Laryngeal Anaplastic Lymphoma Kinase-Positive B-cell Lymphoma: Case Report and Review.

Larynx is an uncommon extranodal site for non-Hodgkin lymphoma (NHL). Anaplastic lymphoma kinase (ALK)-positive B-cell lymphoma is a rare and aggressive form of NHL. A 19-year-old male presented to the ENT department with globus sensation, hoarseness, cervical lymphadenopathy and weight loss. A 70-degree rigid endoscopic examination of the larynx showed a vascular, irregular, submucosal mass arising from the right aryepiglottic fold causing near complete obstruction of the laryngeal airway. PET-CT showed hypermetabolic lesions in the supraglottis, cervical lymph nodes, cervical spine, ribs and abdominal lymph nodes. Biopsy was taken from the supraglottic mass as well as the enlarged cervical lymph nodes, which revealed ALK-positive large B-cell NHL. In this report, we present a rare case of ALK-positive large B-cell NHL of the larynx, discussing its clinical, radiological and pathological features. A limited review of literature is also presented. There is a need to develop a database for the description of lymphomas affecting the larynx and this case report adds to the existing knowledge of this rare entity.

A common presentation of an uncommon pathology: Kimura disease.

Kimura's disease is a chronic, benign inflammatory condition of the subcutaneous tissue. It presents as painless, subcutaneous nodules of the head and neck, which are firm, painless, and may be single or multiple. It is most commonly seen in young adult Asian men. The nodes remain stable or may slowly enlarge over time. Elevated serum immunoglobulin E (IgE) levels, peripheral blood eosinophilia and lymphoid proliferation with eosinophilic infiltration on histopathological examination are the characteristic features. Spontaneous regression is usually seen. The diagnosis of Kimura's disease can be difficult and misleading, and it is important not to ignore histopathological features.

Retropharyngeal Lipomatous Hamartoma: Case Report and Review of Literature.

Introduction: Lipomas of the of the head and neck region are rare, more so in the retropharyngeal space. Lipomas in this region can produce symptoms that demand surgical excision. This paper describes a case of lipomatous hamartoma of the retropharynx, which to the best of our knowledge has not yet been reported in English literature. Case Report: A 53-year-old gentleman presented to the ENT department with snoring, voice change and stridor. Examination revealed a smooth bulge in the posterior wall of the oropharynx causing near complete obstruction of the airway. A contrast enhanced computed tomogram revealed a non-enhancing hypodense lesion in the retropharyngeal space extending from C1-C4 level, which was suggestive of a lipoma. The tumour was surgically excised trans-orally. A limited review of literature is also presented. Conclusion: Trans-oral approach is preferred to external approach for surgical removal of benign retropharyngeal tumours that cause obstructive symptoms, as our case. This approach is safe, effective, and associated with lesser post-operative morbidity.

Paratesticular tumors. A clinicopathological study from a single tertiary hospital in North India.

OBJECTIVE: Paratesticular tumors (PTT) are rare and form a heterogenous group, ranging from benign to malignant high grade sarcomas. This study was undertaken to describe the clinicopathological spectrum of PTTs received over a 20-year period. METHODS: All primary and secondary PTTs diagnosed from 2000 to 2019 in the pathology department of a tertiary care hospital in North India were retrospectively reviewed. Gross, histopathological features and immunohistochemistry (IHC) findings were correlated with clinical details. RESULTS: A total of 169 intra-scrotal tumors were diagnosed during the study period, out of which there were 30 PTTs (in 27 patients) comprising 17.75%. Age range was 4 to 85 years (median 58 years). Benign PTTs were the commonest (n = 21, 70%), followed by metastasis to the paratesticular region (n = 6, 20%) and then primary malignant PTTs (n = 3, 10%). The commonest benign PTT was lipoma (n = 16, 76.19%), followed by adenomatoid tumor (n = 3, 14.28%) with one case each (4.76%) of cellular angiofibroma and hemangioma. Among primary malignant PTT, there were two cases of rhabdomyosarcoma, and one case of biphasic malignant mesothelioma. Metastatic tumors included four cases of prostatic adenocarcinoma, and one case each of pancreatic signet ring cell carcinoma and clear cell renal cell carcinoma. CONCLUSION: PTTs show a wide clinicopathological spectrum. Benign PTTs are commoner than malignant PTTs. Meticulous grossing and histopathological examination supplemented by IHC is essential for an accurate diagnosis of this heterogenous class of tumors, which influences the role of adjuvant therapy and patient prognosis.

