A challenging diagnosis of dermatofibrosarcoma protuberans of the middle finger in an infant: A case report.

INTRODUCTION: Dermatofibrosarcoma protuberans (DFSP) is a rare sarcoma, accounting for less than 0.1 % of tumors. While it predominantly occurs in adults, pediatric cases are unusual. This case report aims to highlight the diagnostic and therapeutic challenges posed by DFSP in infants due to its rarity and slow-growing nature, emphasizing the importance of early diagnosis and prompt intervention. CASE PRESENTATION: We report the case of an 8-month-old infant presenting with a progressive finger mass, initially mistakenly diagnosed as a dermatofibroma. Local excision was done, but the tumor recurred after one year. Subsequent re-excision and skin grafting were performed, and histopathology confirmed DFSP. Despite middle finger amputation three weeks later, a new mass emerged on the adjacent ring finger after one year. This tested negative for DFSP. The fibrous mass has persisted for five years without significant changes. CLINICAL DISCUSSION: DFSP is a rare sarcoma with a higher prevalence in adults. It typically presents as a painless, slow-growing mass and is usually diagnosed by biopsy and immunohistochemistry. Surgical excision with negative margins is the preferred treatment. The rarity and slow-growing nature of DFSP pose challenges in diagnosis and treatment. CONCLUSION: Early diagnosis and prompt surgical intervention are crucial in managing DFSP, especially given its high recurrence potential. Maintaining a high index of suspicion is essential even in very young children. Aggressive resection with negative margins and diligent post-operative surveillance are key strategies to mitigate metastasis risk and improve prognosis in such challenging cases.

Ewing's Sarcoma of the Sinonasal Tract: A Report of Two Challenging Cases.

Ewing's sarcoma (ES) is a malignant small round cell tumor that belongs to the primitive neuroectodermal tumor class. ES generally arises in the long bones of the extremities (skeletal form) and less frequently in the soft tissue of the trunk and extremities (extra-skeletal form). Sinonasal localization of ES is an extremely rare event. About 80% of the patients are less than 20 years old with the highest incidence in the second decade of life. The combination of histopathological examination and ancillary methods (such as immunohistochemistry and molecular genetics) is extremely important to establish the diagnosis of ES. The most effective treatment plan for ES includes a multidisciplinary approach with surgery, radiotherapy and chemotherapy. This is a report of two challenging cases of sinonasal ES in two different age groups. The first case is a 13-years old female patient who presented with right nasal obstruction, anosmia, intermittent epistaxis and hearing loss. The second case is a 24-years old male patient who presented with a history of right nasal obstruction, right eye pain and periorbital edema. We present these cases due to the rarity of the disease and the difficulty of diagnosis.

Ewing's sarcoma of the sinonasal tract with orbital extension: a rare case report and literature review.

Ewing's sarcoma is a rare and aggressive tumor which classified as peripheral primitive neuroectodermal tumor. It commonly arises in the long bones of the extremities and rarely in the head and neck region. Localization of the sinonasal tract is a rare occurrence thus the number of clinical studies published in the literature are limited. The diagnosis of this tumor requires a histopathological examination, immunohistochemistry and cytogenetic analysis. Ewing's sarcomas are characterized by a CD99 positivity in immunohistochemistry stain and a t(11:22)(q24:q12) translocation in cytogenetic study. The treatment of choice is the multimodality treatment including surgery, radiotherapy and chemotherapy. This is a case report of sinonasal and orbital Ewing's sarcoma in a 24-year-old male patient who presented with a history of right nasal obstruction, right eye pain and periorbital edema.

Pediatrics Ewing's Sarcoma of the Sinonasal Tract: A Case Report and Literature Review.

Ewing's sarcoma (ES) is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells. Primary ES commonly occurs in early childhood or adolescence. It may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is a case report of sinonasal ES in a 13-year-old female patient who presented with a 7-month history of right nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss. Clinical examination revealed a right nasal mass pushing the septum to the left side and extending to the nasopharynx. Endoscopic biopsy and histopathological analysis showed a small blue cell tumor suggestive of ES. The patient was treated with surgery, radiotherapy, and chemotherapy. After a follow-up of 5 years, the patient remains recurrence-free with excellent functional status and quality of life.

Ectopic intranasal canine tooth in a child: A rare case report and literature review.

