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A Case of Invasive Sinonasal Carcinosarcoma: The Importance of Early Detection.

1. Introduction

A malignant biphasic neoplasm consisting of an epithelial (squamous) element and mesenchymal component is known as a carcinosarcoma (also known as pleomorphic carcinoma, spindle cell carcinoma, pseudosarcoma, and pseudosarcomatous squamous cell carcinoma) [1, 2] although the exact nomenclature and subclassifications are variable [3]. It is classified under squamous cell carcinoma by the World Health Organization (WHO) [4].

Carcinosarcoma may arise from any squamous epithelium (e.g., salivary glands, respiratory tract, upper aerodigestive tract, and female reproductive organs) [5], but its occurrence in the sinonasal region is extremely rare [1]. There is very little evidence available about its best management.

2. Case Presentation

A 75-year-old gentleman first presented to primary care with a three month history of left-sided headaches and diplopia. He also complained of altered taste sensation and paraesthesia in the left maxillary region. There was no history of epistaxis. The patient attended his general practitioner on multiple occasions and was given sinusitis treatment until a CT scan was finally performed due to persistence of symptoms, upon which the patient was then referred to the ENT service.

He has a background history of fast atrial flutter (on bisoprolol), hypertension, polymyalgia rheumatica, benign prostatic hyperplasia, and previously excised papillary squamous cell carcinoma in the left thigh. He quit smoking 40 years prior to presentation. Otherwise, he had lived independently with WHO performance status of 1 (Karnofsky status 90).

The patient underwent complete head and neck examination including flexible nasoendoscopy. There was no discreet neck lymphadenopathy. Nasoendoscopy revealed a mass down to the left inferior turbinate obscuring the left nasal cavity. He complained of diplopia on the left side on the lateral gaze with proptosis of approximately 3 mm compared to the contralateral side. Vision was 6/30 bilaterally with glasses. There was no relative afferent pupillary defect or papilloedema.

2.1. Investigations. Initial CT scan revealed an aggressive lesion in left ethmoidal and frontal sinuses, invading the left orbit and anterior cranial fossa. CT neck and thorax showed no cervical or chest lymphadenopathy.

Subsequent MRI imaging also showed evidence of bone erosion with breaching of the dura in the vicinity of the left orbitofrontal cortex although there was no signal change in the brain to suggest brain invasion (Figure 1). There was destruction of left lamina papyracea. An incidental right anterior cranial fossa meningioma, distant from sinonasal lesion was also identified. This pathologic finding did not have any significant clinical relevance with the primary disease.

A staged whole body positron-emission tomography (PET) scan showed no other distant lesions but confirmed lesion progression through the frontal sinus.

Our initial differential diagnoses were squamous cell carcinoma, carcinosarcoma, lymphoma, teratocarcinosarcoma, olfactory neuroblastoma, small cell carcinoma and alveolar rhabdomyosarcoma.

2.2. Treatment. After the diagnostic workup, the patient underwent endoscopic examination of the nose and biopsy of the lesion under general anaesthesia. Extensive disease was noted at the ipsilateral maxillary antrum. Histology revealed an extensive necrotic biphasic epithelioid, spindled malignant neoplasm in keeping with carcinosarcoma. Immunohistochemistry afterwards showed no loss of DNA mismatch repair (MMR) protein expression.

2.3. Outcome. After a successful biopsy and radiological investigative workup, the patient was discharged home. Unfortunately in the community, his preexisting comorbidities worsened, and he developed poorly controlled fast atrial flutter and urosepsis with E. Coli bacteraemia. He was readmitted for antimicrobial and supportive treatment. The patient eventually recovered and was discharged.

Discussion was undertaken at the multidisciplinary meeting and with international experts in the field. The consensus is that although the disease can potentially be resected with major surgery such as craniofacial resection, due to its location and size, surgical treatment would have significant risk of bleeding, cerebrospinal fluid (CSF) leak, and meningitis. After detailed consultation and discussion about the possible treatment options including the risks of such procedures with the patient and his family, he was not keen to proceed and opted for palliative radiotherapy and symptomatic management.

The radiotherapy dose delivered was 20 Gy in five days, then a two-week break and finally another 20 Gy in five days. One year after diagnosis, patient tolerated the radiotherapy and continued to lead an independent life.

3. Discussion

In the head and neck area, carcinosarcomas most frequently occur in the larynx and oral cavity, followed by the skin, tonsils, sinonasal tract, pharynx and hypopharynx [6, 7]. Histologically, they have been classified as part of a spectrum of sarcomatoid carcinomas, most of which present in late middle-aged men with a long history of tobacco use. They consist of foci of overt carcinoma admixed with areas of divergent differentiation into mesenchymal tissues [8]. Spindle cell formation is also typical in the sarcomatous component. A recent study suggested that a MET protooncogene mutation may be a prerequisite event in its pathogenesis [9].

Presenting symptoms of sinonasal carcinosarcomas typically include nasal obstruction, epistaxis, facial pain, and headache [10,11]. These are rather nonspecific to the disease. They tend to grow rapidly, with extensive local destruction. Therefore, early diagnosis and aggressive therapy are necessary to improve the often dismal prognosis [2, 8].

