagnose only with strong proof, such as smooth muscle or nerve sheath differentiation. Large cell lymphoma Metastatic carcinoma Parathyroid carcinoma Riedels thyroiditis Angiosarcoma Solid variant of papillary carcinoma Poorly differentiated thyroid carcinoma Thymic and related tumors.

Differential

Questions?

1.

2.

3.

Meis JM, Enzinger FM: Juxta-articular myxoma: a clinical and pathologic study of 65 cases. Hum Pathol 1992; 23:639. Murphey MD, McRae GA: Imaging of soft tissue moxyoma with emphasis on CT and MR and comparison of radiologic and pathologic findings. Radiology 2002; 225:215. Korver RJP, Theunissen PHMH, van de Kreeke WT: Juxta-articular myxoma of the knee in a 5year-old boy: a case report and review of the literature. Eur Radiology 2010; 20:764-768.

References