Table of Contents

Original articles

Pattern of Karyotypic aberrations in Pakistani Patients with de novo acute Myeloid leukemia ..........................................................06Syeda Alia Abbas, Sadia Sultan, Sana Ashar, Syed Muhammad Irfan

Tinospora Cordifolia induces Cell Cycle arrest in Human Oral Squamous Cell Carcinoma Cells ...........................................................10Parveen Bansal, Manzoor Ahmad Malik, Satya N Das, Jasbir Kaur

detection Mutations of JaK2 exon 12 in Patients with JaK2 (v617f)-negative Myeloproliferative disorders .....................................15S. Z. Makani, N. Parsamanesh, S. Mirzaahmadi, M. Hashemi, F. Shaveisi-Zadeh, N. Mansouri, M. Ghazi, A. Movafagh

Hepatocellular Carcinoma Peritoneal Metastasis: role of Cytoreductive Surgery and Hyperthermic intraperitoneal Chemotherapy (HiPeC) ......................................................................................................................................................20John Spiliotis, Georgios Nikolaou, Nikolaos Kopanakis, Dimitra Vassiliadou, Alexios Terra, Elias Efstathiou

The effect of dose-volume Parameters on Central nervous System relapse in Pediatric Patients with acute leukemia receiving Prophylactic Cranial irradiation ............................................................................................................24Zeliha Guzeloz, Ayse Nur Demiral, Fatma Eren, Mehmet Adigul, Ahmet Ergin Capar, Handan Cakmakcı, Sebnem Yilmaz, Ozlem Tufekci, Hale Oren, Riza Çetingoz

long-term results of Post-operative Pelvic image guided intensity Modulated radiotherapy in Gynecological Malignancies ........30Rashi Agrawal, Sowmiya Prithiviraj, Dinesh Singh, Vaishali Zamre, Sandeep Agrawal, Arun Kumar Goel, Kanika Gupta, Bala Subramanian

epidemiology and Survival analysis of Gastrointestinal Stromal Tumors in lebanon: real-life Study from a hospital tumor registry, 2000-2015 ....................................................................................................................38Elie El Rassy, Fadi Nasr, Tarek Assi, Toni Ibrahim, Nathalie Rassy, Joseph Bou Jaoude, Marcel Massoud, Georges Chahine

Case reports

Giant Primary Sinonasal Mucosal Melanoma: a rare Malignancy ..........................................................................................................43Mehtab Alam, Mohd Aslam, Piyush Kant Singh, Shahab Farkhund Hashmi, Syed Abrar Hasan

Sarcomatoid Carcinoma of the Maxilla: a Case report with literature review ......................................................................................48Lahcen Khalfi, Yassine Ziani, Mouna Kairouani, Odry Agbessi, Mohammed Kamal Fiqhi, Alae Guerrouani, Karim El Khatib

neuroendocrine Carcinoma of Gall Bladder: a rare Presentation with review of literature .................................................................51Amit Gupta, Parvez Ahmed, Prashant Durgapal, Pooja Kala, Shalinee Rao, Rajesh Pasricha, Sanjeev Misra

Clear Cell variant of Calcifying epithelial Odontogenic Tumor: a rare Clinical entity ............................................................................55Husain Sabir, Subhash Kumbhare, Saurabh Redij, Namrata Gajbhiye

Hodgkin’s lymphoma as a Multiloculated lung Cavity lesion ................................................................................................................61Aisha M. Al-Osail, Hind S. Al-Saif, Mashail M. Al-Hajri, Hajer M. Al-Zuhair, Deemah A. Al-Abdulhadi, Emad M. Al-Osail, Sarah M. Al-Hajri

Breast adenoid Cystic Carcinoma: a rare Case .......................................................................................................................................66Lamiae El Amarti

review articles

industrial Pollutants and nasopharyngeal Cancer: an Open Question ....................................................................................................70Roberto Menicagli, Gianni Bolla, Laura Menicagli, Anastassia Esseiridou

Conference Highlights/Scientific Contributions

• Highlightsofthe1stCombinedGulfCancerConference,CancerAwareness:RealityandAmbition,2-3April2017,Kuwait ........75

• NewsNotes ............................................................................................................................................................................................84

• Advertisements .....................................................................................................................................................................................88

• ScientificeventsintheGCCandtheArabWorldfor2017 ..................................................................................................................89

