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Year : 2016  |  Volume : 2  |  Issue : 3  |  Page : 152-154

Second primary malignancies in head and neck region: Report on two cases

1 Department of Otorhinolaryngology and Head and Neck Surgery, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India
2 Department of Pathology, Maulana Azad Medical College and Associated Lok Nayak Hospital, New Delhi, India

Date of Web Publication7-Oct-2016

Correspondence Address:
Dr. Divya Gupta
66, Raj Nagar, Pitam Pura, New Delhi - 110 034
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2394-7438.191684

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Incidence of second primary malignancies is on the rise, especially in head and neck region because of increased longevity of the patients and availability of better diagnostic tools. We illustrate two such cases along with their treatment modalities. One of the patients developed malignant fibrous histiocytoma of maxilla as second malignancy which in itself is a very rare malignancy with only six cases reported in the literature so far.

Keywords: Head and neck cancer, malignant fibrous histiocytoma, metachronous malignancies, multiple malignancies, second primary malignancy, synchronous malignancies

How to cite this article:
Gupta D, Singh I, Mahajan N. Second primary malignancies in head and neck region: Report on two cases. MAMC J Med Sci 2016;2:152-4

How to cite this URL:
Gupta D, Singh I, Mahajan N. Second primary malignancies in head and neck region: Report on two cases. MAMC J Med Sci [serial online] 2016 [cited 2020 Sep 19];2:152-4. Available from: http://www.mamcjms.in/text.asp?2016/2/3/152/191684

  Introduction Top

With the advent of newer and multimodal treatment techniques to combat cancer, patients are living longer with comorbidities, metastatic disease, and development of a second primary tumor. Timely detection of recurrence as well as synchronous or metachronous second primary through serial follow-ups is of paramount importance in patients who have been treated for head and neck cancer. We present here two cases of second primary malignancies, one of them with a very rare malignant fibrous histiocytoma (MFH) of maxilla and discuss the problems, importance of follow-ups, and management of such second malignancies.

  Case Reports Top

Case 1

A 56-year-old male, smoker for 20 years, presented to ear, nose, and throat (ENT) clinic, complaining of intermittent bleeding from right nasal cavity for 2 months. Ten years earlier, the patient was diagnosed with carcinoma of right tonsil for which concurrent chemotherapy and radiotherapy were given for 2 months, and the patient was declared free from the disease, confirmed by a positron emission tomography scan performed 8 months later.

Intranasal examination revealed irregular bleeding mass in the right lateral wall. Biopsy showed markedly cellular tumor composed of highly pleomorphic mitotic spindle-shaped cells suggesting MFH. Computed tomography revealed soft tissue density filling the right maxillary sinus with erosion of posterolateral wall and invasion of the infratemporal fossa. The tumor was surgically excised by right total maxillectomy followed by radiotherapy. Two years later, the patient is disease free and leading a normal life with regular follow-up [Figure 1].
Figure 1: (a) Preoperative computed tomography showing opacification of right maxillary cavity with erosion of right hard palate; (b) post-right maxillectomy computed tomography; (c) photomicrograph showing a markedly cellular tumor composed of highly pleomorphic spindle-shaped cells with irregular-shaped nucleus, opened up chromatin, and prominent nucleolus suggestive of malignant fibrous histiocytoma (H and E, ×100); (d) high power view of the same showing a mitotic figure, marked by arrow (H and E, ×400).

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Case 2

A 55-year-old male, smoker for 25 years, presented to ENT outpatient department with a complaint of swelling of the left hard palate. Two years earlier, he had developed carcinoma left mandible with adjacent skin involvement for which excision of primary with segmental mandibulectomy and modified neck dissection was performed. Reconstruction was performed with titanium plating of mandible and forehead and pectoralis major flaps. This was followed by adjuvant radiotherapy [Figure 2].
Figure 2: (a) Oral cavity of the patient showing ulceroproliferative growth in left hard palate; (b) clinical photograph of patient showing reconstructed lower jaw from previous surgery

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Intraoral examination revealed ulceroproliferative lesion in the upper left palate distal to premolars. Biopsy revealed squamous cell carcinoma. The tumor was excised with partial left maxillectomy, and the patient was sent for adjuvant radiotherapy.

  Discussion Top

The incidence of detection of multiple primary malignancies has increased significantly because of better rate of survival of cancer patients due to the availability of advanced diagnostic and management techniques of the tumors. When detected within 6 months of the index tumor, the malignancy is termed as synchronous primary malignancy (SPM) and if identified after this period, it is called metachronous primary malignancy. [1] Bilroth was the first to report multiple primary malignancies, after which Warren and Gates established the criteria for the same which are in use even today. Such histologically malignant tumors occur at different locations, separated anatomically by normal mucosa, and the subsequent tumor is not a result of metastasis of the first one. [2]

The incidence of second primary malignancy in patients with head and neck cancer is about 3-7%. [3] The precise incidence varies with different case series and may also be related to period of follow-up. The menace of second malignancies is far more prevalent as can be expected entirely by chance in head and neck cancer as per the various retrospective series. Various factors have been implicated in their causation such as genetic mutations, immunosuppression, radiotherapy, tobacco chewing, and alcohol. According to a commonly accepted theory, widespread mucosal damage of aerodigestive tract as a result of damage by exogenous carcinogenic agents such as alcohol and tobacco, a phenomenon known as "field cancerization" or "condemned mucosa syndrome" is the principle cause of SPMs. [4]

