Castleman’s Disease Presenting as a Parotid Mass in the Pediatric Population: A Report of 2 Cases

Introduction. Angiofollicular lymph node hyperplasia (Castleman’s disease) is a nonmalignant lymphoproliferative disorder that generally involves the lymph nodes of young adults, most commonly in the mediastinum. Rarely, Castleman’s disease may present in the parotid gland. The disease can be further classified into unicentric or multicentric forms, with considerable differences in presentation, treatment, and prognosis.Case(s). We present cases of two pediatric patients, aged 7 and 11, who both presented with a slow-growing, painless parotid mass. In each case, the mass was excised via a superficial parotidectomy and the diagnosis made postoperatively upon further pathologic examination. At 6 months of follow-up, both had fully intact facial nerve function and no evidence of recurrence.Discussion. Castleman’s disease presents a diagnostic challenge in the head and neck region, as radiographic characteristics and fine needle aspiration results are often inconclusive. Definitive diagnosis requires surgical excision for pathologic examination. The unicentric form generally presents as a painless mass and can be successfully treated with complete excision. The multicentric form is associated with constitutional symptoms and its treatment remains controversial.Conclusion. Although rare, clinicians should be aware of both forms of Castleman’s disease when creating a differential diagnosis for parotid masses.

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Localized Castleman’s Disease in Retroperitoneum Mimicking Broad Ligament Leiomyoma – a Diagnostic Challenge

Nepal Journal of Obstetrics and Gynaecology ◽

10.3126/njog.v10i2.14340 ◽

2016 ◽

Vol 10 (2) ◽

pp. 57-59

Author(s):

S Gon ◽

D Mallik ◽

A Bhattacharya ◽

B Majumdar ◽

M Sengupta

Keyword(s):

Broad Ligament ◽

Castleman’S Disease ◽

Surgical Excision ◽

Young Female ◽

Castleman Disease ◽

Castleman's Disease ◽

Diagnostic Challenge ◽

Diagnostic Dilemma ◽

Vascular Type ◽

Complete Surgical Excision

Primary retroperitoneal tumours pose a diagnostic dilemma and therapeutic challenges because of their location and late detection. Localized Castleman Disease (CD) in retroperitoneal location is a rare occurrence, with incidence of only 4% cases occurring in diverse locations like retroperitoneum. A rare case of unicentric localized Castleman Disease localized in broad ligament is hereby reported which was clinically & radiologically suspected as broad ligament leiomyoma but finally diagnosed as Castleman’s Disease, hyaline vascular type in a young female posing a diagnostic challenge both for the pathologist and clinician. Definite diagnosis is essential as complete surgical excision in case of unicentric Castleman’s Disease has good prognosis after resection.

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Castleman's Disease: A Case Report of the Unicentric Type

Case Reports in Surgery ◽

10.1155/2012/175272 ◽

2012 ◽

Vol 2012 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

J. Gomez-Ramirez ◽

M. Posada ◽

L. Sanchez-Urdazpal ◽

E. Martin-Perez ◽

L. Del Campo ◽

...

Keyword(s):

Lymph Node ◽

Castleman’S Disease ◽

Surgical Excision ◽

Castleman's Disease ◽

Angiofollicular Lymph Node Hyperplasia ◽

Human Herpes Virus 8 ◽

Complete Surgical Excision ◽

Immunodeficiency Virus ◽

Generalized Lymphadenopathy ◽

Aggressive Clinical Course

Castleman's disease, or angiofollicular lymph node hyperplasia, is a relatively rare disorder characterized by the benign proliferation of lymphoid tissue related to the chronic human herpes virus 8 (HHV-8) infection and the human immunodeficiency virus (HIV). Two clinical entities have been described: a unicentric presentation with the disease confined to a single anatomic lymph node and a multicentric presentation characterized by generalized lymphadenopathy and a more aggressive clinical course. Also, three histopathological subtypes have been described: hyaline-vascular, plasma cell, and a mixed variant. Preoperative diagnosis of hyaline-vascular Castleman's disease is difficult, and the definitive result is based on postoperative pathological findings. The gold standard therapy is the complete surgical excision.