4848

Case Report

Sarcomatoid Carcinoma of the Maxilla: a Case report with literature review

Lahcen Khalfi¹, Yassine Ziani², Mouna Kairouani³, Odry Agbessi¹, Mohammed Kamal Fiqhi¹, Alae Guerrouani²*, Karim El Khatib¹

¹Department of Oral and Maxillofacial Surgery, Mohammed V Teaching Military Hospital, Rabat, Morocco. ²Department of Oral and Maxillofacial Surgery, Al Farabi Hospital, Oujda, Morocco. ³Department of Medical Oncology, Regional Center of Oncology, Oujda, Morocco

abstract

Sarcomatoid carcinoma is a very rare malignant and aggressive tumor that can involve the maxillary sinus. We report the case of a 46 y/o male who presented a tumor of the right maxillary sinus with extension to the right nasal cavity. The diagnosis of sarcomatoid carcinoma was set by histology and immunohistochemistry. The patient received concomitant cisplatin based chemoradiotherapy

with no response after 3 months of treatment. He died 6 months later.

Through the present case and the review of literature we discuss all aspects of this entity: clinical presentation, differential diagnoses, pathology, treatment and prognosis.

Keywords: Carcinoma, spindle-cell, Maxillary neoplasms

Corresponding author: Alae Guerrouani, MD, Department of Oral and Maxillofacial Surgery – Al

Farabi Hospital, Oujda, Morocco. Email: alaeguerrouani@hotmail.com

introduction:Initially reported by Martin and Stewart in 1935 (1),

sarcomatoid carcinoma is an unusual variant of squamous cell carcinoma (SCC). It comprises a conventional SCC component and an in situ and/or an invasive malignant spindle component with sarcomatous appearance.

Sarcomatoid carcinoma of head and neck is a rare neoplasm. In the oral cavity, it occurs predominantly on the lips, the tongue, and the mandibular alveolus. Few cases of such lesions have been recorded in the maxilla(2).

We report a rare case of maxillary sarcomatoid carcinoma with a review of the literature.

Case reportA 46-year-old male with no medical history presented

to our department 8 months ago with a persistent and progressive right mid-facial swelling. Examination showed a friable tumor of the right nasal cavity. The hard palate was irregular and reddish in areas. The patient had a restricted interincisal opening (15mm), a periorbital edema and hypoesthesia in the right infraorbital nerve area. A malignant tumour was then highly suspected. We performed a multiple biopsy sampling of the mass under general anesthesia.

Histological examination of the mass found an aggressive tumor with a desmoplastic reaction which was

figure 1. axial CT scan showing a large tumor destroying the maxillary bone with an extension to the right infratemporal fossa, the right nasal cavity and the nasopharynx

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G. J. O. Issue 24, 2017

infiltrating the fibrous stroma, the skeletal muscle and the local fat. The sample contained pleomorphic spindle cells with epithelioid appearance in some areas. Some nuclei were vesicular with 1 or 2 prominent nucleoli. Cytoplasm was abundant and granular. There were focal areas of necrosis. Immunohistochemistry showed a strongly positive staining for pan-cytokeratin and a weak staining for vimentin. No positive staining was detected for smooth muscle actin, desmin, S100, HMB45 or Melan-A. The histological conclusion was a sarcomatoid carcinoma.

Computed tomography (CT) (Fig. 1) and magnetic resonance imaging (MRI) (Fig. 2) of the head showed a large mass (6 cm) in the right maxillary sinus. This tumor invaded the skull base, the right orbit, the nasopharynx, the buccal soft tissues, the infratemporal fossa and the oral cavity with massive osteolytic destruction.

CT of the neck and the chest revealed one 2 cm right median jugulo-carotid node and a unique basal nodule in the right lung which measures 65.3 x 41 x 62 cm. Abdominal and pelvic CT were normal.

The patient was treated with palliative platine-based chemotherapy concomitant with radiotherapy. No response was noticed after 3 months. He died 6 months later.

discussionParanasal sinus cancers account for 0.2-0.8%

of all malignant neoplasms. SCC are representing approximately 70% of all cancers developing in paranasal sinuses (3). Sarcomatoid carcinoma (also known as

spindle cell carcinoma, pseudosarcoma, carcinosarcoma, sarcomatoid squamous cell carcinoma, pleomorphic carcinoma and polypoid carcinoma) is a rare highly malignant variant of SCC (1, 4).

