Predilection For Head Research Articles

Recurrent Giant Myofibroblastic Sarcoma of Neck in a Teenager: Overcoming Treatment Challenges

Sarcomas are rare tumors arising from a variety of mesenchymal tissues which are even rarer in head and neck region amounting 1% only of the 5% of sarcomas. About 80% of head and neck sarcomas originate in soft tissue while the remaining 20% arise from bone (Cormier and Pollock in J Clin 54:94−109, 2004). One among the commonly presenting variants of sarcomas in head and neck that our patient was diagnosed with is Low grade myofibroblastic sarcoma (LGMS). These even though uncommon have a predilection for head and neck region particularly the tongue (Mentzel et al. in Am J Surg Pathol 22:1228−38, 1998; Cai et al. in Virchows Arch 463:827−36, 2013; Meng et al. in Chin Med J 120:363–9, 2007; Demarosi et al. in Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 108:248–54, 2009). LGMS was reclassified as a distinct entity by the WHO classification of soft tissue tumors in 2002 (Qiu et al. in Oncol Lett 9:619−25, 2015). Oncological resection is the mainstay of treatment. In case of bulky tumor, resection and reconstruction would be challenging. We report a recurrent LGMS of 15-year-old girl who presented with a ginormous soft tissue swelling in her left neck region which made her day-to-day activities strenuous and made her non ambulatory and the line of treatment executed in order to get her back on feet, healthy & free of the debilitating tumor.

Middle Ear Embryonal Rhabdomyosarcoma in a Five Years Old Child - A Rare Case Report

Rhabdomyosarcoma is the most common pediatric malignancy with a predilection for head and neck region. Embryonal Rhabdomyosarcoma is a variant of rhabdomyosarcoma which is extremely rare in middle ear. We present a case of middle year embryonal rhabdomyosarcoma in a five-year-old child who was treated as otitis media on first presentation and later misdiagnosed as a vascular aural polyp on histopathology.

SYRINGOCYSTADENOMA PAPILLIFERUM OF SCALP – A CASE REPORT OF A RARE CUTANEOUS ADNEXAL NEOPLASM

Syringocystadenoma papilliferum is a rare benign hamartomatous tumor arising from apocrine or eccrine sweat glands with a predilection for head and neck. Here we report a case of a 30 year old female who presented to our opd with a nodule over scalp since birth which turned into a hairless , exophytic pinkish growth with blood tinged discharge slowly over the last 2 years . A punch biopsy of the lesion revealed syringocystadenoma papilliferum. Excision of the lesion is performed and specimen is sent for (hpe) histopathological examination which revealed syringocystadenoma papilliferum with no malignant transformation. Post operative recovery was uneventful and the patient was followed up till 6 months with no history of recurrence.

Case series: Characteristics of Merkel Cell Carcinoma in patients of the Skin Cancer Center of Hospital Italiano de Buenos Aires- Argentina. 2004-2018.

BACKGROUND: Merkel cell carcinoma is described as a malignant and aggressive neuroendocrine tumor, which represents less than 1% of non-melanoma skin tumors. It is a rare type of skin cancer that is associated with a poor prognosis and a high rate of recurrence. Despite significant advances, its pathogenesis is still poorly understood. Early diagnosis and proper management are essential to improve the survival rate. Although it affects both genders, it has male predominance. It is presented as hard, bright, reddish, and asymptomatic tumors with a certain predilection for head, neck and limbs. The treatment is surgical and in some cases, it requires adjuvant radiation therapy. An early diagnosis and appropriate handling are essential to improve the survival rate. METHODS: We conducted an observational, descriptive study ⸻case series⸻, which included 10 patients diagnosed with MCC, from the dermatology service of the Skin Cancer Center of Hospital Italiano de Buenos Aires, from January 2004 to August 2018. RESULTS: Over the course of 15 years, ten patients, 5 men and 5 women, were clinically and histopathologicallly diagnosed with Merkel Cell Carcinoma, with mean age of presentation of 83 years (range: 74-95 years). There was a high frequency (70%) of immunological alterations due to autoimmune diseases, renal-transplanted patients, and chronic lymphocytic leukemia. The therapeutic option was conventional surgery, with a margin of 3 cm, in six patients and Mohs micrographic surgery in four patients. Three patients developed local recurrence, who received adjuvant radiotherapy. CONCLUSION: The mean age of presentation was 83 years of age; there was no predominance according to sex. Most cases were localized in head and neck. Most of the patients had a concomitant immunological alteration. Most cases were resolved by conventional surgery. Recurrence rate was lower than expected (30%).

