Rare disease
CASE REPORT
Basaloid squamous cell carcinoma of the maxilla Jigna Pathak,1 Shilpa Patel,1 Janaki Subramanian Iyer,1 Neeta Mohanty2 1
Department of Oral Pathology, MGM Dental College & Hospital, Navi Mumbai, Maharashtra, India 2 Department of Oral Pathology and Microbiology, Institute of Dental Sciences, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India, Institute of Dental Sciences, Bhubaneswar, Odisha, India Correspondence to Dr Neeta Mohanty, [email protected] Accepted 14 May 2015
SUMMARY Basaloid squamous cell carcinoma (BSCC) is a distinctive aggressive variant of squamous cell carcinoma. We present a case of a 60-year-old man with tender swelling in the right cheek region for 6 months and continuous unilateral nasal discharge for 2 months. Extraoral examination revealed an ovoid, well-defined swelling from the right infraorbital rim to the angle of the mouth superoinferiorly and the right lateral wall of the nose to preauricular region anteroposteriorly. Intraorally, an ulceroproliferative growth from right upper gingivobuccal sulcus to mid palatine raphe with bicortical expansion was evident. CT revealed a hypodense mass obliterating the right maxillary sinus. Histopathology showed closely packed basaloid cells, with hyperchromatic palisading nuclei, arranged in a solid pattern with a lobular configuration. Prominent areas of comedo necrosis and keratin pearl formation were seen. These features suggested BSCC. The patient underwent surgical excision with adjuvant radiation but was lost to follow-up after 6 months of radiation therapy.
BACKGROUND Basaloid squamous cell carcinoma (BSCC) is defined by WHO (2005) as an aggressive, highgrade variant of squamous cell carcinoma (SCC), and composed of basaloid as well as squamous components.1 The basaloid variety of SCC is uncommon in the head and neck region, with an incidence of 2%,2 and shows predilection to the upper aerodigestive tract. To the best of our knowledge, the previous literature indicates BSCC of the nose and paranasal sinus as a relatively rare occurrence3 with only 33 cases reported to date. We report a case of a 60-year-old man with maxillary sinus swelling and nasal discharge diagnosed as BSCC, highlighting the clinicopathological and therapeutic aspect of sinonasal tract BSCC.
anteroposteriorly (figure 1). Right-sided infraorbital nerve paraesthesia and deviated nasal septum were evident. On intraoral examination, an ulceroproliferative growth measuring 7×5 cm was seen, extending from the upper gingivobuccal sulcus up to the mid-palatine raphe mediolaterally and from 13 to 17 anteroposteriorly with evident bicortical expansion (figure 2). On palpation, the ulcer exhibited everted and rolled out margins with tenderness of the adjacent mucosa. The patient was systemically healthy with no signs of regional lymphadenopathy.
INVESTIGATIONS CT scan imaging at the level of the zygomatic arch revealed a hypodense mass completely obliterating the right maxillary sinus with expansion of the buccal and palatal cortical plates, eroded lateral nasal wall and deviated nasal septum at a lower level (figure 3). Clinical and radiological features suggested an aggressive lesion suspicious of malignancy. An incisional biopsy was performed, after obtaining consent. Histopathologically, H&E-stained sections showed closely packed basaloid cells, with scanty cytoplasm and hyperchromatic nuclei, arranged in a solid pattern with a lobular configuration (figure 4A). Nuclear palisading along the periphery of the neoplastic nest was evident (figure 4B). Prominent areas of comedo type necrosis within the islands were evident (figure 4C). There was an abrupt association of these basaloid cells with foci of squamous differentiation showing keratin pearl
CASE PRESENTATION
To cite: Pathak J, Patel S, Iyer JS, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014209038
A 60-year-old man reported to the department of oral and maxillofacial surgery with pain and swelling in the right cheek for 6 months and continuous unilateral nasal discharge for 2 months. He had a 15-year history of tobacco consumption using mishri ( powdered burnt tobacco leaves) about 6–7 times a day, and a 10-year history of bidi (a thin cigarette made of tobacco wrapped in a leaf ) smoking about 4–5 times a day. Extraoral clinical examination revealed an ovoid, well defined, palpable, firm swelling of 8×6 cm extending from the right infraorbital rim to the angle of the mouth superoinferiorly and to the right lateral wall of the nose to preauricular region
Figure 1 Extraoral view revealing an ovoid, well-defined swelling of right zygomatic region.
Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
1
Rare disease DIFFERENTIAL DIAGNOSIS Owing to its biological and morphological features, the distinction of BSCC from other entities such as adenoid cystic carcinoma (ACC), small cell undifferentiated neuroendocrine carcinoma (SCUNC), basal cell adenocarcinoma (BCA), salivary duct carcinoma (SDC) and basosquamous carcinoma (BC), should be appropriately made. Histopathologically, ACC features a tubular ductal pattern, lack of squamous differentiation, rare mitoses, necrosis and nuclear pleomorphism, which help differentiate it from BSCC. SCUNC exhibits contrasting features such as small areas of crush artefacts and absence of peripheral palisading. Focal necrosis, squamous differentiation and peripheral palisading are less frequent in BCA. Cells with eosinophilic cytoplasm and multiple, large, irregularly shaped cystic spaces lined by papillary projections of ductal epithelium are features of SDC that are absent in BSCC. In BC, the tumour cells have abundant cytoplasm with marked keratinisation and retraction spaces between the islands, and hyalinised stroma, distinguishing it from BSCC.
TREATMENT
Figure 2 Intraoral view revealing an ulceroproliferative growth involving the right maxillary gingivobuccal sulcus to mid-palatine raphe. formation (figure 4D). Several mitotic figures, including atypical mitosis, were present (figure 4E). The neoplastic cells were separated by a sparse connective tissue stroma. The biopsy specimen of the maxillary lesion was diagnosed as BSCC. The patient was subjected to a full body positron emission tomography scan, which ruled out systemic involvement.
Figure 3 CT scan revealing obliteration of right maxillary sinus with expansion of the buccal and palatal cortical plates and erosion of the lateral nasal wall. 2
The patient underwent right side class III maxillectomy—total right side maxillectomy with reconstruction of orbital floor with titanium mesh and placement of split thickness skin graft (figure 5), followed by adjuvant radiation of 60 Gy. The histopathological features of the excised specimen (figure 6) were in accordance with that of the incisional sample.
OUTCOME AND FOLLOW-UP The patient was, however, lost to follow-up after 6 months (figure 7) of adjuvant radiation therapy.
DISCUSSION BSCC of the upper aerodigestive tract is an aggressive, highgrade tumour with an increased tendency to be deeply invasive, multifocal and metastatic, even at initial presentation. As early as 1927, Quick and Cutler4 mentioned the existence of undifferentiated SCC of the nasopharynx, tonsil and tongue base, but they provided no histopathological details other than that this type of tumour is highly malignant and radiosensitive. They recommended the name “transitional cell epidermoid carcinoma”. Subsequently, the first description of BSCC was given by Wain et al.5 BSCC shows a strong predilection for the upper aerodigestive tract (larynx, hypopharynx and base of tongue) with less frequently occurring sites being the nose, paranasal sinuses, external ear, lungs, anus, vagina and uterine cervix.6 Over 45 cases of BSCC involving the oral cavity have been reported, with involvement of the base of the tongue (61%) and floor of the mouth (30%).4 7 The suggested precursor of BSCC is a totipotent primitive cell located in the basal cell layer of the surface epithelium.1 Sinonasal BSCC usually presents with unilateral nasal obstruction or pain, though the chief complaint in the present case was swelling and nasal discharge. This case report of BSCC in the maxillary sinus is consistent with previously reported cases with regard to age, gender and site predilection.1 5 The features of this case fulfilled the criteria laid down by Wain et al,5 and recently by Barnes et al, to diagnose BSCC7 ▸ Site, gender and age predilection for head and neck region in men in sixth to eighth decades. ▸ Clinically, an ulcerated or exophytic mass with submucosal soft tissue infiltration. Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Rare disease
Figure 4 (A) Solid growth of cells arranged in a lobular configuration with sparse connective tissue stroma. Tumour nests show nuclear palisading along the periphery (arrow-head) and central comedo necrosis (arrows) (H&E, ×100). (B) Nuclear palisading along the periphery of the neoplastic nest (arrow) and atypical mitotic figure (arrow-head) (H&E, ×400). (C) Tumour island showing comedo necrosis (H&E, ×400). (D) Abrupt association of these basaloid cells with foci of squamous differentiation showing keratin pearl formation (H&E, ×400). (E) Basaloid cells with scanty cytoplasm and hyperchromatic nuclei. Cells showing increased mitotic activity (arrows) (H&E, ×400). ▸ Solid basaloid-appearing dysplastic islands with biphasic pattern showing comedo type necrosis and a pseudo glandular pattern.
