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 Table of Contents  
Year : 2016  |  Volume : 33  |  Issue : 1  |  Page : 19-23

Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma of the larynx with giant cells and myogenic differentiation: a case report

1 Histopathology Department, King Khalid Hospital, Hail, Kingdom of Saudi Arabia
2 Surgery Department, King Khalid Hospital, Hail, Kingdom of Saudi Arabia

Date of Submission27-Mar-2016
Date of Acceptance19-Apr-2016
Date of Web Publication28-Nov-2016

Correspondence Address:
Laila Seada
Professor, Histopathology Department, Faculty of Medicine, Benha University, Qalyoubia Province, 13518
Kingdom of Saudi Arabia
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1110-208X.194383

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Undifferentiated pleomorphic sarcoma (UPS)/malignant fibrous histiocytoma (MFH) is quite rare in the larynx, with few cases reported. In the present study, we report an UPS/MFH of the larynx arising as a second primary neoplasm. It showed giant cells, spindle areas with vague storiform appearance, and myxoid areas. CD68 was positive in the giant cells and focally in the tumor cells. S100 showed scattered positivity in stellate cells. Desmin and specific muscle actin were negative, whereas h-caldesmon was positive in tumor cells and medium-sized blood vessels, denoting a possible myogenic differentiation. To the best of our knowledge this is one of the rare case reports of UPS/MFH with giant cells in the larynx arising as a second primary neoplasm.

Keywords: giant cells, larynx, second malignancy, undifferentiated pleomorphic sarcoma

How to cite this article:
Seada L, Al Rashid F, Oreiby H. Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma of the larynx with giant cells and myogenic differentiation: a case report. Benha Med J 2016;33:19-23

How to cite this URL:
Seada L, Al Rashid F, Oreiby H. Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma of the larynx with giant cells and myogenic differentiation: a case report. Benha Med J [serial online] 2016 [cited 2021 Dec 5];33:19-23. Available from: http://www.bmfj.eg.net/text.asp?2016/33/1/19/194383

  Introduction Top

Malignant fibrous histiocytoma (MFH) was first described in the early 1960 s and became widely accepted as a specific soft-tissue sarcoma type in the 1970 s [1],[2],[3]. In his 1992 article, Fletcher noted that the majority of cases initially diagnosed as pleomorphic MFH could be reclassified as other tumors after lines of differentiation were discovered on testing with more sensitive techniques [4]. In 2002, the WHO reappraised and modified the terminology and classification of MFH and its subtypes. Pleomorphic sarcoma is the alternate name advocated by the WHO to replace MFH, as it provides an accurate description of the tumor without implying the origin of the tumor cells [5]. Multiple subtypes of MFH, including storiform-pleomorphic, myxoid, giant cell, and inflammatory variants, were defined; subsequently, MFH was regarded as the single most common adult soft-tissue sarcoma [6].

MFH is a fairly common tumor in the deep soft tissues: the most frequent primary sites are the lower (49%) and upper (19%) limbs, but it has been reported even in the retroperitoneum and abdomen (16%), although localization in the larynx is quite rare [7],[8].

  Case report Top

The study design was approved by the local ethical committee of our organization. We here report a 75-year-old female patient with a past history of Hurthle cell carcinoma of the thyroid for which she had been operated upon twice (in 2002 and 2009) and received both chemotherapy and radiotherapy but discontinued treatment against medical advice. She had been admitted in 8 April 2014 to our hospital complaining of absolute dysphagia (for solids since 1 month; then for liquids since 2 days).

Computed tomography scan of the neck after intravenous contrast showed three soft-tissue lesions at the level of the thyroid cartilage. The larger one seen on the right side was prevertebral, and retropharyngeal in location (from which the biopsy was taken), measuring 5.4 × 4.3 × 3.8 cm, showing heterogeneous contrast enhancement and small areas of breakdown with slightly ill-defined borders ([Figure 1] and [Figure 2]). The lesion was seen creating a mass effect upon the upper portion of the laryngopharynx encasing the right aspect of the thyroid cartilage, encroaching upon the right sternomastoid muscle, attenuating the right jugular vein, and laterally displacing the right carotid artery. One of the other two lesions was at the midline just inclined to the right side, and the other one was on the left side anterior to the left sternomastoid. A few bilateral deep cervical lymph nodes, the larger on the left side measuring 1.5 cm, appeared cystic.
Figure 1 Computed tomography (CT) scan of laryngeal mass.

