Cyclopean and Prodigious-Giant Cell Tumour Soft                       Tissue

Anubha Bajaj

Histopathologist in A.B. Diagnostics, New Delhi, India

Correspondence: Anubha Bajaj, Histopathologist in A.B. Diagnostics, New Delhi, India. 

E-mail: [email protected]

Received: July 25, 2023                                       |                                 Published: August 26, 2023

Citation: Anubha B. Cyclopean and Prodigious-Giant Cell Tumour Soft Tissue. AOJ Emerg and Int Med. 2023;1(1):03-05.

Giant cell tumour of soft tissue is an exceptionally encountered, low grade neoplasm whichmorphologically simulates and appears as an analogue of giant cell tumour of bone. Associated withminimal malignant potential, neoplasm appears devoid of genomic mutations within H3F3A gene, a feature which segregates the lesion from giant cell tumour of bone. Neoplasm is additionally designated as soft tissue giant cell tumour of low malignant potential, giant cell tumour of soft parts, extra-skeletal giant cell tumour orthe infrequent osteoclastoma of soft tissue. Tumefaction may be optimally subjected to comprehensive surgical eradication.

Although tumour incidence remains undetermined, an equivalent gender predilection is encountered. Average age of disease emergence is ~40 years although the condition may be discerned within infancy or ninth decade. Giant cell tumour of soft tissue may be concurrent with Paget’s disease of bone.1,2

Giant cell tumour of soft tissue commonly incriminates distal upper extremities as arms or hand. Besides, thighs are frequently implicated. Commonly multifocal, the neoplasm may be confined to superficial or deep seated soft tissue.

1. Clinically, a painless tumefaction is exemplified.1,2 Upon gross examination, a well circumscribed, nodular, fleshy, reddish brown or grey tumourappears confined to the dermis or subcutaneous tissue. An estimated 30% neoplasms emerge beneath superficial fascia. Tumour magnitudemay extend up to 10 centimetres. Cut surface is gritty on account of peripheral calcification.1,2

Upon cytological examination, tumefaction is constituted of mononuclear cells intermingled within an intercellular stroma. Stromal cells are pervaded with poorly defined cytoplasm and elliptical to spindle shaped nuclei. Significant cellular or nuclear atypia is absent.                                              

Osteoclast-like giant cells imbued with bland, vesicular nuclei are evenly distributed throughout the lesion.1,2

Upon microscopy, a multinodular, diffusely infiltrative soft tissue neoplasm is delineated. Tumefaction demonstrates an admixture of bland, spherical or elliptical mononuclear cells, spindle shaped cells and osteoclast-like, multinucleated giant cells. Tumour nodules constituted of spindle shaped cells with commingled stroma mayexhibit a storiform or fascicular configuration.3,4

Giant cell tumour of soft tissue exemplifiesperipheral dissemination of metaplastic bone which may expand into centric zones.                           

Focal haemorrhage and cystic areas, simulating secondary aneurysmal bone cyst-like alterations are frequently encountered. Tumefaction delineates significant cellular andnuclear atypia along with numerous mitotic figures. Vascular tumour invasion is delineated in up to 50% instances. Tumour necrosis is uncommonly enunciated.3,4 

Figure 1. Giant cell tumour of soft tissue comprised of osteoclast-like giant cells intermingled within a stroma composed of elliptical or spindle shaped mononuclear cells configuring a focal storiform pattern. Foci of haemorrhage seen.6

Figure 2. Giant cell tumour of soft tissue delineating osteoclast-like giant cells commingled within fascicles of stroma constituted of oval and spindle shaped, mononuclear cells with a focal storiform pattern. Focal haemorrhage is encountered.7 

TNM classification of thoracic and limb soft tissue sarcoma3,4

Primary Tumour

• T1: Tumour magnitude is ≤ 5centimetres
• T2: Tumour magnitude is > 5 centimetres and ≤10 centimetres
• T3: Tumour magnitude is >10 centimetres and ≤15 centimetres
• T4: Tumour magnitude is >15 centimetres