Gan et al. in their study of 2230 patients with squamous cell carcinoma of head and neck found a lower 3-year SPM rate (5.2%) in primary oropharyngeal carcinoma cases as compared to patients with nonoropharyngeal malignancies (8.3%). Patients with index oropharyngeal cancer most commonly were found to have SPM in nontobacco-related sites, whereas the most common locations of SPMs in nonoropharyngeal cancer were tobacco-related sites including lungs, esophagus, and bladder. Out of 236 SPMs reported in 2230 patients, only one had SPM in sinus cavity. [5] Both our cases had ipsilateral maxillary sinus SPM detected after 10 and 2 years of index cases, respectively.

MFH, also known as undifferentiated sarcoma is an uncommon jawbone tumor, which has been reported previously in a meager number of fourteen patients (six in maxilla) in the literature following radiotherapy for primary cancer. [6] This SPM can also be categorized as radiation-induced sarcoma as per the case selection criteria by Cahan et al. [7] These radiation-induced sarcomas are more aggressive in nature; hence, they tend to have poorer outcomes when compared to stage-matched soft tissue sarcomas, independent of irradiation.

It has been acknowledged that the incidence of SPMs is directly linked to the extent of evaluation, length of follow-up, curability of primary tumor, and the patterns of alcohol and tobacco use. Therefore, it becomes imperative that the problem is focused and addressed from several angles. After definitive treatment of primary tumor, routine screening is must for accessible sites such as hypopharynx that must be supplemented with annual chest radiograph. In addition, panendoscopy must be considered to evaluate inaccessible sites such as esophagus and lungs which are common sites for nonoropharyngeal SPMs. Second, the role of chemoprevention and strong smoking cessation programs needs exploration.

Despite having realized the importance of vigorous follow-ups in patients with head and neck cancer to allow timely detection of a recurrence or SPM, enormous workload in the hospitals often keeps playing a spoiler. There has been a striking discrepancy in the strategies involved in the work-up and follow-up of these patients and a few have questioned the effectiveness of the same. Boysen et al. [8] calculated the "recurrence pick-up rate" and subsequent "cure rate" per number of follow-up consultations carried out and found that the "recurrence cure rate" to be 1:250 consultations in patients initially treated with combined irradiation and major surgery.

Newer imaging modalities with radiotracers and optical diagnostics along with detailed clinical and endoscopic examination have made it possible to identify a tumor accurately. Genetic classification and the persistence of habits such as smoking or alcohol can further stratify the patients in high- and low-risk groups. It can be conceded easily that SPMs are on their way to increase because of improvement in diagnostic techniques, supportive care measures, and better management of primaries, and despite their being huge medical advancement, our current destructive local and systemic anticancer approaches do not revolutionize the cancerization process. Continuous surveillance and an effective chemopreventive regimen would be the vital keys in combating SPMs.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Suzuki T, Takahashi H, Yao K, Inagi K, Nakayama M, Makoshi T, et al. Multiple primary malignancies in the head and neck: A clinical review of 121 patients. Acta Otolaryngol Suppl 2002;547:88-92.  Back to cited text no. 1
Warren S, Gates O. Multiple primary malignant tumors: A survey of the literature and a statistical study. Am J Cancer 1932;15:1348-414.  Back to cited text no. 2
Atienza JA, Dasanu CA. Incidence of second primary malignancies in patients with treated head and neck cancer: A comprehensive review of literature. Curr Med Res Opin 2012;28:1899-909.  Back to cited text no. 3
Lippman SM, Hong WK. Second malignant tumors in head and neck squamous cell carcinoma: The overshadowing threat for patients with early-stage disease. Int J Radiat Oncol Biol Phys 1989;17:691-4.  Back to cited text no. 4
Gan SJ, Dahlstrom KR, Peck BW, Caywood W, Li G, Wei Q, et al. Incidence and pattern of second primary malignancies in patients with index oropharyngeal cancers versus index nonoropharyngeal head and neck cancers. Cancer 2013;119:2593-601.  Back to cited text no. 5
Koyama T, Kobayashi T, Maruyama S, Abé T, Swelam WM, Kodama Y, et al. Radiation-induced undifferentiated high-grade pleomorphic sarcoma (malignant fibrous histiocytoma) of the mandible: Report of a case arising in the background of long-standing osteomyelitis with a review of the literature. Pathol Res Pract 2014;210:1123-9.  Back to cited text no. 6
Cahan WG, Woodard HQ, Higinbotham NL, Stewart FW, Coley BL. Sarcoma arising in irradiated bone: Report of eleven cases 1948. Cancer 1998;82:8-34.  Back to cited text no. 7
Boysen M, Lövdal O, Tausjö J, Winther F. The value of follow-up in patients treated for squamous cell carcinoma of the head and neck. Eur J Cancer 1992;28:426-30.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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