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Figure 2 Computed tomography (CT) scan of laryngeal mass.

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Computed tomography scan of the chest revealed multiple bilateral scattered subpleural nodules of variable sizes, a hypodense focal lesion of the liver at the left lobe, and multiple mediastinal lymph nodes, the largest measuring 16 × 11 mm. As the patient refused flexible endoscopy and examination, tracheotomy and biopsy were performed. The biopsy specimen measured 1 × 1 × 0.5 cm, was firm in consistency, and grayish-white in color. Histopathology showed a poorly differentiated subepithelial neoplasm with polymorphic morphology. Areas with vague storiform appearance and myxoid and spindle cell fascicles were recognized ([Figure 3] and [Figure 4]). Other areas showed polygonal cells with abundant eosinophilic cytoplasm ([Figure 5]). Osteoclast giant cells were seen scattered within the tumor and associated with stromal hemorrhage. In some areas, especially under the epithelium, cells showed wavy nuclei and eosinophilic cytoplasm ([Figure 6]). Mitosis was frequent in polygonal cells and ranged from 15 to 40 per 10 high-power fields ([Figure 7]). Immunostaining for CD68 showed strong positivity in giant cells and focally in tumor cells ([Figure 8]). Caldesmon was diffusely positive in tumor cells and in medium-sized blood vessels ([Figure 9] and [Figure 10]). Desmin-negative and muscle-specific actin was focally positive in spindle areas. S100 showed scattered positivity in stellate cells ([Figure 11]), whereas Ki-67 showed 40–50% labeling index. CD31, AE1/AE3, EMA, and tyrosinase antibodies were negative.{Figure3}
Figure 4 Areas of vague storiform appearance and spindled fascicles (H&E × 400).

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Figure 5 Polygonal cells with abundant cytoplasm and osteoclast-like giant cells (H&E × 400).

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Figure 6 Subepithelial areas with stellate cells (H&E × 100).

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Figure 7 Osteoclast-like giant cells within other areas showing mitosis in polygonal cells (H&E × 400).

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Figure 8 Strong cytoplasmic CD68 positivity in giant cells (Strept ABC/DAB × 400).

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Figure 9 Positive cytoplasmic and membranous immunostaining for h-caldesmon in tumor cells and in medium-sized blood vessels (Strept ABC/DAB × 400).

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Figure 10 Positive cytoplasmic and membranous immunostaining for h-caldesmon in tumor cells and in medium-sized blood vessels (Strept ABC/DAB × 400).

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Figure 11 S100 cytoplasmic immunostaining in stellate cells (Strept ABC/DAB × 100).

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  Discussion Top

The term MFH implies that the tumor cells are of fibroblastic and histiocytic origin; however, the precise origin of MFH cells has been disputed and the concept of fibrohistiocytic differentiation has been challenged [7]. Furthermore, the morphologic pattern seen with pleomorphic MFH is shared by a variety of poorly differentiated malignant neoplasms. The origin from an undifferentiated mesenchymal cell, capable of differentiation several lines, is also postulated [4].

The WHO nomenclature now designates storiform-pleomorphic MFH as ‘undifferentiated high-grade pleomorphic sarcoma’, giant cell MFH as ‘undifferentiated pleomorphic sarcoma with giant cells’, and inflammatory MFH as ‘undifferentiated pleomorphic sarcoma with prominent inflammation’. These three undifferentiated pleomorphic sarcomas (UPS) remain categorized as ‘so-called fibrohistiocytic tumors’ in the WHO classification. Myxoid MFH, however, is seen as a specific entity, now termed ‘myxofibrosarcoma’ by the WHO, and reallocated from the fibrohistiocytic category to the myofibroblastic tumor category [5].