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Schwannoma Base of Tongue: Report of a Rare Case and Review of Literature

International Journal of Head and Neck Surgery ◽

10.5005/jp-journals-10001-1161 ◽

2013 ◽

Vol 4 (3) ◽

pp. 133-135 ◽

Cited By ~ 2

Author(s):

Hitendra Prakash Singh ◽

SP Agarwal ◽

Nidhi Verma ◽

Madhukar Vashistha ◽

Amit Chaddha ◽

...

Keyword(s):

Rare Case ◽

Neck Region ◽

Surgical Excision ◽

Needle Aspiration ◽

Review Of Literature ◽

Complete Excision ◽

Base Of Tongue ◽

Magnetic Resonance Imaging Mri ◽

Needle Aspiration Cytology ◽

Slow Growing

ABSTRACT Schwannomas or neurilemmomas are benign, slow growing, usually solitary and encapsulated tumor, originating from Schwann cells of the nerve sheath. Extracranially, 25% of all schwannomas are located in the head and neck region. The intraoral lesions show a predilection for the tongue, followed by the palate, buccal mucosa, lips and gingiva respectively. Among these, base of tongue schwannoma are extremely rare. Schwannomas involving the tongue base remain asymptomatic unless they attain appreciable size. Diagnostic investigations include ultrasound scanning, computed tomography, magnetic resonance imaging (MRI), and fine-needle aspiration cytology. MRI is superior to other imaging modalities for the examination of the base of the tongue. The definitive diagnosis is based on histopathology. Surgical excision or enucleation with preserve nerve function is the treatment of choice for this rare tumor. The most common approach for complete excision is transoral route for base of tongue schwannoma. Authors report a case of base of tongue schwannoma which was treated with surgery by transoral route. How to cite this article Singh HP, Kumar S, Verma N, Vashistha M, Chaddha A, Agarwal SP, Babu S. Schwannoma Base of Tongue: Report of a Rare Case and Review of Literature. Int J Head Neck Surg 2013;4(3):133-135.

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Castleman’s disease presenting as parotid mass: a case report

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20190786 ◽

2019 ◽

Vol 5 (2) ◽

pp. 504

Author(s):

Ankur Batra ◽

Megha Goyal

Keyword(s):

Castleman’S Disease ◽

Surgical Excision ◽

Needle Aspiration ◽

The Body ◽

Unknown Etiology ◽

Castleman's Disease ◽

Nerve Preservation ◽

Parotid Region ◽

The Right ◽

Conclusive Diagnosis

Castleman’s disease is a rare, benign, lymphoproliferative disorder of unknown etiology. It can involve any lymph node group in the body with mediastinum being the commonest site. Salivary glands are affected rarely. We report a 35 year old male patient who presented with slowly progressive, painless right sided parotid swelling for 3 years. Clinical examination showed a 4×3 cm single, firm, non-tender, non-pulsatile swelling with smooth surface and normal overlying skin present in the right parotid region. Magnetic resonance imaging (MRI) revealed a well-defined hyper-intense mass lesion on T2 measuring 3.9×3×2.8 cm and Fine needle aspiration cytology (FNAC) showed intense lymphoplasmacytic infiltrates. No conclusive diagnosis could be made on the basis of FNAC. So, the excision of parotid mass with facial nerve preservation was done. The final histopathology confirmed the diagnosis as Castleman’s disease. Although Castleman’s disease in the parotid gland is rare, clinicians should consider it as the differential diagnosis of any solid tumors that exhibit non-specific presenting characteristics and surgical excision is preferred treatment for unicentric disease.

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Lipofibromatosis: An Unusual Head and Neck Mass in the Paediatric Age Group

Philippine Journal of Otolaryngology Head and Neck Surgery ◽

10.32412/pjohns.v26i1.605 ◽

2011 ◽

Vol 26 (1) ◽

pp. 39-41

Author(s):

Yogender Singh Kadian ◽

Kamal Nain Rattan ◽

Shalini Aggarwal ◽

Shilpi Modi ◽

Rajnish Kalra

Keyword(s):