Cystic lymphangiomas of the tongue: 3 rare cases and a literature review of classifications and therapeutic possibilities

BackgroundCystic lymphangiomas of the tongue are rare. The treatment consists of complete resection and management is generally interdisciplinary due to vital, functional, and esthetic comorbidities.This work is a report of three rare cases of cystic lymphangioma of the tongue, with curious mode of presentation and good outcome, as well as a literature review of clinical features, classifications, and therapeutic possibilities.Case presentationThis work presents three patients. The first one presented with a lymphangioma in the junction between base and body of tongue; the clinical aspect was first similar to hemangioma, and then the diagnosis was re-established after slight enlargement of the mass. The patient was treated with sclerotherapy with great outcome.The second patient presented with a large lymphangioma which presented a clinical resemblance with a tumor of the tongue, the diagnosis was established upon radiological aspect and confirmed upon histological findings.The third patient presented with a macroglossia which appeared 3 years after excision of the cystic hygroma of the neck; the patient was surgically treated with excellent outcome.ConclusionCystic lymphangiomas are rare hamartomatous lymphatic malformations, and they have a predilection for head and neck but the lingual localization is quite rare. Treatment is complete excision. Management is generally interdisciplinary. The main objective is to prevent alteration to function, to treat esthetic comorbidities, and prevent speech impediment especially in very young children.This is the cases of three young children with a rare lingual cystic lymphangioma, with adapted treatment and satisfactory outcome, with a literature review of clinical presentation, classifications, and therapeutic approaches.

RABDOMIOSSARCOMA MAMÁRIO BILATERAL EM ADOLESCENTE: UM RELATO DE CASO

Objetivo: Relatar um caso clínico de rabdomiossarcoma em adolescente com topografia mamária bilateral como lesão inicial ao diagnóstico. Essa neoplasia possui predisposição de desenvolvimento maior no sexo masculino e predileção por cabeça e pescoço e, em seguida, pelo trato geniturinário. Portanto, o seu desenvolvimento em uma adolescente feminina e em região mamária, como forma de lesão primária e de acometimento bilateral, é extremamente rara. Descrição: Paciente feminina, 14 anos, branca, com história de aumento de volume de ambas as mamas há 2 meses, astenia há 1 mês, escondeu seu problema por vergonha. Sem história familiar de câncer. Exame físico: BEG, corada, eupneica, presença de lesão tumoral vegetante e endurecida de 14 X 16 cm na mama direita e massa tumoral palpável endurecida de 10 X 10 cm em mama esquerda. Linfonodomegalia cervical esquerda de 2 cm, microadenopatia axilar bilateral. Diagnóstico em estádio IV. Comentários: A topografia mamária como manifestação inicial desses tumores é extremamente rara. O desconhecimento de informações sobre câncer, somado ao pudor dos adolescentes, pode ter contribuído ao retardo no diagnóstico e para o estádio avançado da doença dessa paciente.

Amiodarone Prevents Atrial Fibrillation Induced by Arsenic Trioxide in Extramedullary Plasmacytoma Involving Lumbar Disc: A Novel Case Report

Extramedullary plasmacytoma (EMP), characterized by infiltration of malignant plasmablastic clones outside the bone marrow in absence of multiple myeloma. Accounting for approximately 4% of all plasma cell dyscrasias, it has a predilection for head and neck region.

Neuro-ophthalmic complications of IgG4-related disease

IgG4-related disease (IgG4-RD) is increasingly recognized as a fibroinflammatory disease with a plethora of organ-specific manifestations but a particular predilection for head and neck tissues, including the nervous system. This review discusses general features and organ-specific presentations of IgG4-RD as well as treatment considerations, particularly emphasizing features of neuro-ophthalmic interest. IgG4-RD is emerging as a common cause of several fibroinflammatory disorders in the head and neck that were previously considered idiopathic, such as sclerosing orbital pseudotumor, orbital myositis, hypophysitis, and hypertrophic pachymeningitis. New and unusual presentations continue to be described, including a number of vascular manifestations. Substantial progress has been made in elucidating the cell types involved in IgG4-RD, and new pathogenic models are being proposed. Although clinicopathologic correlation remains the cornerstone of diagnosis, ancillary tests such as flow cytometry for circulating plasmablasts and PET-computed tomography have high sensitivity, and certain radiologic features are recognized to be particularly suggestive, such as infraorbital nerve enlargement in IgG4-RD orbitopathy. IgG4-RD often responds to steroids but incomplete responses and relapses are common. Rituximab is emerging as a promising new therapy. The current review summarizes manifestations of IgG4RD that are of particular relevance to neuro-ophthalmic practice.