Most undifferentiated high-grade pleomorphic sarcomas and UPS with giant cells occur in the deep soft tissues of the extremities or trunk. The most common site of involvement of UPS with prominent inflammation is the retroperitoneum, although intra-abdominal and deep soft-tissue locations have been encountered [9]. MFH has been reported to occur in the lungs, liver, kidneys, bladder, scrotum, vas deferens, heart, aorta, stomach, small intestine, orbit, central nervous system, paraspinal area, dura matter, facial sinuses, nasal cavity, oral cavity, nasopharynx, and soft tissues of the neck. UPS/MFH of the larynx is rare, with only few cases reported in literature. The giant cell subtype has been reported in only few previous case reports [8],[9],[10].

Guo et al. [4] assessed 33 previously diagnosed MFH cases based on the WHO classification to identify whether some of the MFH diagnoses would change. Among the 33 cases, 17 cases (51.5%) of MFH had their diagnosis changed, including five leiomyosarcomas, three malignant peripheral nerve sheath tumors, one fibrosarcoma, one inflammatory myofibrosarcoma, one giant cell tumor, and one angiomatoid fibrous histiocytoma. The remaining 16 cases (48.5%) were reconfirmed as MFH/UPS. Only vimentin was always expressed in MFH/UPS, whereas some of the tumors were positive for myogenic antigen and CD68 [4].

MFH may also occur secondary to radiation exposure and shrapnel injury and may be seen adjacent to metallic fixation devices, including total joint prostheses. Early and complete surgical removal using wide or radical resection is indicated because of the aggressive nature of the tumor [8],[9].

We have presented a case of laryngeal tumor presented as a second malignancy after irradiation due to thyroid carcinoma. It fulfilled the criteria for the diagnosis of UPS with giant cells. The immunohistochemical positivity for h-caldesmon in tumor cells suggests a myogenic differentiation.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Fletcher CD, Gustafson P, Rydholm A, Willén H, Akerman M. Clinicopathologic re-evaluation of 100 malignant fibrous histiocytomas: prognostic relevance of subclassification. J Clin Oncol 2001; 19:3045–3050.  Back to cited text no. 1
Fletcher CDM, Unni KK, Mertens F. . World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone. So-called fibrohistiocytic tumours. Lyon:IARC Press; 2002. 120–125.  Back to cited text no. 2
Nakayama R, Nemoto T, Takahashi H, Ohta T, Kawai A, Seki K et al. Gene expression analysis of soft tissue sarcomas: characterization and reclassification of malignant fibrous histiocytoma. Mod Pathol 2007; 20:749–759.  Back to cited text no. 3
Guo H, Xiong Y, Nong L, Zhang S, Li T. Reassessment of the pathological diagnosis in 33 cases of malignant fibrous histiocytoma. Beijing Da Xue Xue Bao 2008; 40:374–379.  Back to cited text no. 4
Pastore A, Grandi E, Targa L, Marchese Ragona R. Malignant fibrous histiocytoma of the larynx. Presentation of a clinical case and review of the literature. Acta Otorhinolaryngol Ital 2001; 21:361–364.  Back to cited text no. 5
Sabesan T, Xuexi W, Yongfa Q, Pingzhang T, Ilankovan V. Malignant fibrous histiocytoma: outcome of tumours in the head and neck compared with those in the trunk and extremities. Br J Oral Maxillofac Surg 2006; 44:209–212.  Back to cited text no. 6
Nascimento AF, Raut CP. Diagnosis and management of pleomorphic sarcomas (so-called ‘MFH’) in adults. J Surg Oncol 2008; 97:330–339.  Back to cited text no. 7
Resta L, Pennella A, Fiore MG, Botticella MA. Malignant fibrous histiocytoma of the larynx after radiotherapy for squamous cell carcinoma. Eur Arch Otorhinolaryngol 2000; 257:260–262.  Back to cited text no. 8
Alessandrini M, De Padova A, Saccoccio A, Ambrogi V, Napolitano B, Palmieri G, Bruno E. Post-irradiation malignant fibrous histiocytoma of the larynx: a case report with an unusual metastatic spread pattern. Auris Nasus Larynx 2009; 36:609–613.  Back to cited text no. 9
Anghelina F, Ioniţă E, Chiuţu L, Mogoantă CA, Ciolofan S, Iosif C, Ceauşu M. Malignant fibrous histiocytoma of larynx with giant cell: case report and histological-clinical considerations. Rom J Morphol Embryol 2009; 50:481–485.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8], [Figure 9], [Figure 10], [Figure 11]


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