Mucosal melanoma of the head-and-neck

Mucosal melanoma of the head-and-neck region: A single institutional clinical experience

Mathumitha Ramesh, Siddhartha Nanda, Biplab Misra
Department of Radiotherapy, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India

Date of Web Publication

14-Jan-2019

Correspondence Address:
Dr. Siddhartha Nanda
Department of Radiotherapy, All India Institute of Medical Sciences, Raipur, Chhattisgarh 
India

Source of Support: None, Conflict of Interest: None

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DOI: 10.4103/sajc.sajc_326_18

 

How to cite this article: Ramesh M, Nanda S, Misra B. Mucosal melanoma of the head-and-neck region: A single institutional clinical experience. South Asian J Cancer 2019;8:26-40

How to cite this URL: Ramesh M, Nanda S, Misra B. Mucosal melanoma of the head-and-neck region: A single institutional clinical experience. South Asian J Cancer [serial online] 2019 [cited 2019 Jan 17];8:26-40. Available from: http://journal.sajc.org/text.asp?2019/8/1/26/250091

Dear Editor,

Due to the rarity of mucosal melanoma, the scientific knowledge is limited compared to its cutaneous counterpart. Weber reported the first case of mucosal melanoma of the head-and-neck region in 1856.^[1] The largest case series of mucosal melanoma of the head-and-neck region was reported by Bachar et al.^[2] with 61 patients over 41 years and in India, Gupta et al.^[3] reported 42 patients over the period of 8 years. This is one such attempt with four cases over 1 year.

A 26-year-old female patient presented with the complaints of epistaxis and nasal obstruction. Computed tomography-scan (CT) revealed a large expansile soft-tissue mass in the right maxillary sinus extending into the nasal cavity, right upper alveolar arch, right orbit, and right buccal space. Biopsy and immunohistochemistry confirmed the diagnosis of malignant melanoma [Figure 1] and [Figure 2].

Figure 1: (a) Stratified squamous epithelium lined tissue with underlying diffuse sheets of epithelioid melanocytes along with brownish-black pigment (H and E). (b) Individual tumour cells showing clear cytoplasm and pleomorphic vesicular nuclei with prominent nucleoli (c) HMB 45 expression. (d) Melan-A positivity Click here to view

Figure 2: Computed tomographyscan reconstructed saggital view Click here to view

The patient underwent right total maxillectomy with right orbital exenteration and the bony margin was involved by tumor. Adjuvant radiotherapy of 60 Gy in 6 weeks was delivered. Unfortunately, 4 months after the completion of treatment, the patient developed recurrent right submandibular lymphnode, bilateral lung metastasis along with nodular lesions in retroperitoneal and right infrarenal region. The patient was started on palliative chemotherapy (paclitaxel + carboplatin). After three cycles of chemotherapy, contrast-enhanced CT scan (CECT) showed all the retroperitoneal and infrarenal lesions had regressed completely, whereas there was a partial reduction in the right submandibular lymphnode and lung metastasis. In view of the good response, the patient was given three more cycles of the same chemotherapy regimen. After 1 month, the follow-up CECT scan showed residual right submandibular lymph node and persisting bilateral lung metastasis. The patient was put on Tablet Sorafenib. Due to toxicity, she was shifted to an alternative chemotherapy regimen (dacarbazine + cisplatin). After three cycles, positron-emission tomography (PET-CT scan) showed the persistence of the previous disease and an appearance of new metastatic involving right iliac bone for which the patient received palliative radiotherapy. At present, the patient is put on tablet Imatinib and has a stable disease condition until the last follow-up.

A 43-year-old male presented with complaint of blackish mass in the right buccal mucosa. The biopsy from this lesion confirmed melanocarcinoma. He underwent inferior partial maxillectomy, and the postoperative histopathology showed malignant melanoma. All margins were uninvolved by tumor with no perineural invasion or lymphovascular invasion [Figure 3].