Figure 5 Right side class III maxillectomy—total maxillectomy with reconstruction of orbital floor with titanium mesh and placement of split thickness graft. Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Figure 6
Excised specimen.
Figure 7 therapy.
Postoperative extraoral view after adjuvant radiation 3
4
Features of reported cases of BSCC of the sinonasal tract
Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Case no
Author
Age
Gender
Symptoms
Location
Tobacco/alcohol
Therapy
Recurrence
Metastases/local invasions
Clinical outcome
Follow-up period
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34
Wan et al12 Wedenberge et al13 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Paulino et al14 Paulino et al14 Lu et al15 Lu et al15 Oikawa et al10 Oikawa et al10 Yu et al11 Yu et al11 Yu et al11 Yu et al11 Vasudev et al16 Lee et al3 Gu et al17 Bhanja et al2 Stanculescu et al18 Ishida et al19 Ishida et al19 Present case
67 55 53 81 69 32 72 33 41 75 64 79 56 46 86 79 50 59 86 36 78 60 59 47 69 48 60 58 56 76 51 85 60 60
M M F F M M M F F F M F M M F M F M M M M M M M M M F F F M M F M M
Epistaxis Cyst-like swelling Epistaxis Obstruction Blurred vision Obstruction Obstruction Obstruction and diplopia Obstruction Obstruction Nasal mass Sinusitis and headache Nasal mass Obstruction and epistaxis Bulky nasal mass Intranasal mass Dyspnoea Epistaxis Epistaxis Epistaxis Cheek swelling, pain Cheek swelling, diplopia Unknown Unknown Unknown Unknown Epistaxis, painful mass Epistaxis, obstruction Decreased vision, headache Painful ulcerative lesion Nasal obstruction Nasal tumour, Exophthalmos Obstruction Pain, swelling, nasal discharge
Nasal cavity Left maxillary tuberosity Left nasal septum Left nasal cavity Left nasal cavity Nasal cavity and night maxillary sinus Nasal cavity and left maxillary sinus Sinuses, bilateral Left nasal cavity Left maxillary sinus Left nasal cavity Left maxillary sinus Right nasal septum Right nasal septum/cavity Left nasal septum/turbinate Right nasal cavity Nose Nose Nasal cavity Nasal cavity Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Right nasal cavity, Paranasal sinus Right inferior turbinate, nasal floor Sphenoid sinus Infraorbital Left nasal cavity, paranasal sinus Maxillary sinus Maxillary sinus Right maxillary sinus
NE Yes/no No/no NE No/no Yes/yes Yes/unknown NE No/no NE No/yes Yes/no Yes/no No/no Yes/no No/no Yes/yes Yes/yes NE NE NE NE NE NE NE NE No/no NE Yes/no NE No/no NE NE Yes/no
S S S, R, C S S S S S, R, C S, R S, R S S S, R S, R for RD Biopsy only S, R C, R S, R NE NE NE NE NE NE NE NE S, R S S, R, C S S, R S, R S, C S, R
None None 2 at 2 and 8 years None 1 within 1 year Many, local, within 1 year 1 within 6 months None Multiple at 2, 3, 4, 5 years 1 at 2 years None 1 within 1 year None 1 within 8 months None None NE NE NE NE NE NE NE NE NE NE NE None None None NE NE 1 within 1.5 years None
None None None None None Brain Brain Bone and lung Lung Dura None Bone and lung Cervical lymph nodes None None None None NE NE None None Orbit, skull base. Lung NE NE NE NE NE None None None NE Orbit, dura Dura, Lung, Liver None
NED NED DDD Alive DDD DDD DDD DDD AWD AWD Dead, NED DDD Alive, NED AWD DDD Alive, NED AWD NED DDD AWD NED DDD DDD DDD DDD Alive NE NED NED NED NE AWD DDD NED
4 months 5 months 8 years 3 years 1 year 7 years 1 year 1 year 5 years 2 years 4 years 1 year 2 years 8 months 6 months 1 month 1 year 1 year 2 years 1.5 years 25 months 6 months 1 year 1 year 2.5 years 3.5 years NE 17 months 6 months 8 months NE 10 months 18 months LTF, 6 months
AWD, alive with disease; BSCC, basaloid squamous cell carcinoma; C, chemotherapy; DDD, dead due to disease; LTF, lost to follow-up; NE, no evidence; NED, no evidence of disease; R, radiotherapy; S, surgery.