INTRODUCTION: Intranasal teeth are a rare form of ectopic teeth. Their clinical manifestation are truly variable. In children, intranasal teeth are typically associated with cleft lip and alveolus. PRESENTATION OF CASE: Here, we report a case of 11 years -old girl presented with right nasal obstruction and occasional headache without any obvious etiology. 0Computed tomography of the paranasal sinuses (coronal and axial view) revealed displaced right upper maxillary tooth with the crown oriented inferiorly and medially toward and within the lower right anterior nasal cavity. She underwent surgery by anterior rhinoscopy and endoscopic guidance. The patient's symptoms were resolved completely post-operatively and remained symptom-free for 18 months postoperatively. DISCUSSION: Ectopic intranasal tooth is a rare phenomenon, with a male predominance and around half of all patients are diagnosed before adulthood. Ectopic intranasal tooth arising from inferior turbinate is very rare. No clear etiological factor has been suggested in most of the reported cases. Idiopathic etiology has been described as an etiologic factor for ectopic teeth. The treatment of intranasal teeth is early surgical extraction to alleviate the symptoms and prevent the possible morbidities. CONCLUSION: Intranasal teeth are a rare form of ectopic teeth encountered to otolaryngology clinic and may cause a variety of symptoms and complications. CT is very useful; it confirms the diagnosis and facilitates surgical planning. Early diagnosis and treatment are very important to avoid their possible complications.

A rare case of nasal septal schwannoma: Case report and literature review.

INTRODUCTION: Sinonasal schwannoma accounts for only 4% of head and neck nerve sheath tumors. Schwannoma of the nasal septum is still the rarest, with only few cases previously reported. CASE PRESENTATION: This study presents the case of a 64-year-old Saudi female of the Eastern Province who presented a several-month history of unilateral progressive nasal obstruction and recurrent episodes of epistaxis. The diagnosis of nasal septal schwannoma was made on the basis of a histopathological examination. The case was managed successfully by endoscopic endonasal excision with no postoperative recurrence. DISCUSSION: Nasal septal schwannoma is rare and has only 32 reported cases. Diagnosis primarily depends on histopathological examination. Endoscopic endonasal excision of the mass is considered the ideal treatment for such pathology. CONCLUSION: Schwannoma of the nasal septum is rare. However, it should be included as a differential diagnosis in a patient presenting with a unilateral nasal mass.

Bilateral dentigerous cysts and ectopic teeth in the maxillary sinuses: A case report and literature review.

INTRODUCTION: Dentigerous cysts are the second most common form of benign odontogenic cysts. These cysts are commonly single lesions. Bilateral and multiple dentigerous cysts are very rare. CASE PRESENTATION: This article presents a rare case report of bilateral dentigerous cysts associated with two ectopic teeth located atypically in the maxillary sinuses in a 19-year-old male manifesting as bilateral upper jaw pain, post-nasal discharge and recurrent sinusitis which was managed via the endonasal endoscopic approach by enucleation of the cysts and teeth removal. CONCLUSION: This paper presented our experience in the management of a rare case of bilateral dentigerous cysts associated with ectopic teeth in the maxillary sinuses. Ct scan of the paranasal sinuses confirmed the diagnosis and the endonasal endoscopic approach is superior to the external approaches in preserving physiological function in managing these patients.

Reliability of the Arabic Glasgow benefit inventory after otolaryngology interventions.

We aim to translate the Glasgow benefit inventory (GBI) questionnaire into Arabic language and assess its reliability in scoring the benefit of patients after otolaryngology interventions. This is a pilot study of an ongoing prospective cohort study. The original GBI questionnaire was translated into Arabic language and back-translated into English by two different experts. The questionnaire was given to patients who were asked to fill it themselves. A group of consecutive adult patients were included in the study. Fifty-one patients were included. Reliability using Cronbach's α was higher than 0.70 for total score and the three subscales. Five loading factors explained 72.9% of the variance reached. The mean benefit of the GBI total score was 30.0 ± 36.37. Subscales scores were as follows: 35.21 ± 25.98 for the general health, 25.81 ± 45.98 for the physical benefit and 29.08 ± 34.45 for the social support. The Arabic GBI questionnaire is reliable to evaluate the quality of life after otolaryngology interventions for Arabic speaking population.