Head And Neck ◽

Neck Region ◽

Surgical Excision ◽

Needle Aspiration ◽

Neck Mass ◽

Soft Tissue Neoplasm ◽

Complete Excision ◽

Complete Surgical Excision ◽

Paediatric Age ◽

Rare Lesion

Objective: To describe a rare case of lipofibromatosis presenting as a head and neck mass in a 6-year-old child. Method: Design: Case Report Setting: Tertiary Public General Hospital Patient: One Result: A six-year-old male child admitted with a large right head and neck region mass underwent complete excision of a possible soft tissue neoplasm following investigations which included Fine Needle Aspiration Cytology, Ultrasonography and Computed Tomography. Histopathological examination yielded lipofibromatosis, a very rare lesion with a distinctive fibrofatty pattern. The patient was well with no recurrence after three months of follow up. Conclusion: Although lipofibromatosis is a rare lesion in children and has a predilection for distal extremities, it may also present as a mass in the head and neck area. Complete surgical excision is feasible and is the only treatment option available for this rare lesion Keywords: lipofibromatosis

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Primary seminoma arising in the posterior mediastinum: a diagnostic challenge

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2021.2028 ◽

2021 ◽

Author(s):

Sara Parini ◽

Paolo Spina ◽

Esther Papalia ◽

Renzo Boldorini ◽

Michele Abruzzese ◽

...

Keyword(s):

Incidental Finding ◽

Germ Cell Tumors ◽

Surgical Excision ◽

Needle Aspiration ◽

Posterior Mediastinum ◽

Anterior Mediastinum ◽

Tracer Uptake ◽

Pathology Report ◽

Diagnostic Challenge ◽

Complete Excision

Primary mediastinal germ cell tumors are a rare finding, and one third of them are seminomas. Seminomas are found in the anterior mediastinum, whereas they are extremely rare within the posterior compartment. Most clinicians would not consider a primary seminoma in the differential diagnosis of a posterior mediastinal mass, as only two cases have been reported in literature. Here we present the case of a 57-year-old male with a primary seminoma arising in the left posterior mediastinum. He was asymptomatic and the mass was an incidental finding. Positron emission tomography (PET) revealed a small area with an avid tracer uptake. Transthoracic needle aspiration led to a non-diagnostic result. Due to the strong suspect of malignancy, a surgical excision was chosen to obtain a diagnosis. He underwent complete excision, and pathology report demonstrated a mediastinal seminoma. Subsequent further staging did not reveal any other location of the disease. Given the complete excision of the primary tumor, active surveillance was the treatment of choice. The patient is free of disease 48 months after diagnosis.

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Castleman’s disease: an entity forgotten in ENT

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20211593 ◽

2021 ◽

Vol 7 (5) ◽

pp. 905

Author(s):

Mohamed Riyas Ali ◽

Vikram Wadhwa ◽

Ravi Meher ◽

Reena Tomar ◽

Karishma Singh ◽

...

Keyword(s):

Castleman’S Disease ◽

Treatment Modalities ◽

Retropharyngeal Space ◽

Castleman's Disease ◽

Complete Excision ◽

Vascular Type ◽

Hyaline Vascular Type ◽

Systemic Lymphadenopathy ◽

Rare Site ◽

Usual Site

Castleman’s disease (CD) usually presents as localized or systemic lymphadenopathy or as an extra nodal mass. The usual site of presentation are mediastinum, retroperitoneum, axilla and mesentery. Only 3 cases of CD have been reported in retro pharyngeal space. We report a case of 20 year old male patient with retropharyngeal mass. He presented with difficulty in swallowing, change in voice and respiratory distress. The mass was removed in-toto transorally after performing elective tracheostomy. The histopathological findings were consistent with hyaline vascular type of CD. He was decannulated after two day and postoperative period was uneventful. Postoperative CT imaging confirmed the complete excision of tumor and patient is on follow up, with no signs of recurrence. The presentation of tumour in the retropharyngeal space which is a rare site of occurrence add to the uniqueness of this case. Unicentric CD has an excellent prognosis and surgery is the management of choice. Its clinical features, histological subtypes, treatment modalities and prognosis are discussed.