Rare disease
Table 1
Rare disease ▸ Presence of abrupt foci of squamous differentiation with or without keratin pearls, and surface mucosal epithelium showing dysplastic features. Sinonasal tract BSCC is uncommon and only limited studies have been reported by authors (as mentioned in table 1). In the previously reported cases, the nasal cavity and paranasal sinus were the most commonly affected sinonasal sites. Nodal metastasis is not uncommon in BSCC. Winzenburg et al8 showed significant differences in the survival of BSCC with and without lymph node metastases, with survival of 18.6 and 47.6 months, respectively, which was seconded by Ereno et al in 2008.9 Regional and distant metastases have been reported as 64% and 44%, respectively, by Oikawa et al,10 and 75% and 35–50%, respectively, of cases in the series by Winzenburg et al,8 with 38% mortality and median survival of 17 months.7 BSCC requires aggressive multimodality therapy, including radical surgical excision, neck dissection, radiotherapy and, often, chemotherapy. The aggressive biological behaviour of BSCC is well documented with 3-year and 5-year overall survival rates of 53% and 32%, respectively.11
Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4 5 6 7
8
9 10
Learning points
11
▸ Basaloid squamous cell carcinoma (BSCC) is an uncommon but distinct malignant tumour considered to be very aggressive, and it often presents as an advanced stage lesion with locoregional and distant metastases. ▸ Sino nasal BSCC, though rare, mimics BSCC of common mucosal sites of the upper aerodigestive tract, requiring equally aggressive intervention with multimodality therapy. ▸ Careful histological differentiation from other tumours with overlapping features holds significance for early accurate diagnosis, treatment planning and prognosis.
12 13 14 15 16 17
18
Competing interests None declared. Patient consent Obtained.
19
Cardesa A, Zidar N, Ereno C. Basaloid squamous cell carcinoma. In: World Health Organization classification of tumours. Lyon, France: IARC Press, 2005:124–5. Bhanja KR, Mallick SK, Sharma S. Cutaneous basaloid squamous cell carcinoma of the face, a rare variant: a case report. Int J Case Rep Image 2012;3:20–3. Lee SJ, Ko IJ, Jun SY, et al. Basaloid squamous cell carcinoma in nasal cavity. Clin Exp Otorhinolaryngol 2009;2:207–10. Ide F, Shimoyama T, Horie N, et al. Basaloid squamous cell carcinoma of the oral cavity: case report and review of 45 cases. Oral Oncol 2002:120–4. Wain SL, Kier R, Volmer RT. Basaloid squamous cell carcinoma of the tongue, hypopharynx and larynx report of 10 cases. Hum Pathol 1986;17:1158–66. Jacqueline AW, Thompson LDR, Wenig BM. Basaloid squamous cell carcinoma of the sinonasal tract. Cancer 1999;85:841–54. Rachel JR, Kumar NS, Jain NK. Basaloid squamous cell carcinoma of retromolar trigone: a case report with review of literature. J Oral Maxillofac Pathol 2011;15:192–6. Winzenburg SN, Niehans GA, George E, et al. Basaloid squamous cell carcinoma: a clinical comparison of two histological types with poorly differentiated squamous cell carcinoma. Otolaryngol Head Neck Surg 1998;119:471–5. Cosme E, Ayman G, Maddi G, et al. Basaloid squamous cell carcinoma of the head and neck. Head Neck Pathol 2008;2:83–91. Oikawa K, Tabuchi K, Nomura M. Basaloid squamous cell carcinoma of the maxillary sinus: a report of two cases of the head and neck. AurisNasus Larynx 2007;34:119–23. Yu GY, Gao Y, Peng X, et al. A clinicopathologic study on basaloid squamous cell carcinoma in the oral and maxillofacial region. Int J Oral Maxillofac Surg 2008;37:1003–8. Wan SK, Chan JKC, Tse KC. Basaloid squamous cell carcinoma of the nasal cavity. J Laryngol Otol 1992;106:370–1. Wedenberge C, Jesslen P, Lundqvist G, et al. Basaloid squamous cell carcinoma of the maxilla. Oral Oncol 1997;33:141–4. Paulino