Published by : PROFESSIONAL MEDICAL PUBLICATIONS
|October December 2005||
Naushaba Rahat1 & Saleem Sadiq2
Rhinosporidiosis is a rare chronic granulomatous infection that usually manifests as vascular friable polyps arising from the nasal mucosa or external structures of the eyes caused by Rhinosporidium seeberi, recognized as water mold-related fungus.
We report a case of rhinosporidiosis in a twenty six years old male presenting at the Department of ENT, Jinnah Postgraduate Medical Centre Karachi with the complaints of blockage of nose and bleeding from nose for four months. EUA and endoscopy revealed polypoid mass in nasopharynx and nose. CT scan revealed a soft tissue mass in the nasopharynx with the impression of a polyp or a neoplasm. Histological examination revealed variable sized cysts with a chitinous wall consistent with rhinosporidiosis.
Key words: Rhinosporidium seeberi, chronic granulomatous infection.
Pak J Med Sci October-December 2005 Vol. 21 No. 4 494-496
1. Dr. Naushaba Rahat
Department of Pathology,
Peoples Medical College for Girls,
2. Dr. Saleem Sadiq
Department of Pathology,
Basic Medical Sciences Institute,
Jinnah Postgraduate Medical Centre,
Prof. Saleem Sadiq
Department of Pathology
Jinnah Postgraduate Medical Centre
Received for publication: November 10, 2004
Accepted: June 15, 2005
Rhinosporidiosis is a chronic granulomatous infection that usually manifests as vascular friable polyps arising from the nasal mucosa or external structures of the eyes. It was initially described by Seeber, in 1900 in an individual from Argentina1. The etiological agent of rhinosporidiosis is Rhinosporidium seeberi, that has never been cultured. R. seeberi, traditionally have been believed to be a fungus. Recent 18s ribosomal ribonucleic acid (rRNA) gene analysis has placed R. seeberi into a novel group of aquatic parasites2. Most persons with rhinosporidiosis have had bathing or working exposure to stagnant water1. It is endemic in India, Srilanka, Bangladesh with only sporadic occurrence in United States3. It is uncommon in Pakistan4. Thought to be a zoonotic organism because it is seen in cattle, horse and mules5.It is clinically characterized by formation of papillomatous or polypoid mass4.
We report a case of rhinosporidiosis, a rare infectious disease seen in the Department of Pathology, Basic Medical Sciences Institute of J.P.M.C. This is the second such case seen in the last 10 years in our Department.
A twenty six years old male presented at the Department of ENT, JPMC with complaint of bleeding from nose for four months, and blockage of nose . EUA and endoscopy revealed polypoid mass in nose and nasopharynx. CT scan of paranasal sinuses revealed a soft tissue mass in the nasopharynx, measuring 4 x 3 cm related to left side. Left maxillary, sphenoidal and frontal sinuses were clear. Both orbits were normal in limits. Bone structure was normal. Rigid nasal endoscopy and biopsy of the mass was done.
Examination revealed two irregular grey white soft tissue pieces, larger measuring 2 x 1.5 x 1 cm and smaller 1.5 x 1.5 cm. Microscopic examination revealed polypoidal fragments of nasal mucosa with mucous glands. A mild to moderate chronic inflammatory infiltrate, and scattered cysts of varying sizes with an outer chitinous shell with an inner cellulose wall enclosing large numbers of spores, smaller trophic forms and a foreign body giant cell reaction (Fig-I).
Figure-I: Photomicrograph of nasopharyngeal mucosa showing
numerous cysts of Rhinosporidium seeberi (Haematoxylin and Eosin x 200)
Rhinosporidiosis is a chronic granulomatous infectious disease, characterized by hyperplastic polypoid lesions of the mucous membrane, predominantly nasal but, rarely of the skin and viscera5.
The condition was first observed by Professor Malbren, of Buenos Aires, in 1892. In 1896 Seeber re-examined this material and included it in his thesis for the M.D. degree, but failed to name it. Belou in 1903, designated the organism as Coccidium seeberi, though Wernicke apparently had suggested the name in 1900. The first case recognized in India was by O’Kinealyi in 1894. Minchin and Fantham in 1905 reviewed the material of O’Kinealyi’s case and called the organism Rhinosporidium kinealyi, being unaware of the fact that it had earlier been named Coccidium seeberi. Ashworth after a study of Rhinosporidium, proved that it was not a sporozoa, but belonged to the group phycomycetes in the sub-order of Chytridineae, and called it Rhinosporidium seeberi, which has become its accepted name6. Since then the microbe has been considered a fungus by most microbiologists, although its taxonomy has been debated. Using consensus polymerase chain reaction (PCR) approach, Fredrick and colleagues amplified a portion of the R. seeberi 18SrRNA gene directly from infected tissue. Analysis of the aligned sequence and inference of phylogenetic relationships showed that R. seeberi is a protist from a novel clade of Parasites that infect fish and amphibians and R. seeberi is not a classic fungus, but rather the first known human pathogen from the Drips clade, a novel clade of aquatic protistant parasites2.