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Recurrent and Hodgkin’s lymphoma-associated multicentric Castleman’s disease in head and neck region

International Journal of Otorhinolaryngology and Head and Neck Surgery ◽

10.18203/issn.2454-5929.ijohns20204193 ◽

2020 ◽

Vol 6 (10) ◽

pp. 1882

Author(s):

Vinodh Kumar Selvaraj ◽

Deleep Kumar Gudipudi

Keyword(s):

Monoclonal Antibodies ◽

Hodgkin’S Lymphoma ◽

Treatment Options ◽

Castleman’S Disease ◽

Neck Region ◽

Hodgkin's Lymphoma ◽

Castleman's Disease ◽

First Case ◽

Field Radiation ◽

Involved Field

Background: Castleman’s disease (CD) is a rare lymphoproliferative disorder. It can involve single (unicentric CD) or multiple (multicentric CD) lymph nodal regions. It occurs predominantly in mediastinum, and treatment options include surgery, radiotherapy, chemotherapy, and monoclonal antibodies. Methods: Here, we describe two cases of CD which presented with stridor. The first case was a 38-year-old male, a recurrent multicentric CD in retropharyngeal and cervical lymph nodal regions, treated with radiotherapy and rituximab. The second case was a 25-year-old male, a multicentric CD in lower cervical lymph nodal region, treated with steroids and radiotherapy. He subsequently developed Hodgkin’s lymphoma and was treated for the same with chemotherapy and involved-field radiation therapy (IFRT).Results: Post-treatment, both the patients were asymptomatic and progression-free at 15 months and 42 months follow-up, respectively.Conclusions: Combined modality of treatment with radiotherapy and chemotherapy or monoclonal antibodies offers good local control in multicentric CD.

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Nodular fasciitis of the parotid gland engulfing the facial nerve: a conservative approach

BMJ Case Reports ◽

10.1136/bcr-2019-231203 ◽

2019 ◽

Vol 12 (10) ◽

pp. e231203

Author(s):

Stephen Bennett ◽

Kristian Hutson ◽

Olakunle Ajayi ◽

Andreas Hilger

Keyword(s):

Parotid Gland ◽

Facial Nerve ◽

Benign Lesion ◽

Surgical Excision ◽

Needle Aspiration ◽

Nodular Fasciitis ◽

Conservative Approach ◽

Complete Excision ◽

Diagnostic Uncertainty ◽

Accepted Practice

Nodular fasciitis (NF) is a rapidly growing benign lesion rarely reported in the parotid gland. It shares cytological features with other benign and malignant parotid neoplasms and may be difficult to diagnose based on fine needle aspiration cytology alone. Given this diagnostic conundrum, surgical excision for histology is recommended to facilitate definitive diagnosis. A case with significant involvement of the facial nerve is described, which has not previously been reported in the literature. Despite features of potential malignancy, the decision was taken to biopsy the lesion and not proceed to complete excision in an attempt to reduce the risk of facial nerve injury. Expert opinion later diagnosed NF. Contrary to accepted practice, where diagnostic uncertainty remains around the malignant potential of a lesion, risks and benefits of complete excision versus a conservative approach should be carefully weighed especially when the facial nerve is involved and NF is a possible diagnosis.

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Asymptomatic nodule on face: Dermoscopic and histopathological clue for diagnosis

Nepal Journal of Neuroscience ◽

10.3126/njn.v17i1.28369 ◽

2020 ◽

Vol 17 (1) ◽

pp. 63-65

Author(s):

Anisha Joshi ◽

Deeptara Pathak Thapa

Keyword(s):

Head And Neck ◽

Peripheral Nerves ◽

Neck Region ◽

Surgical Excision ◽

Benign Tumors ◽

Complete Excision ◽

Head And Neck Region ◽

The Common ◽

Slow Growing ◽

Rare Diagnosis

Schwannomas/ neurilemmomas are benign tumors of nerve sheath arising from Schwann cells that form myelin sheath around peripheral nerves. They are usually solitary, slow growing and encapsulated lesions. Head and neck are the common sites. We report a case of a 38 years old Nepalese female who had presented with a solitary asymptomatic, slow growing nodule on the left side of the chin for the last three years. Dermoscopy of the lesion revealed arborizing vessels with brownish pigmentation overlying a whitish to pinkish background. Complete excision of the lesion was performed. Histopathological evaluation of the lesion revealed schwannoma. Though schwannomas are a rare diagnosis, they should be considered as a differential diagnosis of any unilateral, asymptomatic, slow growing nodule in the head and neck region. Dermoscopy is a useful tool which helps to differentiate schwannoma from other lesions. Histopathology is the gold standard for diagnosis and the treatment of choice is surgical excision.

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