AFG, Singh B, Shah JP, et al. Basaloid squamous cell carcinoma of the head and neck. Laryngoscope 2000;110:1479–82. Lu SY, Eng HL, Huang CC, et al. Basaloid squamous cell carcinoma of the sinonasal tract: report of two cases. Otolaryngol Head Neck Surg 2006;134:883–5. Vasudev P, Tadross OB, Radhi J. Basaloid squamous cell carcinoma: two case reports. Cases J 2009;2:1–4. Gu X, Eskandari F, Fowler M. Sphenoid sinus basaloid squamous cell carcinoma presenting as a sellar mass: report a case with review of the literature. Head and Neck Pathol 2011;5:81–5. Stanculescu L, Vermesan O, Grintescu I, et al. A rare case of basaloid squamous cell carcinoma of the maxilla. Rom J Morphol Embryol 2012;53:1081–5. Ishida M, Okabe H. Basaloid squamous cell carcinoma of the maxillary sinus: report of two cases in association with cathepsin K expression. Oncol Lett 2013;5:1755–9.
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Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
5
CASE REPORT
Basaloid squamous cell carcinoma of the maxilla Jigna Pathak,1 Shilpa Patel,1 Janaki Subramanian Iyer,1 Neeta Mohanty2 1
Department of Oral Pathology, MGM Dental College & Hospital, Navi Mumbai, Maharashtra, India 2 Department of Oral Pathology and Microbiology, Institute of Dental Sciences, Siksha ‘O’ Anusandhan University, Bhubaneswar, Odisha, India, Institute of Dental Sciences, Bhubaneswar, Odisha, India Correspondence to Dr Neeta Mohanty, [email protected] Accepted 14 May 2015
SUMMARY Basaloid squamous cell carcinoma (BSCC) is a distinctive aggressive variant of squamous cell carcinoma. We present a case of a 60-year-old man with tender swelling in the right cheek region for 6 months and continuous unilateral nasal discharge for 2 months. Extraoral examination revealed an ovoid, well-defined swelling from the right infraorbital rim to the angle of the mouth superoinferiorly and the right lateral wall of the nose to preauricular region anteroposteriorly. Intraorally, an ulceroproliferative growth from right upper gingivobuccal sulcus to mid palatine raphe with bicortical expansion was evident. CT revealed a hypodense mass obliterating the right maxillary sinus. Histopathology showed closely packed basaloid cells, with hyperchromatic palisading nuclei, arranged in a solid pattern with a lobular configuration. Prominent areas of comedo necrosis and keratin pearl formation were seen. These features suggested BSCC. The patient underwent surgical excision with adjuvant radiation but was lost to follow-up after 6 months of radiation therapy.
BACKGROUND Basaloid squamous cell carcinoma (BSCC) is defined by WHO (2005) as an aggressive, highgrade variant of squamous cell carcinoma (SCC), and composed of basaloid as well as squamous components.1 The basaloid variety of SCC is uncommon in the head and neck region, with an incidence of 2%,2 and shows predilection to the upper aerodigestive tract. To the best of our knowledge, the previous literature indicates BSCC of the nose and paranasal sinus as a relatively rare occurrence3 with only 33 cases reported to date. We report a case of a 60-year-old man with maxillary sinus swelling and nasal discharge diagnosed as BSCC, highlighting the clinicopathological and therapeutic aspect of sinonasal tract BSCC.
anteroposteriorly (figure 1). Right-sided infraorbital nerve paraesthesia and deviated nasal septum were evident. On intraoral examination, an ulceroproliferative growth measuring 7×5 cm was seen, extending from the upper gingivobuccal sulcus up to the mid-palatine raphe mediolaterally and from 13 to 17 anteroposteriorly with evident bicortical expansion (figure 2). On palpation, the ulcer exhibited everted and rolled out margins with tenderness of the adjacent mucosa. The patient was systemically healthy with no signs of regional lymphadenopathy.