Figure 3: Stratified squamous epithelium lined tissue with underlying sheets of epithelioid melanocytes arranged in an organoid pattern and fascicles of spindled melanocytes along with brownish-black pigment. Inset-epithelioid melanocytes showing indistinct cytoplasmic borders and pleomorphic vesicular nuclei with prominent nucleoli Click here to view

The patient received immunotherapy as adjuvant treatment. However, after 3 months of disease-free interval, the patient had regional reccurrence in right level II lymphnode confirmed by fine-needle aspiration cytology (FNAC) and PET-CT scan [Figure 4]. The patient underwent bilateral modified radical neck dissection.

Figure 4: Computed tomographyscan and positron emission tomography scan showing right level II lymphnode involvemet Click here to view

Following which the patient received radiotherapy of 60 Gy in 6 weeks. The patient had no evidence of disease until last follow-up.

A 62-year-old male presented with complaints of Left nasal blockage, protruding Left nasal mass covering the whole of the Left nasal cavity and swelling in the Left side of the hard palate. The CECT-scan showed a locally advanced lesion in the Left nasal cavity, Left maxillary cavity which was extending to the oral cavity along with liver and lung metastasis. The biopsy and immunohistochemical of nasal mass confirmed the diagnosis of malignant mucosal melanoma [Figure 5] and [Figure 6].

Figure 5: (a) Diffuse positivity for HMB 45. (b) Diffuse positivity for Melan A. (c) Diffuse positivity for S 100. (d) Vimentin expression by tumour cells. (e) Negative staining for CK 20 and (f) negative staining for synaptophysin Click here to view

Figure 6 : Computed tomography-scan (axial and reconstructed saggital view) showing a large sino-nasal mass Click here to view

The patient was treated with palliative chemotherapy (dacarbazine, cisplatin, and vinblastine X six cycles). Later, he received palliative radiotherapy for epistaxis. At the last follow-up, the patient had no bleeding from the local site and had a stable disease.

The patient was treated with palliative chemotherapy (dacarbazine, cisplatin, and vinblastine X six cycles). Later, he received palliative radiotherapy for epistaxis. At the last follow-up, patient had no bleeding from the local site and had stable disease.

A 68-year-old female presented with complaints of bulging of the left eye with occasional bleeding from the left eye. CT-scan showed an intraorbital mass on the posterolateral wall of the left orbit. She underwent left orbital exenteration, and the postoperative histopathology confirmed the diagnosis of malignant melanoma of the eye. Neck dissection was planned, but the patient was lost to follow-up [Table 1].

Table 1: Summarised Observertaions Click here to view

In western studies,^[2],[4] the mean age was 65–70 years. In an Indian study,^[3] mean age was 53 years which was similar to our series (49.7 years). The majority of mucosal melanoma arises in the sinonasal and oral cavity^[2],[3] which correlates with our study. According to Szabo,^[5] this is mainly due to the high density of melanocytes in these regions.

According to many studies,^[2],[6],[7] the female patients were relatively younger and had a better prognosis. The female patient in our series is the youngest. Nasal obstruction, pain, and epistaxis were the most common symptoms in our sinonasal melanoma patients which is similar to a study by Meleti et al.^[6]

According to Bakkal et al.,^[8] the local, regional, and systemic recurrences were 20%, 50%, and 80%, respectively, and all patients with metastasis had lung involvement. Two of our patients who had metastatic disease had lung involvement, and one had developed regional recurrence. As surgery is the primary modality of treatment,^[9] three of our patients who had localized disease underwent surgery.

Mendenhall et al.^[9] stated that the radiation could reduce local recurrence but has no survival benefit. One of our patients who underwent adjuvant radiotherapy had developed distant metastasis after 3 months of disease-free survival (DFS). Another patient who received immunotherapy as adjuvant treatment developed regional recurrence after 3 months of DFS.

As noted by various studies,^[6],[10] our experience with these patients showed that the outcome was not affected by treatment modality chosen. With the use of hypofractionation schedules and newer treatment delivery techniques, radiotherapy presently improves loco-regional control^[7],[11] in malignant melanoma which was once thought to be radio-resistant.^[12] However, larger case series and longer follow-up are required to bring further light to this topic.

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.