Clinically, it looks like a polypoid exophytic growth or an ulcerated mass. It affects elderly men, usually in their sixth or seventh decade (1, 6).

Because of the necrotic tissues that usually cover the surface of sarcomatoid carcinomas, superficial biopsy can lead to misdiagnosis. When sarcomatoid carcinoma is suspected, biopsies should include deep tissues in the base of the tumor (7). The diverse nomenclature of sarcomatoid carcinoma reflects the uncertain histogenesis of the spindle cell components (1). Spindle cells seen in sarcomatoid carcinoma are arranged in a whorled pattern. These cells show elongated nuclei and ropes of collagen. Tumoral cells infiltrate the dermis separately and do not form nests or cords as usually seen in SCC. Signs of cancer aggressiveness are usually found such as deep infiltration of the dermis, hypoderm, and fascia. Sarcomatoid carcinoma is sometimes difficult to differentiate from desmoplastic melanoma or some atypical fibroxanthoma. Immunohistochemistry is therefore usually required. Sarcomatoid carcinoma stains positively for high-molecular weight cytokeratin and epithelial membrane antigen (1, 8, 9). Our case satisfies these criteria.

Sometimes electron microscopy can be useful in addition to immunohistochemistry to confirm the diagnosis, especially in poorly differentiated sarcomatoid carcinomas. Treatment of sarcomatoid carcinomas is among the most challenging because of their high stage presentation at the time of diagnosis, but also because of the lack of therapeutic standards. The prognosis remains ominous. Cisplatin based chemotherapy with concomitant radiotherapy was used in variable radiation doses. This protocol seems not very effective as we noticed in this case.

The effectiveness of surgical removal seems better, though maxillary sarcomatoid carcinomas are difficult to remove because of their usually quick and wide extension to the skull base and the orbits and their propensity for destruction. Thus, prognosis is unfavorable for most patients with a reduced survival rate. Cranial and facial location is considered as a bad prognosis factor. Other predictors of outcome are: the tumor size, the invasion depth and the clinical stage of the disease. Metastatic disease have bad prognosis. When possible, radical surgery results in a good prognosis (6, 9) which is rarely possible in maxillary sarcomatoid carcinomas.

figure 2. Coronal Mri scan showing a large tumor of the maxilla with extension to the right orbit, the skull base and the right nasal cavity

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Sarcomatoid Carcinoma of the Maxilla Lahcen Khalfi, et. al.

ConclusionSarcomatoid carcinoma of the maxillary bone is very

rare and have a bad prognosis. Histological diagnosis may be difficult and usually needs immunohistochemistry to distinguish sarcomatoid carcinoma from other spindle cell tumors. Treatment is not yet well codified and lacks efficiency. Multicentric randomized studies are necessary to increase the efficiency of treatment options.

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and maxillofacial surgery 2012;114:e31-e35

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3. Marcello Roter M Santos et al. Squamous cell carcinoma at maxillary sinus: clinicopathologic data in a single Brazilian institution with review of literature. Int J Clin Exp Pathol 2014;7(12):8823-8832

4. R. J. Middlehurst, C. W. Blackburn, P. Sloan. Spindle-cell carcinoma: a case report. Britich Journal of Oral and Maxillofacial Surgery 1990;28:114-116.

5. Batsakis JG, Rice DH, Howard DR. The pathology of head and neck tumours: spindle cell lesions (sarcomatoid carcinomas, nodular fasciitis and fibrosarcoma) of the aerodigestive tracts, part 14. Head Neck Surg 1982;4:499-513.

6. Guarino M, Tricomi P, Giordano F, Cristofori E. Sarcomatoid carcinomas: pathological and histopathogenic considerations. Pathology 1996;28:298-305.

7. Munakata R, Cheng J, Nakajima T, Saku T. Case report spindle cell carcinoma of the gingival: report of an autopsy case. J Oral Pathol Med 1998;27:180-4.

8. Gupta R, Singh S, Hedau S, Nigam S, Das BC, Singh I, et al. Spindle cell carcinoma of the head and neck: an immunohistochemical and molecular approach to its pathogenesis. J Clin Pathol 2007;60:472-5

9. Takata T, Ito H, Ogawa I, Miyauchi M, Ijuhin N, Nikai H. Spindle cell squamous carcinoma of the oral region an immunohistochemical and ultrastructural study on the histogenesis and differential diagnosis with a clinicopathological analysis of six cases. Vichows Arch A Pathol Anat 1991;419:177-182.