INVESTIGATIONS CT scan imaging at the level of the zygomatic arch revealed a hypodense mass completely obliterating the right maxillary sinus with expansion of the buccal and palatal cortical plates, eroded lateral nasal wall and deviated nasal septum at a lower level (figure 3). Clinical and radiological features suggested an aggressive lesion suspicious of malignancy. An incisional biopsy was performed, after obtaining consent. Histopathologically, H&E-stained sections showed closely packed basaloid cells, with scanty cytoplasm and hyperchromatic nuclei, arranged in a solid pattern with a lobular configuration (figure 4A). Nuclear palisading along the periphery of the neoplastic nest was evident (figure 4B). Prominent areas of comedo type necrosis within the islands were evident (figure 4C). There was an abrupt association of these basaloid cells with foci of squamous differentiation showing keratin pearl
CASE PRESENTATION
To cite: Pathak J, Patel S, Iyer JS, et al. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2014209038
A 60-year-old man reported to the department of oral and maxillofacial surgery with pain and swelling in the right cheek for 6 months and continuous unilateral nasal discharge for 2 months. He had a 15-year history of tobacco consumption using mishri ( powdered burnt tobacco leaves) about 6–7 times a day, and a 10-year history of bidi (a thin cigarette made of tobacco wrapped in a leaf ) smoking about 4–5 times a day. Extraoral clinical examination revealed an ovoid, well defined, palpable, firm swelling of 8×6 cm extending from the right infraorbital rim to the angle of the mouth superoinferiorly and to the right lateral wall of the nose to preauricular region
Figure 1 Extraoral view revealing an ovoid, well-defined swelling of right zygomatic region.
Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
1
Rare disease DIFFERENTIAL DIAGNOSIS Owing to its biological and morphological features, the distinction of BSCC from other entities such as adenoid cystic carcinoma (ACC), small cell undifferentiated neuroendocrine carcinoma (SCUNC), basal cell adenocarcinoma (BCA), salivary duct carcinoma (SDC) and basosquamous carcinoma (BC), should be appropriately made. Histopathologically, ACC features a tubular ductal pattern, lack of squamous differentiation, rare mitoses, necrosis and nuclear pleomorphism, which help differentiate it from BSCC. SCUNC exhibits contrasting features such as small areas of crush artefacts and absence of peripheral palisading. Focal necrosis, squamous differentiation and peripheral palisading are less frequent in BCA. Cells with eosinophilic cytoplasm and multiple, large, irregularly shaped cystic spaces lined by papillary projections of ductal epithelium are features of SDC that are absent in BSCC. In BC, the tumour cells have abundant cytoplasm with marked keratinisation and retraction spaces between the islands, and hyalinised stroma, distinguishing it from BSCC.
TREATMENT
Figure 2 Intraoral view revealing an ulceroproliferative growth involving the right maxillary gingivobuccal sulcus to mid-palatine raphe. formation (figure 4D). Several mitotic figures, including atypical mitosis, were present (figure 4E). The neoplastic cells were separated by a sparse connective tissue stroma. The biopsy specimen of the maxillary lesion was diagnosed as BSCC. The patient was subjected to a full body positron emission tomography scan, which ruled out systemic involvement.
Figure 3 CT scan revealing obliteration of right maxillary sinus with expansion of the buccal and palatal cortical plates and erosion of the lateral nasal wall. 2
The patient underwent right side class III maxillectomy—total right side maxillectomy with reconstruction of orbital floor with titanium mesh and placement of split thickness skin graft (figure 5), followed by adjuvant radiation of 60 Gy. The histopathological features of the excised specimen (figure 6) were in accordance with that of the incisional sample.
OUTCOME AND FOLLOW-UP The patient was, however, lost to follow-up after 6 months (figure 7) of adjuvant radiation therapy.