Acknowledgment

We would like to show our gratitude to our departmental staff and collegues, especially Dr. Nighat Hussain, Additional Professor and Dr. Vandita Singh, Assistant Professor from the Department of Pathology, All India Institute of Medical Sciences, Raipur, for their assistance in preparation in the manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 

References

1.	Weber CO. Surgical Experience and Research, in Addition to Interesting Observations from the Surgical Clinic and the Protestant Hospital Bonn. Berlin, Germany: Reimer G; 1859. p. 304-5.
2.	Bachar G, Loh KS, O'Sullivan B, Goldstein D, Wood S, Brown D, et al. Mucosal melanomas of the head and neck: Experience of the princess Margaret hospital. Head Neck 2008;30:1325-31.
3.	Gupta T, Agarwal J, Singh S, Ghosh-Laskar S, Chaturvedi P, Kane S, et al. Mucosal melanoma of the head and neck: Tata memorial hospital experience. Int J Head Neck Surg 2010;1(3):141-5.
4.	Manolidis S, Donald PJ. Malignant mucosal melanoma of the head and neck: Review of the literature and report of 14 patients. Cancer 1997;80:1373-86.
5.	Szabo G. The number of melanocytes in human epidermis. Br Med J 1954;1:1016-7.
6.	Meleti M, Leemans CR, de Bree R, Vescovi P, Sesenna E, van der Waal I, et al. Head and neck mucosal melanoma: Experience with 42 patients, with emphasis on the role of postoperative radiotherapy. Head Neck 2008;30:1543-51.
7.	Wada H, Nemoto K, Ogawa Y, Hareyama M, Yoshida H, Takamura A, et al. A multi-institutional retrospective analysis of external radiotherapy for mucosal melanoma of the head and neck in Northern Japan. Int J Radiat Oncol Biol Phys 2004;59:495-500.
8.	Bakkal FK, Başman A, Kızıl Y, Ekinci Ö, Gümüşok M, Ekrem Zorlu M, et al. Mucosal melanoma of the head and neck: Recurrence characteristics and survival outcomes. Oral Surg Oral Med Oral Pathol Oral Radiol 2015;120:575-80.
9.	Mendenhall WM, Amdur RJ, Hinerman RW, Werning JW, Villaret DB, Mendenhall NP, et al. Head and neck mucosal melanoma. Am J Clin Oncol 2005;28:626-30.
10.	Gaze MN, Kerr GR, Smyth JF. Mucosal melanomas of the head and neck: The Scottish experience. The Scottish melanoma group. Clin Oncol (R Coll Radiol) 1990;2:277-83.
11.	Ballo MT, Ang KK. Radiation therapy for malignant melanoma. Surg Clin North Am 2003;83:323-42.
12.	Paterson R. Classification of tumors in relation to radiosensitivity. Br J Radiol 1933;6:218-33.

Solitary fibrous tumour of pleura (SFTP)

Giant solitary fibrous tumor: A rare case report

Sanjay Sharma¹, Prriya Eshpuniyani¹, Kirti Bhushan¹, Kanishka Gautam Siddharth¹, Sameer Pathan^2
1 Department of Surgical Oncology, Asian Cancer Institute, Sion, Mumbai, Maharastra, India
² Department of Pathology, Asian Cancer Institute, Sion, Mumbai, Maharastra, India

Date of Web Publication

14-Jan-2019

Correspondence Address:

Dr. Prriya Eshpuniyani
Department of Surgical Oncology, Asian Cancer Institute, Sion, Mumbai, Maharastra 
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/sajc.sajc_204_18

 

How to cite this article: Sharma S, Eshpuniyani P, Bhushan K, Siddharth KG, Pathan S. Giant solitary fibrous tumor: A rare case report. South Asian J Cancer 2019;8:17-26

How to cite this URL: Sharma S, Eshpuniyani P, Bhushan K, Siddharth KG, Pathan S. Giant solitary fibrous tumor: A rare case report. South Asian J Cancer [serial online] 2019 [cited 2019 Jan 17];8:17-26. Available from: http://journal.sajc.org/text.asp?2019/8/1/17/250086