Sural nerve schwannoma: a case report

The differential diagnosis for a swelling on the posterior aspect of lower leg is very few. The least of all suspected is a Benign peripheral nerve sheath tumor that too a schwannoma. Though schwannomas can be found in any part of the body especially with a predilection for head and neck, on the contrary they are extremely rare in the extremities especially the lower extremity. Here we present one of the few cases of a solitary schwannoma, confirmed on Histopathology, arising from the sural nerve of the right leg which did not present with any other symptoms even though itself being of a considerable size. The tumor was successfully removed with no postoperative complications and recurrence on follow up.

Haemangioma of tongue: A rare case report

Vascular anomalies include a wide range of tumors and malformations. Among vascular anomalies, hemangioma is the most common. Hemangioma is considered as hamartomatous lesion, and half of these have predilection for head and neck region, but rarely seen in the oral cavity. Here, we report a case of hemangioma in the posterior third of the tongue in a 10-year-old female, which appeared localized well defined, reddish pink and lobulated mass with well-defined margins on intra-oral examination. A well-defined homogeneously hyperechoic space occupying lesion (measuring = 4.4 cm × 4.2 cm) seen on ultrasonography which showed abnormally increased vascularity on color Doppler suggesting a vascular etiology. Further evaluation was done on contrast enhanced computed tomography scan and magnetic resonance imaging, which confirms its vascular nature and diagnosed as a case of hemangioma.

La maladie de Kimura : une cause méconnue de syndrome néphrotique à lésions glomérulaires minimes de l’adulte

Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Kidney disease is thought to be rare in KD. About a case of adult-onset nephrotic syndrome with minimal change disease, we comment Kimura's disease and its associated kidney damage. Kimura disease should be suspected and included in the diagnosis of adult-onset nephrotic syndrome with minimal change disease.

English

A pilomatricoma is a benign appendage tumor related to hair cells matrix. Pilomatricoma usually occurs as a solitary firm lesion with predilection for head and neck, and upper extremities. It is more common in children. AIM: The objective of this study is to know the clinical presentation and histopathology of this condition. MATERIALS AND METHODS: This is a 4 yrs retrospective and 1 yr prospective study carried in Upgraded Department of pathology, Osmania general hospital between June 2008 and May 2013. Patients data such as sex, age, site of occurrence, clinical presentation were considered.The study included 15 patients with histopathologically diagnosed pilomatricoma. RESULTS: A total of 15 cases were included in our study with an age range from 6-36 year.Out of which benign tumors were 14 (93.3%) and malignant 1 (6.7%). The female: male ratio was 3:2. The sizes of the tumours range from 2-3cm. Most of the cases were seen in Upper extremity-7 cases (46.7%), followed by Head and neck region6 cases (40.0%) and Trunk-2 cases (13.3%). The most common clinical presentation was sebaceous cyst. CONCLUSION: Pilomatricoma generally present as firm subcutaneous nodules in children and young adults and are often misdiagnosed. The physicians and surgeons should be familiar with this entity and consider it in the differential diagnosis of a superficial mass.

Low-grade myofibroblastic sarcoma of the distal femur

INTRODUCTIONLow-grade myofibroblastic sarcoma (myofibrosarcoma) is described to be a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for head and neck. Low-grade myofibroblastic sarcoma of bone is extremely rare. PRESENTATION OF CASEA 50-year-old woman was admitted to our hospital because she had experienced right knee pain for 2 years. Plain radiography showed a honeycombed lesion on the right distal femur, and computed tomography showed a bone tumor with cortex destruction invading the soft tissue. A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. However, histological examination of the extraosseous lesion indicated a slightly hypercellular area containing scattered spindle-shaped atypical cells with enlarged nuclei, suggestive of low-grade sarcoma. Spindle-shaped atypical cells were immunohistochemically positive for SMA. A final diagnosis of low-grade myofibroiblastic sarcoma of the bone was made from a surgically resected specimen. DISCUSSIONThe patient was alive and well with no evidence of disease at 15 months after the surgery without any additional therapy. CONCLUSIONExtensive sampling of a tumor may be necessary to determine the true nature of the tumor and to make an accurate diagnosis.