DISCUSSION BSCC of the upper aerodigestive tract is an aggressive, highgrade tumour with an increased tendency to be deeply invasive, multifocal and metastatic, even at initial presentation. As early as 1927, Quick and Cutler4 mentioned the existence of undifferentiated SCC of the nasopharynx, tonsil and tongue base, but they provided no histopathological details other than that this type of tumour is highly malignant and radiosensitive. They recommended the name “transitional cell epidermoid carcinoma”. Subsequently, the first description of BSCC was given by Wain et al.5 BSCC shows a strong predilection for the upper aerodigestive tract (larynx, hypopharynx and base of tongue) with less frequently occurring sites being the nose, paranasal sinuses, external ear, lungs, anus, vagina and uterine cervix.6 Over 45 cases of BSCC involving the oral cavity have been reported, with involvement of the base of the tongue (61%) and floor of the mouth (30%).4 7 The suggested precursor of BSCC is a totipotent primitive cell located in the basal cell layer of the surface epithelium.1 Sinonasal BSCC usually presents with unilateral nasal obstruction or pain, though the chief complaint in the present case was swelling and nasal discharge. This case report of BSCC in the maxillary sinus is consistent with previously reported cases with regard to age, gender and site predilection.1 5 The features of this case fulfilled the criteria laid down by Wain et al,5 and recently by Barnes et al, to diagnose BSCC7 ▸ Site, gender and age predilection for head and neck region in men in sixth to eighth decades. ▸ Clinically, an ulcerated or exophytic mass with submucosal soft tissue infiltration. Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Rare disease
Figure 4 (A) Solid growth of cells arranged in a lobular configuration with sparse connective tissue stroma. Tumour nests show nuclear palisading along the periphery (arrow-head) and central comedo necrosis (arrows) (H&E, ×100). (B) Nuclear palisading along the periphery of the neoplastic nest (arrow) and atypical mitotic figure (arrow-head) (H&E, ×400). (C) Tumour island showing comedo necrosis (H&E, ×400). (D) Abrupt association of these basaloid cells with foci of squamous differentiation showing keratin pearl formation (H&E, ×400). (E) Basaloid cells with scanty cytoplasm and hyperchromatic nuclei. Cells showing increased mitotic activity (arrows) (H&E, ×400). ▸ Solid basaloid-appearing dysplastic islands with biphasic pattern showing comedo type necrosis and a pseudo glandular pattern.
Figure 5 Right side class III maxillectomy—total maxillectomy with reconstruction of orbital floor with titanium mesh and placement of split thickness graft. Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Figure 6
Excised specimen.
Figure 7 therapy.
Postoperative extraoral view after adjuvant radiation 3
4
Features of reported cases of BSCC of the sinonasal tract
Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
Case no
Author
Age
Gender
Symptoms
Location
Tobacco/alcohol
Therapy
Recurrence
Metastases/local invasions
Clinical outcome
Follow-up period
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34
Wan et al12 Wedenberge et al13 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Jacqueline et al6 Paulino et al14 Paulino et al14 Lu et al15 Lu et al15 Oikawa et al10 Oikawa et al10 Yu et al11 Yu et al11 Yu et al11 Yu et al11 Vasudev et al16 Lee et al3 Gu et al17 Bhanja et al2 Stanculescu et al18 Ishida et al19 Ishida et al19 Present case
67 55 53 81 69 32 72 33 41 75 64 79 56 46 86 79 50 59 86 36 78 60 59 47 69 48 60 58 56 76 51 85 60 60
M M F F M M M F F F M F M M F M F M M M M M M M M M F F F M M F M M
Epistaxis Cyst-like swelling Epistaxis Obstruction Blurred vision Obstruction Obstruction Obstruction and diplopia Obstruction Obstruction Nasal mass Sinusitis and headache Nasal mass Obstruction and epistaxis Bulky nasal mass Intranasal mass Dyspnoea Epistaxis Epistaxis Epistaxis Cheek swelling, pain Cheek swelling, diplopia Unknown Unknown Unknown Unknown Epistaxis, painful mass Epistaxis, obstruction Decreased vision, headache Painful ulcerative lesion Nasal obstruction Nasal tumour, Exophthalmos Obstruction Pain, swelling, nasal discharge