Systemic and primary cutaneous anaplastic large cell lymphoma: Clinical features, morphological spectrum, and immunohistochemical profile.

BACKGROUND: T-cell lymphomas with anaplastic morphology typically comprise of anaplastic lymphoma kinase positive, anaplastic large cell lymphoma (ALK+ ALCL), ALK-negative ALCL (ALK- ALCL), and primary cutaneous ALCL (PC-ALCL). However, other entities such as diffuse large B-cell lymphoma, peripheral T-cell lymphoma, Hodgkin lymphoma, and undifferentiated carcinoma can also show similar anaplastic features. AIMS: To study the clinical features and histological spectrum of ALCL and emphasize the role of immunohistochemistry (IHC) in their diagnosis and categorization. SETTING AND DESIGN: Eight cases of ALCL diagnosed over a period of 4 years were selected for the study. MATERIALS AND METHODS: Histopathological review and IHC was performed on all cases. Two ALK+ ALCL cases were tested by fluorescent in situ hybridization (FISH) for t(2;5)(p23;q35). RESULTS: There were four cases of ALK+ ALCL and two each of ALK- ALCL and PC-ALCL. Histologically, all the subtypes showed pleomorphic and "hallmark" cells with strong CD30 expression and variable loss of T-cell antigens. One case of PC-ALCL was leukocyte common antigen (LCA) negative. Epithelial membrane antigen was positive in all the six systemic ALCL cases. Two cases tested for t(2;5)(p23;q35) by FISH were positive. CONCLUSIONS: Diagnosis of ALCL is based on recognizing the key morphological features, especially the presence of "hallmark" cells. IHC is essential for confirmation of diagnosis and excluding other malignancies with anaplastic morphology. The inclusion of CD30 in the initial IHC panel will help identify LCA negative cases and avoid misdiagnosis.

Histoplasmosis Presenting as a Laryngeal Ulcer in an Immunocompetent Host.

Histoplasmosis is a granulomatous disease of worldwide distribution caused by a dimorphic fungus Histoplasma capsulatum. Majority of primary infections in immunocompetent hosts are asymptomatic or may present with flu-like illness. Histoplasmosis may occur in three forms: (i) Primary acute pulmonary form, (ii) chronic pulmonary and (iii) disseminated form. The manifestations of disseminated form of histoplasmosis are fever, weakness, weight loss, hepatosplenomegaly, and mucocutaneous lesions. The mucosal involvement could be oropharyngeal or laryngeal involvement. We report an unusual case of histoplasmosis presenting as a laryngeal ulcer in an immunocompetent host.

Mullerian choristoma in a case of spinal dysraphism.

It is extremely rare to find mullerian choristomas in association with spinal dysraphism, with <10 cases published in English literature. We report a case of heterotopic uterus and fallopian tube-like tissue within a lumbar subcutaneous lipoma associated with spina bifida and tethered cord. A 21-year-old lady presented with lumbar swelling since birth and dull pain in the lower back. Magnetic resonance imaging showed spina bifida at level L3 and L4, tethering of the cord and a subcutaneous lipomatous swelling. Biopsy revealed lobules of fibroadipose tissue embedded in which were seen organoid cystic structures containing prominent smooth muscle coats in their wall. These cystic structures were lined by the endometrium and showed fallopian tube-like papillary infoldings. Immunohistochemistry showed estrogen receptor positivity in the epithelium, stroma, and smooth muscles. The epithelial cells were also positive for cancer antigen 125 and cytokeratin 7 while the stromal cells showed CD10 positivity, supporting mullerian derivation. The pathogenesis and differential diagnosis of such lesions is discussed.