466. Neoadjuvant Chemotherapy in Merkel's Cell Carcinoma a Case Report

Background: Merkel's cell carcinoma (MCC) is an uncommon and aggressive neuroendocrine skin cancer and has predilection for Head and Neck regions and limbs. The growing incidence and recognition of this cancer in elderly or immunosuppressed individuals suggests that it is becoming an increasing clinical challenge. MCC recently has been demonstrated to have a probable viral pathogenesis related to a novel member of the polyomavirus (termed Merkel cell polyomavirus [MCV]). Five-years overall survival rates is 33%, lower than in melanoma. Surgery is the primary treatment and there are no conclusive data about adjuvancy.

Chondroid Syringoma of Nose

ABSTRACT Chondroid Syringoma is a rare cutaneous tumor which usually arises in middle age with predilection for head and neck region. We report a case of chondroid syringoma of nose with review of literature. How to cite this article Chandregowda BV, Smitha SG, Bhardwaj S, Krishnamurthy T. Chondroid Syringoma of Nose. Clin Rhinol Int J 2012;5(1):30-31.

Vulvar Merkel Cell Carcinoma: A Rare Entity

ABSTRACT Merkel cell carcinoma is a primary small cell carcinoma of the skin that resembles oat cell carcinomas of the lung. This tumor has a predilection for head and neck region but may rarely occur as isolated vulvar tumor. It has aggressive malignant behavior and should not be missed while evaluating vulvar masses. We report a case of 45-year-old Mrs I, who presented with recurrent vulvar swelling and was diagnosed as stage III Merkel cell carcinoma of the vulva. Synonyms Primary neuroendocrine carcinoma, Trabecular carcinoma, Primary small cell carcinoma, Cutaneous apudoma.

Low-grade myofibroblastic sarcoma of the tongue

Low-grade myofibroblastic sarcoma is a neoplasm of atypical myofibroblasts with fibromatoseslike features and a predilection for head and neck sites, including the oral cavity. These lesions have only been characterized in the last 2 decades, and controversies in the concept of neoplastic myofibroblasts still exist. Lack of obvious cytological atypia may result in their being mistaken for reactive fascitislike lesions or fibromatosis and architectural similarities to fibrosarcoma or leiomyosarcoma may complicate the diagnostic process. This paper describes a spindle cell neoplasm in a 40-year-old man that was diagnosed 9 years ago as an unclassifiable myofibroblastic proliferation. The recurrent tumor, which presented 6 years following excision of the original tumor, was subsequently classified as a low-grade myofibroblastic sarcoma. The morphological, immunohistochemical, and electron microscopic features of this unusual sarcoma and the most likely differential diagnoses are discussed.

Malignant eccrine hidradenoma of neck causing acute heart failure

Tumours of eccrine sweat glands are uncommon, with complex classification and different terms used even for the same tumour. Therefore, for practical purposes, it may be sufficient to differentiate between benign and malignant. Malignant eccrine hidradenoma has a predilection for head and neck, with high incidence of recurrence following surgical excision and also regional and distant metastases. We describe a case, which presented as a medical emergency with symptoms of severe anaemia and acute heart failure secondary to intermittent bleeding from a huge ulcerative neck lesion, which was subsequently diagnosed as eccrine hidradenocarcinoma. The tumour was successfully treated with complete surgical excision and reconstruction followed by radiotherapy. Two years postoperatively, the patient is very well with no sign of recurrence.

Recurrent Nephrotic Syndrome Associated with Kimura’s Disease in a Young Non-Oriental Male

Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Renal disease is rarely associated with it. We describe a young non-Oriental male with KD and relapsing steroid-responsive minimal-change nephrotic syndrome. This case of KD associated nephrotic syndrome is unique in the relapsing nature of the nephropathy and the non-Oriental origin of the patient. We comment on KD and nephrotic syndrome and on treatment strategy.