Regional lymph nodes

• N0: Regional lymph node metastasis absent
• N1: Regional lymph node metastasis present

Distant metastasis

• M0: Distant metastasis absent
• M1: Distant metastasis present into pulmonary parenchyma or diverse viscera

Staging of soft tissue sarcoma is denominated as

• stage Ia: Tumour magnitude is ≤ 5 centimetres with absent regional lymph node or distant metastasis. Tumour cells are GX or G1
• stage Ib: Tumour magnitude is> 5 centimetres with absent regional lymph node or distant metastasis. Tumour cells are GX or G1
• stage II: Tumour magnitude is ≤5 centimetres with absent regional lymph node or distant metastasis. Tumour cells are G2 or G3
• stage IIIa: Tumour magnitude is > 5 centimetres and ≤10 centimetres with absent regional lymph node or distant metastasis. Tumour cells are G2 or G3
• stage IIIb: Tumour magnitude is > 10 centimetres with absent regional lymph node or distant metastasis. Tumour cells are G2 or G3
• stage IV:Tumour magnitude is variable and accompanied by regional lymph node metastasis OR distant metastasis into sites such as pulmonary parenchyma or diverse viscera. Tumour cell grade is variable.3,4

Giant cell tumour of soft tissue is immune reactive to vimentin, smooth muscle actin (SMA), CD68 or p63. Osteoclast-like giant cells appear reactive to tartrate resistant acid phosphatase (TRAP), TRAIL, RANKL and osteoprotegerin.4,5

Giant cell tumour of soft tissue is immune non reactive to CD45, S100 protein, desmin, lysozyme, SATB2 or H3.3G34W.4,5 Giant cell tumour of soft tissue appears devoid of somatic mutations withinH3F3A gene, a feature which segregates the tumefaction from giant cell tumour of bone.4,5 Giant cell tumour of soft tissue requires segregation from neoplasms such as giant cell tumour of bone, localized variant of tenosynovial giant cell tumour, giant cell rich variants of undifferentiated pleomorphic sarcoma, malignant melanoma with osteoclast-like giant cells orleiomyosarcoma with osteoclast-like giant cells.4,5 In the absence of cogent clinical symptoms or radiographic features, distinction of giant cell tumour of soft tissue from giant cell tumour of bone can be challenging.4,5 Upon imaging, tumefaction is confined to soft tissue. Few neoplasms may be accompanied by mineralization. Besides, the neoplasm may manifest as a cystic lesion demonstrating intrinsic nodules. Adjacent soft tissue exhibits nonspecific alterations.                                                          

Giant cell tumour of soft tissue is appropriately alleviated with surgical extermination of the neoplasm.4,5

Conservative management with surgical eradication and obtainment of tumour free surgical perimeter is optimal and recommended mode of therapy. Localized tumour reoccurrence appears in ~10% instances.       

Regional lymph node and distant metastasis are uncommonly discerned. However, malignant metastatic giant cell tumour of soft tissue is documented.4,5

References 

1. Lee SW, Lee J, Kim SJ et al. Giant cell tumour of soft tissue of the colon: a case report and review of the literature. BMC Gastroenterol. 2022;22:317.  
2. Chen P, Hu Q, Wu J. Giant cell tumour of soft tissue-a rare cause of mass in the liver: a case report. Front Surg. 2022.
3. Wakely PE. Giant cell tumour of soft tissue: FNA cytopathology of 4 cases, review of the literature, and comparison with giant cell tumour of bone. Cancer Cytopathol.2022;130:120–127.
4. Agaimy A, Michal M, Stoehr R et al. Recurrent novel HMGA2-NCOR2 fusions characterize a subset of keratin-positive giant cell-rich soft tissue tumors. Mod Pathol. 2021.34:1507–1520.
5. Luangxay T, Osako T, Yonekura R et al. Giant cell tumour of soft tissue of the breast: case report with H3F3A mutation analysis and review of the literature. Pathol Res Pract.2020.
6. Image 1 Courtesy: Research gate.
7. Image 2 Courtesy: Semantic scholar.

Copyright: ©2023 Bajaj. This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and build upon your work non-commercially.