In a Japanese case report of maxillary carcinosarcoma, the patient died despite intensive radiochemotherapy and total maxillectomy due to rapid tumour recurrence and metastasis. However, it appeared the carinomatous component responded to the radiochemotherapy but that was not the case with the sarcomatous component [11].

Nonetheless, aggressive treatment may not cease disease progression and improve survival. Cheong et al. [12] reported on a 61-year-old male patient who underwent total maxillectomy and modified radical neck dissection and died shortly afterwards with sternal metastasis, despite having had extensive surgery with curative intent.

Table 1 shows the similar cases of sinonasal carcinosarcoma reported in the literature [1, 2, 9-31]. The optimal treatment of this disease remains undetermined. It is difficult to ascertain the effectiveness of the treatment modalities (a combination of surgery, radiotherapy, and chemotherapy) from the small number of cases available in the literature. They generally confer a poor outcome and a high recurrence rate. A recent large case-control analysis of 15 sinonasal patients in America showed an average five-year disease-specific survival of 48.5%, which is significantly poorer than controls with carcinosarcoma at other anatomical sites [5]. Therefore we advocate, when patients are operatively fit with resectable disease, they should undergo aggressive surgical treatment with adjuvant radiotherapy as an attempt to improve outcome. This is because radiotherapy alone tends to convey a less favourable outcome, and the role of chemotherapy is unclear.

Since the optimal management of carcinosarcoma remains uncertain, it is intuitive to study a related but separate (also highly aggressive) entity known as teratocarcinosarcoma [26, 33-35]. This tumour type also includes a component of neuroectodermal tissue and is much more prevalent in the medical literature. According to a recent systemic review of 49 patients, it is recommended that optimal treatment involves radical surgical resection followed by radiation therapy [25].

If more doctors are aware of this condition and the associated presenting symptoms, perhaps earlier diagnoses can be made. Therefore, patients may potentially get the option of a curative total resection, since the invasion of the skull base and surrounding structures is a poor prognostic factor.

In conclusion, sinonasal carcinosarcomas represent rare neoplasms, with aggressive character and unfavourable prognosis. Here we present a case of extensive sinonasal carcinosarcoma extending into the anterior cranial fossa and into the orbit and also a review of the current international literature regarding this rare yet aggressive neoplasm. There is currently a lack of specific guidelines on the optimal management of sinonasal carcinosarcoma, and the treatment represents a challenge for the clinicians. The key message that we would like to disseminate to our colleagues is the importance of suspicion and early detection, as well as the necessity to adopt a holistic approach when counselling patients.

https://doi.org/10.1155/2018/2745973

Consent

The patient has consented for publication in written form.

Conflicts of Interest

The authors declare that there are no conflicts of interest regarding the publication of this article.

References

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Jason Yuen [ID], (1) Vinay Varadarajan, (2) Marios Stavrakas [ID], (2) Samiul Muquit, (1) and Hisham Khalil [ID], (2)

(1) South West Neurosurgery Centre, Derriford Hospital, Plymouth PL6 8DH, UK

(2) Department of Ear, Nose and Throat, Derriford Hospital, Plymouth PL6 8DH, UK

Correspondence should be addressed to Hisham Khalil; hisham.khalil@plymouth.ac.uk

Received 20 February 2018; Accepted 19 March 2018; Published 22 April 2018

Academic Editor: Kamal Morshed

Caption: FIGURE 1: MRI head scan shows breaching of dura mater. Red arrow denotes lesion of interest. (a) Axial slide. (b) Coronal slide.
TABLE 1: Cases of sinonasal carcinosarcoma that are reported
in the literature.

Number         Age (years)/sex         Location

1                    62/F           Maxillary sinus
2                    71/M             Maxillary +
                                    ethmoid sinuses
3                    65/F           Maxillary sinus
4                   57/F,          Nasoethmoid sinus
               (postradiation)
5                    60/M         Nasomaxillary sinus
6                    53/M             Maxillary +
                                    ethmoid sinuses
7                    81/F           Maxillary sinus
8                    47/M           Maxillary sinus
9                    54/M           Maxillary sinus
10 case          Mean age at       Sinonasal track,
  series         diagnosis 54        unclear exact
  (19 cases)    (range, 42-66)         locations
               Male 11 Female 8
11                   75/M            Nasal cavity
12                   60/M           Maxillary sinus
                                   and nasal cavity
13                   29/F           Nasopharyngeal
14                   60/M             Maxillary +
                                    ethmoid sinuses
15                   29/M            Nasal cavity
16                   52/M         Frontal, sphenoid,
                                     ethmoid, and
                                   maxillary sinuses
17                   61/M           Maxillary sinus
18 case          Mean age at        Nasal cavity 7
  series        diagnosis 60.3     Maxillary sinus 5
  (15 cases)      (SD, [+ or        Ethmoid sinus 1
                   -]21.3)          Frontal sinus 1
                    Male 6         Sphenoid sinus 1
                   Female 9
19                   66/M           Maxillary sinus
20                   78/F            Ethmoid sinus
21                   66/M           Maxillary sinus
22                   68/M           Maxillary sinus
23                   46/M           Maxillary sinus
24                   54/M           Sphenoid sinus
25                   55/M           Maxillary sinus
26                   35/F           Maxillary sinus
27                   75/M              Ethmoid +
                                    frontal sinuses