Majority of cases are reported from India, Srilanka and Bangladesh. It has been recognized in many other parts of the world, like South America, United States, England, Egypt, South Africa6. Predominantly male are involved (male to female ratio 4:1).1 It is uncommon in Pakistan4. Mode of transmission is via water or dust, from which the endospores penetrate the nasal cavity mucosa, mature into sporangium within the submucosal compartment and after maturation burst with release of sporangia into surrounding tissue6. No immune deficiency has been associated with infection1. Common sites of involvement are nose and nasopharynx. Other sites are larynx, tracheobronchial tree, esophagus, conjunctiva, ear, bone and skin6.
It usually presents as single or multiple, pedunculated or sessile masses, pink to deep red in colour, usually described as strawberry like appearance. They bleed easily with a history of nasal obstruction or epistaxis. In 1923, Ashworth described in detail the life cycle of the organism in tissue1. Histologically the infected tissue reveals granulomatous reaction (mixed cell granuloma), pseudocystic abscesses and fibrosis around the causative organism4.
The various stages in the life cycle of the organism may be found in the tissue, but more readily recognizable are the sporangia in varing stages of maturity and sizes, ranging from 120 to 300 microns in diameter and characterized by an outer chitinous shell with an inner cellulose wall enclosing thousands of spores7 (Fig 1). Diagnosis is established by observing the characteristic appearance of the organism in tissue biopsies1. R. seeberi is clearly seen in hematoxylin and eosin stained section.
The wall of the maturing sporangium and the spores are stained positively by the Gridley’s technique, periodic acid schiff (PAS), Gomori methenamine silver (GMS). The condition must be distinguished from the other polypoid lesions of the nasal cavity1: both non-neoplastic and neoplastic.
The causes of non-neoplastic polyps include inflammatory polyps associated with chronic sinusitis, allergic rhinitis, tuberculosis, lepromatous leprosy, rhinoscleroma and inflammatory pseudotumours8. The causes of neoplastic polyps include squamous cell papilloma, juvenile angiofibroma, schwannoma, nasopharyngeal carcinoma, rhabdomyosarcoma, fibrosarcoma and malignant lymphoma9. Histologically inflammatory polyps are characterized by polypoid lesions, covered by respiratory epithelium, with a marked edematous, vascular myxomatous stroma, containing fibroblasts, eosinophils, plasma cells and lymphocytes5. Tuberculosis can produce polypoid mass in nasal cavity8, histologically characterized by caseating granulomas, giant cells of Langhans type and chronic inflammatory infiltrate. Lepromatous leprosy, histologically is characterized by extensive infiltration of Mycobacterium. leprae-laden histiocytes in the submucosa8. Other differential include rhinoscleroma, which was microscopically characterized by Mikulicz cells, the Russell bodies and bacillus Klebsiella rhinoscleromatis. Inflammatory pseudotumors are tumor like lesions, histologically heterogenous consisting of edematous or hyalinized fibrous tissue infiltrated with varying number of plasma cells, Russell bodies and lymphocytes8.
1. Hospenthal DR. Rhinosporidiosis [online] [Accessed March 8,2002]. URL: http://www.emedicine.com/med/topic2029.htm
2. Fredricks DN, Jolley JA, Lepp PW etal: Rhinosporidium seeberi: a human pathogen from a novel group of aquatic protistan parasites. Emerg Infect Dis 2000; 6(3):273-82.
3. Hafiz MM, Haroon AA. Prevention of recurrence of rhinosporidiosis after conventional treatment. Pak J Otolaryngology 1992; 8:173-76.
4. Saqulain G, Jalisi M. Rhinosporidiosis: Presenting as a mass in the oral cavity. Pak J Otolaryngology 1996; 12:218-20.
5. Wenig BC, Rhinosporidiosis, Atlas of head and neck Pathology WB Saunders, Philadelphia , 1993, 20.
6. Thomas T, Gopinath N, Betts RH. Rhinosporidiosis of the Bronchus. Br J Surg 1957 44:316-19.
7. Friedman I, Osbom DA. Rhinosporidiosis In: Pathology of granulomas and neoplasms of the nose and paranasal sinus, Churchill Livingston New York 1982; pp.64-69.
8. Firdousi FA, Sadiq S, Kadar AA. Rhinoscleroma. J Pak Med Assoc 2000; 50(8):276-77.
9. Shanmugaratnam K. Histological typing of upper respiratory tract tumours. World Health Organization, Internation Histological Classi-fication of tumours Geneva; 1978; p.19.
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