Dear Editor,

Solitary fibrous tumour of pleura (SFTP) is a very rare benign spindle cell mesenchymal tumour, first described in 1931.^[1] Most commonly originates from pleura and represents 5% of tumors of pleura.^{[1],[2],[3],[4]} However, 10%–20% of these tumors are locally aggressive or malignant.^[2],[3] Complete en bloc surgical resection with free resection margins is the treatment of both benign and malignant types of SFTP.^{[1],[2],[3],[5]}

A 56-year-old man, nonsmoker with no comorbidities presented with a cough for 5 months and left-sided heaviness and dyspnea for 1 month. He had no other positive history, and his general examination was normal. Examination of lungs revealed decreased breath sounds throughout the left chest. Chest X-ray [Figure 1] showed a large, left hemithorax mass with no visible lung parenchyma and shift of mediastinum to the right. Contrast-enhanced computed tomography (CECT) scan of the thorax [Figure 2] confirmed the presence of a large lobulated heterogeneous pleural-based mass measuring approximately 20 cm × 20 cm × 10 cm within left pleural space with gross pleural effusion resulting in complete collapse of left lung toward the hilum. Laboratory investigations were within normal limits other than low hemoglobin of 10 g/dl. His pulmonary function test showed poor forced expiratory volume 1 values (39%) and forced vital capacity (34%). CT-guided tru-cut biopsy of the mass revealed spindle cell neoplasm with low mitotic index and mild cytologic atypia. Immunohistochemistry (IHC) showed positivity for Vimentin, BCL2, CD99, and MIB (16%) confirming low-grade spindle cell sarcoma pleuropulmonary origin.

Figure 1: Large mass left hemithorax with complete collapse of left lung and mediastinal shift to the right Click here to view

Figure 2: Contrast enhanced computed tomography thorax: Large lobulated mass in left hemithorax with pleural effusion and mediastinal shift to right Click here to view

The patient underwent left posterolateral thoracotomy with vertical extension of the incision transecting three ribs. En mass excision of the tumor (measuring 32 cm × 26 cm, weighing 3.8 kg) was done with a small sliver of the lung [Figure 3]. Lung was found to be normal though collapsed intraoperatively hence was conserved. Post-operative recovery was uneventful other than delayed removal of the intercostal drainage tube to allow complete expansion of lung. The patient was discharged on the 10^th postoperative day. Chest X-ray at discharge showed a fairly expanded left lung with shift of mediastinum to normal position [Figure 4].

Figure 3: En Toto excision of mass with sliver of lung tissue (32 cm × 26 cm) weighing 3.8 kg Click here to view

Figure 4: Postoperative day 7 Chest X-ray showing fairly expanded lung with mediastinum in normal position Click here to view

First described in 1931 by Klemperer and Rabin,^[1] SFTP is a very rare benign spindle cell mesenchymal tumor most commonly originating from the pleura and it represents 5% of the tumors of the pleura.^{[1],[2],[3],[4]}

The peak incidence of diagnosis of SFTPs is the fifth and sixth decades of life with equal sex predilection.^[1],[2],[4]

Our patient was a 56-year-old man with no comorbidities.

SFTPs are usually asymptomatic, diagnosed incidentally and clinical course of the disease is unpredictable.^[1],[2],[4] However, patient can present with various respiratory symptoms such as a cough, chest pain, and dyspnea.^[2],[4] This tumor is usually benign, but up to 20% of cases can be malignant.^[1] Patients with benign tumors are symptomatic in 54%–67% of the cases, whereas malignant tumors are symptomatic in around 75% of cases.^[4]

Our patient had symptoms of a cough and breathlessness since few months.