Number           Stage          Treatment

1                  ND              RT
2               T4aN0M0       Preoperative
                              RT + TM + RE
3                  ND            TM + RT
4                  ND        Tumor excision,
                              ethmoidectomy
                            and turbinectomy
5                T3N0M0       TM + RT + CT
6               T4aN0M0     TM + craniofacial
                                resection
                                + RT + CT
7                T3N0M0         TM + RT +
                              2nd operation
8                  ND             PM+RT
9                T3N3M0          RT + CT
10 case          T1/2 3       Surgery alone
  series        T3/4 16       2 Surgery and
  (19 cases)     N0 19       RT 7, RT alone
                 M0 19         2 CT + RT 2
11               T4N0M0     Surgery + CT + RT
12              Unclear          CT + RT
13              T1N2cM0            RT
14               T3N0M0       TM + RT + CT
15               T2N0M0          Surgery
16              T4aN0M0       TM + RT + CT
17              T4aN2cM1    TM with modified
                             neck dissection
18 case          T1/2 3      Surgery alone 3
  series        T3/4 10     surgery and RT 7
  (15 cases)      TX 2      surgery and RT 7
                  N0 9         RT alone 4
                  N+ 0        No therapy 1
                  NX 6
                 M0 14
                  M1 0
                  MX 1
19               T3N0M0       PM + RT + CT
20               T4N0M0       Surgery + RT
21             Recurrence      Palliative
22             Recurrence      Palliative
23               T4N0M0        Palliative
24              T4 N and    Surgery + CT + RT
               M unclear
25              T4aN0M0            RT
26               T4N0M0       PM + CT + RT
27              T4aN0M0            RT

Number                Outcome              Author, year

1              No marked improvement     Meyer and Shklar,
                 of the tumor, DWD           1957 [24]
2              ND about postoperative    Feinmesser etal.,
                  tumor condition,           1982 [16]
                    death due to
                   intracerebral
                     abscess at
                postoperative period
3                 LR, DWD 8 months       Ampil, 1985 [14]
                       later
4                LR 5 months after         Hafiz et al.,
                  surgery, outcome           1987 [17]
                     uncertain
5                 LR, DWD 2 months       Sonobe, 1989 [11]
                   later (diffuse
                 metastasis in lung
                     and brain)
6                Disease free after          Shindo et
                      9 months             al., 1990 [2]
7                 LR, DWD 3 months          Sanabre et
                    after second          al., 1998 [27]
                     operation
8               LR, DWD after 1 year         Furuta et
                                          al., 2001 [10]
9                  DWD 4 months;             Howard et
                 possibly from lung       al., 2007 [19]
                     metastasis
10 case            Mean follow-up            Doshi et
  series          38 months (6-40)        al., 2010 [15]
  (19 cases)     5 with disease at
                  last follow-up 0
                 death at follow-up
11               Disease-free after         Terada and
                      5 years           Kawasaki, 2011 [30]
12               DWD after 9 months      Terada, 2011 [29]
13                   Clinically             Lim et al.,
                  free-of-disease            2012 [22]
                 2 months after RT
14                     LR, FL             Moon, 2013 [1]
15               Disease-free after      Gupta, 2013 [32]
                      6 months
16                       LR                  Alem and
                                        AlNoury, 2014 [13]
17               DWD shortly after           Cheong et
                    surgery with          al., 2014 [12]
                 sternal metastasis
18 case              Five-year               Patel et
  series          disease-specific         al., 2015 [5]
  (15 cases)     survival of 48.5%
19               LR, DWD 10 months         Ando et al.,
                   after initial             2015 [9]
                    presentation
20                  Disease-free           Iqbal et al.,
                  after 36 months            2015 [20]
21              DWD after 12 months
22               DWD after 1 month
23               DWD after 5 months
24              DWD after 12 months         Liu et al.,
                                             2016 [23]
25                 No significant           Hasnaoui et
                   effect, died 4         al., 2017 [18]
                    months after
                initial examination
26              DWD after 12 months         Soltani et
                                          al., 2018 [28]
27              Remained independent      Our case, 2018
                  after 12 months

ND, not described; RT, radiation therapy; DWD, dead with disease;
TM, total maxillectomy; RE, removal of eye; LR, local recurrence;
CT, chemotherapy; PM, partial maxillectomy; FL, follow-up loss;
LNs, lymph nodes.
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Title Annotation:Case Report
Author:Yuen, Jason; Varadarajan, Vinay; Stavrakas, Marios; Muquit, Samiul; Khalil, Hisham
Publication:Case Reports in Otolaryngology
Date:Jan 1, 2018
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