Nasal cavity Left maxillary tuberosity Left nasal septum Left nasal cavity Left nasal cavity Nasal cavity and night maxillary sinus Nasal cavity and left maxillary sinus Sinuses, bilateral Left nasal cavity Left maxillary sinus Left nasal cavity Left maxillary sinus Right nasal septum Right nasal septum/cavity Left nasal septum/turbinate Right nasal cavity Nose Nose Nasal cavity Nasal cavity Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Maxillary sinus Right nasal cavity, Paranasal sinus Right inferior turbinate, nasal floor Sphenoid sinus Infraorbital Left nasal cavity, paranasal sinus Maxillary sinus Maxillary sinus Right maxillary sinus
NE Yes/no No/no NE No/no Yes/yes Yes/unknown NE No/no NE No/yes Yes/no Yes/no No/no Yes/no No/no Yes/yes Yes/yes NE NE NE NE NE NE NE NE No/no NE Yes/no NE No/no NE NE Yes/no
S S S, R, C S S S S S, R, C S, R S, R S S S, R S, R for RD Biopsy only S, R C, R S, R NE NE NE NE NE NE NE NE S, R S S, R, C S S, R S, R S, C S, R
None None 2 at 2 and 8 years None 1 within 1 year Many, local, within 1 year 1 within 6 months None Multiple at 2, 3, 4, 5 years 1 at 2 years None 1 within 1 year None 1 within 8 months None None NE NE NE NE NE NE NE NE NE NE NE None None None NE NE 1 within 1.5 years None
None None None None None Brain Brain Bone and lung Lung Dura None Bone and lung Cervical lymph nodes None None None None NE NE None None Orbit, skull base. Lung NE NE NE NE NE None None None NE Orbit, dura Dura, Lung, Liver None
NED NED DDD Alive DDD DDD DDD DDD AWD AWD Dead, NED DDD Alive, NED AWD DDD Alive, NED AWD NED DDD AWD NED DDD DDD DDD DDD Alive NE NED NED NED NE AWD DDD NED
4 months 5 months 8 years 3 years 1 year 7 years 1 year 1 year 5 years 2 years 4 years 1 year 2 years 8 months 6 months 1 month 1 year 1 year 2 years 1.5 years 25 months 6 months 1 year 1 year 2.5 years 3.5 years NE 17 months 6 months 8 months NE 10 months 18 months LTF, 6 months
AWD, alive with disease; BSCC, basaloid squamous cell carcinoma; C, chemotherapy; DDD, dead due to disease; LTF, lost to follow-up; NE, no evidence; NED, no evidence of disease; R, radiotherapy; S, surgery.
Rare disease
Table 1
Rare disease ▸ Presence of abrupt foci of squamous differentiation with or without keratin pearls, and surface mucosal epithelium showing dysplastic features. Sinonasal tract BSCC is uncommon and only limited studies have been reported by authors (as mentioned in table 1). In the previously reported cases, the nasal cavity and paranasal sinus were the most commonly affected sinonasal sites. Nodal metastasis is not uncommon in BSCC. Winzenburg et al8 showed significant differences in the survival of BSCC with and without lymph node metastases, with survival of 18.6 and 47.6 months, respectively, which was seconded by Ereno et al in 2008.9 Regional and distant metastases have been reported as 64% and 44%, respectively, by Oikawa et al,10 and 75% and 35–50%, respectively, of cases in the series by Winzenburg et al,8 with 38% mortality and median survival of 17 months.7 BSCC requires aggressive multimodality therapy, including radical surgical excision, neck dissection, radiotherapy and, often, chemotherapy. The aggressive biological behaviour of BSCC is well documented with 3-year and 5-year overall survival rates of 53% and 32%, respectively.11
Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2 3 4 5 6 7
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Learning points
11
▸ Basaloid squamous cell carcinoma (BSCC) is an uncommon but distinct malignant tumour considered to be very aggressive, and it often presents as an advanced stage lesion with locoregional and distant metastases. ▸ Sino nasal BSCC, though rare, mimics BSCC of common mucosal sites of the upper aerodigestive tract, requiring equally aggressive intervention with multimodality therapy. ▸ Careful histological differentiation from other tumours with overlapping features holds significance for early accurate diagnosis, treatment planning and prognosis.
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Competing interests None declared. Patient consent Obtained.
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Pathak J, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2014-209038
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