Preoperative diagnosis of the tumor is a difficult challenge. Chest X-ray is the first modality of the investigation. However, CECT of the thorax is considered to be the most important examination.^[1],[2],[4] CT scans usually demonstrate a well-defined and occasionally lobulated mass with soft tissue attenuation appearing on the pleural surface, and displacement of the surrounding structures.^[4] On CT, SFTP can be detected to arise from the parietal pleura, lung fissure or visceral pleura. As per literature size of SFTPs range from a few millimeters to tens of centimeters (0.8–26 cm).^[4] Signs which may lead to the suspicion of a tumor malignancy can be the existence of clinical symptoms, mean tumor diameter >10 cm, fibrous adherences, pleural effusion and positive histology for Ki67 10% or greater.^[2]

Image-guided tru-cut biopsy is useful for the preoperative diagnosis.^[2],[4],[5] IHC plays a key role regarding the distinction of SFTP from mesotheliomas and sarcomas.^[2] Both benign and malignant varieties of SFTP are CD34, CD99, Vimentin, and BCL-2-positive.^{[1],[2],[3],[4]}

CECT Scan of our patient showed the left hemithorax completely occupied by tumor (20 cm × 20 cm × 10 cm) with the complete collapse of the left lung and mediastinal shift to the right. Tru-cut biopsy showed it to be spindle cell neoplasm and IHC showed positivity for Vimentin, bcl-2, CD99, and MIB (16%) confirming low-grade spindle cell sarcoma of pleuropulmonary origin.

Majority of SFTs of the thorax are benign, whereas 10%–20% are locally aggressive or malignant.^[2],[3] Complete en bloc surgical resection with free resection margins is the treatment of both benign and malignant types of SFTP.^{[1],[2],[3],[5]} As these tumors are not primary lung tumors, lung parenchyma resection should be kept as minimal as possible with clear margins for oncological safety. A lobectomy or pneumonectomy could be carried out in larger tumors or in intraparenchymal tumors. If the tumor arises from the parietal pleura, a thoracic wall resection could be considered for complete curative resection.^[2]

Our patient underwent in toto excision of a large parietal pleural based tumor weighing around 3.8 kg and measuring 32 cm × 26 cm with a small sliver of the lung.

Definitive diagnosis of SFTP is histologically made after the surgical resection of the tumor. Risk of recurrence after complete surgical resection can range from <2% in benign SFTP to 63% in malignant forms.^[5]

Final histopathology and IHC in our report confirmed it to be solitary fibrous tumor.

Adjuvant chemotherapy is usually not a part of the management of SFTP. However, it could be used in incompletely resected tumors, malignant sessile SFTPs or in cases of chest wall invasion and concurrent pleural effusion.^[2] Treatment for recurrence after surgical treatment is resurgery.^[2]

Metastases from SFTP have been observed to bones, brain, lungs, and intra-abdominal lymph nodes.^[2],[4] Malignant and larger SFTP are more likely to develop metastases mandating a long-term follow-up in these patients.

We report here a rare giant SFTP with complete curative excision of the tumor and complete reexpansion of the lung alleviating the patient's symptoms completely. Patient has been asymptomatic at the end of 6 months follow-up.

To the best of our knowledge, an SFTP of these dimensions and weight has not been reported in the literature.

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

Nil.

Conflicts of interest

There are no conflicts of interest.

 

References

1.	Supakul R, Sodhi A, Tamashiro CY, Azmi SS, Kadaria D. Solitary fibrous tumor of the pleura: A Rare cause of pleural mass. Am J Case Rep 2015;16:854-7.
2.	Hohenforst-Schmidt W, Grapatsas K, Dahm M, Zarogoulidis P, Leivaditis V, Kotoulas C, et al. Solitary fibrous tumor: A center's experience and an overview of the symptomatology, the diagnostic and therapeutic procedures of this rare tumor. Respir Med Case Rep 2017;21:99-104.
3.	Czimbalmos C, Csecs I, Polos M, Bartha E, Szucs N, Toth A, et al. Uncommon presentation of a rare tumour – Incidental finding in an asymptomatic patient: Case report and comprehensive review of the literature on intrapericardial solitary fibrous tumours. BMC Cancer 2017;17:612.
4.	Vejvodova S, Spidlen V, Mukensnabl P, Krakorova G, Molacek J, Vodicka J, et al. Solitary fibrous tumor – Less common neoplasms of the pleural cavity. Ann Thorac Cardiovasc Surg 2017;23:12-8.
5.	Tan JH, Hsu AA. Challenges in diagnosis and management of giant solitary fibrous tumour of pleura: A case report. BMC Pulm Med 2016;16:114.